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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

Originally publishedhttps://doi.org/10.1161/CIR.0000000000000602Circulation. 2019;139:e637–e697

Table of Contents

  • Preamble e638

  • 1. Introduction e641

    • 1.1. Methodology and Evidence Review e641

    • 1.2. Organization of the Writing Committee e641

    • 1.3. Document Review and Approval e641

    • 1.4. Scope of the Guideline e642

    • 1.5. Abbreviations e642

  • 2. Background and Pathophysiology e643

    • 2.1. Anatomic and Physiological Terms e643

    • 2.2. Severity of ACHD e643

    • 2.3. The ACHD Anatomic and Physiological Classification e643

  • 3. General Principles e646

    • 3.1. ACHD Program e646

    • 3.2. Access to Care e648

    • 3.3. Delivery of Care e648

    • 3.4. Evaluation of Suspected and Known CHD e648

      • 3.4.1. Electrocardiogram e648

      • 3.4.2. Ionizing Radiation Principles e651

      • 3.4.3. Echocardiography e651

      • 3.4.4. CMR Imaging e651

      • 3.4.5. Cardiac Computed Tomography e651

      • 3.4.6. Cardiac Catheterization e651

      • 3.4.7. Exercise Testing e651

    • 3.5. Transition Education e651

    • 3.6. Exercise and Sports e652

    • 3.7. Mental Health and Neurodevelopmental Issues e652

    • 3.8. Endocarditis Prevention e652

    • 3.9. Concomitant Syndromes e652

    • 3.10. Noncardiac Medical Issues e653

    • 3.11. Noncardiac Surgery e653

    • 3.12. Pregnancy, Reproduction, and Sexual Health e653

      • 3.12.1. Pregnancy e653

      • 3.12.2. Contraception e654

    • 3.13. Heart Failure and Transplant e654

      • 3.13.1. Heart Failure e654

      • 3.13.2. Heart Transplant e654

    • 3.14. Palliative Care e654

    • 3.15. Cyanosis e654

    • 3.16. Pharmacological Therapy for ACHD e654

  • 4. Specific Lesions e655

    • 4.1. Shunt Lesions e655

      • 4.1.1. Atrial Septal Defect e655

      • 4.1.2. Anomalous Pulmonary Venous Connections e657

      • 4.1.3. Ventricular Septal Defect e657

      • 4.1.4. Atrioventricular Septal Defect e658

      • 4.1.5. Patent Ductus Arteriosus e659

    • 4.2. Left-Sided Obstructive Lesions e660

      • 4.2.1. Cor Triatriatum e660

      • 4.2.2. Congenital Mitral Stenosis e660

      • 4.2.3. Subaortic Stenosis e660

      • 4.2.4. Congenital Valvular Aortic Stenosis e661

        • 4.2.4.1. Turner Syndrome e661

      • 4.2.5. Supravalvular Aortic Stenosis e661

      • 4.2.6. Coarctation of the Aorta e662

    • 4.3. Right-Sided Lesions e663

      • 4.3.1. Valvular Pulmonary Stenosis e663

        • 4.3.1.1. Isolated PR After Repair of PS e664

      • 4.3.2. Branch and Peripheral Pulmonary Stenosis e665

      • 4.3.3. Double-Chambered Right Ventricle e665

      • 4.3.4. Ebstein Anomaly e666

      • 4.3.5. Tetralogy of Fallot e667

      • 4.3.6. Right Ventricle to Pulmonary Artery Conduit e669

    • 4.4. Complex Lesions e669

      • 4.4.1. Transposition of the Great Arteries e669

        • 4.4.1.1. Transposition of the Great Arteries With Atrial Switch e669

        • 4.4.1.2. Transposition of the Great Arteries With Arterial Switch e670

        • 4.4.1.3. Congenitally Corrected Transposition of the Great Arteries e671

      • 4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle) e672

      • 4.4.3. Double Outlet Right Ventricle e673

      • 4.4.4. Severe PAH and Eisenmenger Syndrome e673

        • 4.4.4.1. Severe PAH e673

        • 4.4.4.2. Eisenmenger Syndrome e674

      • 4.4.5. Coronary Anomalies e674

        • 4.4.5.1. Anomalous Coronary Artery Evaluation e674

        • 4.4.5.2. Anomalous Aortic Origin of Coronary Artery e676

        • 4.4.5.3. Anomalous Coronary Artery Arising From the PA e676

  • 5. Evidence Gaps and Future Directions e676

  • References e678

  • Appendix 1 Author Relationships With Industry and Other Entities (Relevant) e693

  • Appendix 2 Reviewer Relationships With Industry and Other Entities (Comprehensive) e695

Preamble

Since 1980, the American College of Cardiology (ACC) and American Heart Association (AHA) have translated scientific evidence into clinical practice guidelines (guidelines) with recommendations to improve cardiovascular health. These guidelines, which are based on systematic methods to evaluate and classify evidence, provide a cornerstone for quality cardiovascular care. The ACC and AHA sponsor the development and publication of guidelines without commercial support, and members of each organization volunteer their time to the writing and review efforts. Guidelines are official policy of the ACC and AHA.

Intended Use

Practice guidelines provide recommendations applicable to patients with or at risk of developing cardiovascular disease. The focus is on medical practice in the United States, but guidelines developed in collaboration with other organizations can have a global impact. Although guidelines may be used to inform regulatory or payer decisions, they are intended to improve patients’ quality of care and align with patients’ interests. Guidelines are intended to define practices meeting the needs of patients in most, but not all, circumstances and should not replace clinical judgment.

Clinical Implementation

Management in accordance with guideline recommendations is effective only when followed by both practitioners and patients. Adherence to recommendations can be enhanced by shared decision making between clinicians and patients, with patient engagement in selecting interventions on the basis of individual values, preferences, and associated conditions and comorbidities.

Methodology and Modernization

The ACC/AHA Task Force on Clinical Practice Guidelines (Task Force) continuously reviews, updates, and modifies guideline methodology on the basis of published standards from organizations, including the Institute of Medicine,P-1, P-2 and on the basis of internal reevaluation. Similarly, the presentation and delivery of guidelines are reevaluated and modified on the basis of evolving technologies and other factors to facilitate optimal dissemination of information to healthcare professionals at the point of care.

Toward this goal, this guideline continues the introduction of an evolved format of presenting guideline recommendations and associated text called the “modular knowledge chunk format.” Each modular “chunk” includes a table of related recommendations, a brief synopsis, recommendation-specific supportive text, and when appropriate, flow diagrams or additional tables. References are provided at the end of the document in their respective sections. Additionally, this format will facilitate seamless updating of guidelines with focused updates as new evidence is published, as well as content tagging for rapid electronic retrieval of related recommendations on a topic of interest. This evolved approach format was instituted when this guideline was near completion; therefore, the present document represents a transitional format that best suits the text as written. Future guidelines will fully implement this format, including provisions for limiting the amount of text in a guideline.

Recognizing the importance of cost–value considerations in certain guidelines, when appropriate and feasible, an analysis of the value of a drug, device, or intervention may be performed in accordance with the ACC/AHA methodology.P-3

To ensure that guideline recommendations remain current, new data are reviewed on an ongoing basis, with full guideline revisions commissioned in approximately 6-year cycles. Publication of new, potentially practice-changing study results that are relevant to an existing or new drug, device, or management strategy will prompt evaluation by the Task Force, in consultation with the relevant guideline writing committee, to determine whether a focused update should be commissioned. For additional information and policies regarding guideline development, we encourage readers to consult the ACC/AHA guideline methodology manualP-4 and other methodology articles.P-5–P-8

Selection of Writing Committee Members

The Task Force strives to avoid bias by selecting experts from a broad array of backgrounds. Writing committee members represent different geographic regions, sexes, ethnicities, races, intellectual perspectives/biases, and scopes of clinical practice. The Task Force may also invite organizations and professional societies with related interests and expertise to participate as partners, collaborators, or endorsers.

Relationships With Industry and Other Entities

The ACC and AHA have rigorous policies and methods to ensure that guidelines are developed without bias or improper influence. The complete relationships with industry and other entities (RWI) policy can be found online. Appendix 1 of the present document lists writing committee members’ relevant RWI. For the purposes of full transparency, writing committee members’ comprehensive disclosure information is available online. Comprehensive disclosure information for the Task Force is also available online.

Evidence Review and Evidence Review Committees

In developing recommendations, the writing committee uses evidence-based methodologies that are based on all available data.P-4–P-7 Literature searches focus on randomized controlled trials but also include registries, nonrandomized comparative and descriptive studies, case series, cohort studies, systematic reviews, and expert opinion. Only key references are cited.

An independent evidence review committee (ERC) is commissioned when there are 1 or more questions deemed of utmost clinical importance that merit formal systematic review. The systematic review will determine which patients are most likely to benefit from a drug, device, or treatment strategy and to what degree. Criteria for commissioning an ERC and formal systematic review include: a) the absence of a current authoritative systematic review, b) the feasibility of defining the benefit and risk in a time frame consistent with the writing of a guideline, c) the relevance to a substantial number of patients, and d) the likelihood that the findings can be translated into actionable recommendations. ERC members may include methodologists, epidemiologists, healthcare providers, and biostatisticians. The recommendations developed by the writing committee on the basis of the systematic review are marked with “SR.”

Guideline-Directed Management and Therapy

The term guideline-directed management and therapy (GDMT) encompasses clinical evaluation, diagnostic testing, and pharmacological and procedural treatments. For these and all recommended drug treatment regimens, the reader should confirm the dosage by reviewing product insert material and evaluate the treatment regimen for contraindications and interactions. The recommendations are limited to drugs, devices, and treatments approved for clinical use in the United States.

Class of Recommendation and Level of Evidence

The Class of Recommendation (COR) indicates the strength of the recommendation, encompassing the estimated magnitude and certainty of benefit in proportion to risk. The Level of Evidence (LOE) rates the quality of scientific evidence that supports the intervention on the basis of the type, quantity, and consistency of data from clinical trials and other sources (Table 1).P-4–P-6

Table 1. Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care* (Updated August 2015)

Table 1.

The reader is encouraged to consult the full-text guidelineP-9 for additional guidance and details about adult congenital heart disease because this executive summary contains mainly the recommendations.

Glenn N. Levine, MD, FACC, FAHA

Chair, ACC/AHA Task Force on Clinical Practice Guidelines

1. Introduction

1.1. Methodology and Evidence Review

The recommendations listed in this guideline are, whenever possible, evidence-based. An initial extensive evidence review, which included literature derived from research involving human subjects, published in English, and indexed in MEDLINE (through PubMed), EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline, was conducted from April 2014 to November 2014. Key search words included but were not limited to the following: adult congenital heart disease, anesthesia, aortic aneurysm, aortic stenosis, atrial septal defect, arterial switch operation, bradycardia, bicuspid aortic valve, cardiac catheterization, cardiac imaging, cardiovascular magnetic resonance, cardiac reoperation, cardiovascular surgery, chest x-ray, cirrhosis, coarctation of the aorta, congenital heart defects, congenitally corrected transposition of the great arteries, contraception, coronary artery abnormalities, cyanotic congenital heart disease, dextro-transposition of the great arteries, double inlet left ventricle, Ebstein anomaly, echocardiography, Eisenmenger syndrome, electrocardiogram, endocarditis, exercise test, Fontan, heart catheterization, heart defect, heart failure, infertility, l-transposition of the great arteries, medical therapy, myocardial infarction, noncardiac surgery, patent ductus arteriosus, perioperative care, physical activity, postoperative complications, pregnancy, preoperative assessment, psychosocial, pulmonary arterial hypertension, hypoplastic left heart syndrome, pulmonary regurgitation, pulmonary stenosis, pulmonary valve replacement, right heart obstruction, right ventricle to pulmonary artery conduit, single ventricle, supravalvular pulmonary stenosis, surgical therapy, tachyarrhythmia, tachycardia, tetralogy of Fallot, transplantation, tricuspid atresia, Turner syndrome, and ventricular septal defect. Additional relevant studies published through January 2018, during the guideline writing process, were also considered by the writing committee, and added to the evidence tables when appropriate. The final evidence tables, included in the Online Data Supplement, summarize the evidence used by the writing committee to formulate recommendations. References selected and published in this document are representative and not all-inclusive.

As noted in the preamble, an independent ERC was commissioned to perform a formal systematic review of critical clinical questions related to adult congenital heart disease (ACHD), the results of which were considered by the writing committee for incorporation into this guideline. Concurrent with this process, writing committee members evaluated study data relevant to the rest of the guideline. The findings of the ERC and the writing committee members were formally presented and discussed, and then recommendations were developed. The systematic review reports on “Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease”S1.1-1 and “Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease”S1.1-2 are published in conjunction with this guideline.

1.2. Organization of the Writing Committee

The writing committee consisted of pediatric and adult congenital cardiologists, interventional cardiologists, electrophysiologists, surgeons, and an advance practice nurse. The writing committee included representatives from the ACC, AHA, and American Association for Thoracic Surgery (AATS), American Society of Echocardiography (ASE), Heart Rhythm Society (HRS), International Society for Adult Congenital Heart Disease (ISACHD), Society for Cardiovascular Angiography and Interventions (SCAI), and the Society of Thoracic Surgeons (STS).

1.3. Document Review and Approval

This document was reviewed by 3 official reviewers each nominated by the ACC and AHA, and 1 to 2 reviewers each from the AATS, ASE, HRS, ISACHD, SCAI, STS; and 32 individual content reviewers. Reviewers' RWI information was distributed to the writing committee and is published in this document (Appendix 2).

This document was approved for publication by the governing bodies of the ACC and the AHA and endorsed by the AATS, ASE, HRS, ISACHD, SCAI, and STS.

1.4. Scope of the Guideline

The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease,”S1.4-1 which was the first US guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease,”S1.4-2 particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (<18 years of age) with CHD or adults with acquired VHD, heart failure (HF), or other cardiovascular disease.

The prevalence of ACHD is growing because of the success of pediatric cardiology and congenital cardiac surgery in diagnosing and treating congenital heart defects in children. Improved survival to adulthood is most striking for those with the most severe disease, with survival to age 18 years now expected for 90% of children diagnosed with severe CHD.S1.4-3–S1.4-5 Patients with ACHD are a heterogeneous population, both in underlying anatomy and physiology, as well as surgical repair or palliation. Consequently, although the prevalence of ACHD is increasing, the population of patients with a given congenital abnormality or specific repair may be relatively small.S1.4-3,S1.4-6–S1.4-8

Patients with CHD are not cured of their disease after successful treatment in childhood. Almost all patients with ACHD will have sequelae of either their native CHD or its surgical repair or palliation, although these sequelae can take decades to manifest. The heterogeneity of the population and the long symptom-free intervals constrain the ability to generate data applicable across the population of ACHD or to adults with specific lesions or repairs. Despite the difficulty in studying ACHD populations, there is a growing body of high-quality data in these patients to guide the care of this relatively “new” population, and whenever feasible, these data were used to develop recommendations. Recommendations are made based on the available data; however, when important clinical issues lacked data, first principles, extrapolation from data in other populations, and expert consensus are used to guide care. Patients with ACHD may have concomitant disease to which other existing guidelines apply, such as coronary artery disease, HF, and arrhythmias. The data from acquired heart disease populations may apply to some patients with ACHD, and those circumstances are acknowledged in these recommendations and referenced accordingly.

Patients with ACHD who are cared for in ACHD centers have better outcomes than those cared for in centers without ACHD expertise,S1.4-9 and this need for specialized care is noted throughout the guideline. These recommendations are intended to provide guidance to a wide variety of providers caring for patients with ACHD, including general, pediatric, and ACHD cardiologists, as well as surgeons, primary care providers, and other healthcare providers.

In developing the 2018 ACHD guideline, the writing committee reviewed previously published guidelines and related scientific statements. Table 2 contains a list of publications and scientific statements deemed pertinent to this writing effort; it is intended for use as a resource and does not repeat existing guideline recommendations.

Table 2. Associated Guidelines and Statements

TitleOrganizationPublication Year (Reference)
Guidelines
 SyncopeACC/AHA/HRS2017S1.4-10
 Supraventricular tachycardiaACC/AHA/HRS2015S1.4-11
 Cardiopulmonary resuscitation and emergency cardiovascular care—Part 8: postcardiac arrest careAHA2015S1.4-12
 Non-ST-elevation acute coronary syndromesAHA/ACC2014S1.4-13
 Perioperative cardiovascular evaluation and noncardiac surgeryACC/AHA2014S1.4-14
 Atrial fibrillationAHA/ACC/HRS2014S1.4-15
 Stable ischemic heart diseaseACC/AHA/ACP/AATS/PCNA/SCAI/STS2014,S1.4-16
2012S1.4-17
 Assessment of cardiovascular riskACC/AHA2014S1.4-18
 Blood cholesterol to reduce atherosclerotic cardiovascular risk in adultsACC/AHA2014S1.4-19
 Overweight and obesity in adultsAHA/ACC/TOS2014S1.4-20
 Lifestyle management to reduce cardiovascular riskAHA/ACC2014S1.4-21
 Valvular heart diseaseAHA/ACC2017S1.4-22
 High blood pressure in adultsACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA2017S1.4-23
 Aortic valve and ascending aortaSTS2013S1.4-24
 ST-elevation myocardial infarctionACC/AHA2013S1.4-25
 Heart failureACC/AHA/HFSA2017S1.4-26
 Device-based therapy for cardiac rhythm abnormalitiesACC/AHA/HRS2012S1.4-27
 Coronary artery bypass graft surgeryACC/AHA2011S1.4-28
 Percutaneous coronary interventionACC/AHA/SCAI2011S1.4-29
 Secondary prevention and risk reduction therapyAHA/ACC2011S1.4-30
 Cardiovascular disease in womenAHA/ACC2011S1.4-31
 Grown-up congenital heart diseaseESC2010S1.4-32
 Thoracic aortic diseaseACC/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM2010S1.4-33
 Adult congenital heart diseaseCCS2010S1.4-34
 Infective endocarditisESC2009S1.4-35
Scientific statements
 Imaging for patients with transposition of the great arteriesASE2016S1.4-36
 Cardiac chamber quantification by echocardiographyASE2015S1.4-37
 Consensus on arrhythmia management in ACHDPACES/HRS2014S1.4-38
 Imaging for patients with repaired tetralogy of FallotASE2014S1.4-39
 Thoracic aortic diseaseCCS2014S1.4-40
 Promotion of physical activity for children and adults with CHDAHA2013S1.4-41
 Neurodevelopmental outcomes in children with CHDAHA2012S1.4-42
 Pregnancy in women with heart diseaseESC2011S1.4-43
 Transition to adulthood for adolescents with CHDAHA2011S1.4-44
 Pulmonary hypertensionACC/AHA2009S1.4-45
 Prevention of infective endocarditisAHA2007S1.4-46

AATS indicates American Association for Thoracic Surgery; ABC, Association of Black Cardiologists; ACC, American College of Cardiology; ACHD, adult congenital heart disease; ACP, American College of Physicians; ACPM, American College of Preventive Medicine; ACR, American College of Radiology; AGS, American Geriatrics Society; AHA, American Heart Association; APhA, American Pharmacists Association; ASA, American Stroke Association; ASE, American Society of Echocardiography; ASH, American Society of Hypertension; ASPC, American Society of Preventive Cardiology; CCS, Canadian Cardiovascular Society; CHD, congenital heart disease; ESC, European Society of Cardiology; HFSA, Heart Failure Society of America; HRS, Heart Rhythm Society; NMA, National Medical Association; PACES, Pediatric and Congenital Electrophysiology Society; PCNA, Preventive Cardiovascular Nurses Association; SCA, Society of Cardiovascular Anesthesiologists; SCAI, Society for Cardiovascular Angiography and Interventions; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society for Vascular Medicine; and TOS, The Obesity Society.

1.5. Abbreviations

AbbreviationMeaning/Phrase
AAOCAanomalous aortic origin of the coronary artery
ACHDadult congenital heart disease
APanatomic and physiological
ARaortic regurgitation
ASDatrial septal defect
AVSDatrioventricular septal defect
BAVbicuspid aortic valve
CCTcardiac computed tomography
CCTGAcongenitally corrected transposition of the great arteries
CHDcongenital heart disease
CMRcardiovascular magnetic resonance
CoAcoarctation of the aorta
CPETcardiopulmonary exercise test
CTcomputed tomography
CTAcomputed tomography angiography
d-TGAdextro-transposition of the great arteries
ECGelectrocardiogram
ERCEvidence Review Committee
GDMTguideline-directed management and therapy
HFheart failure
ICDimplantable cardioverter-defibrillator
IEinfective endocarditis
LVleft ventricular
LVOTleft ventricular outflow tract
PApulmonary artery
PAHpulmonary arterial hypertension
PDApatent ductus arteriosus
PRpulmonary regurgitation
PSpulmonary stenosis
Qp:Qspulmonary–systemic blood flow ratio
RVright ventricular
RVOTright ventricular outflow tract
SCDsudden cardiac death
TEEtransesophageal echocardiography
TGAtransposition of the great arteries
TOFtetralogy of Fallot
TRtricuspid regurgitation
TTEtransthoracic echocardiography
VHDvalvular heart disease
VSDventricular septal defect

2. Background and Pathophysiology

2.1. Anatomic and Physiological Terms

The International Society for Nomenclature of Pediatric and Congenital Heart Disease (also known as the Nomenclature Working Group) defined, codified, mapped, and archived examples of nomenclatures and developed standards for terminology.S2.1-1–S2.1-5 The International Paediatric and Congenital Cardiac Code (IPCCC) nomenclature for anatomic lesions and repairs is used in this guideline (http://ipccc.net).S2.1-6

2.2. Severity of ACHD

In a patient with CHD, severity of disease is determined by native anatomy, surgical repair, and current physiology. Prior documents, including the 2008 ACHD guideline,S2.2-1 relied primarily on anatomic classifications to rank severity of disease. However, patients with the same underlying anatomy may have very different repairs and experienced variable physiological consequences of those repairs. For example, a patient with tetralogy of Fallot (TOF) after a valve-sparing primary repair may have excellent biventricular function with normal exercise capacity and no arrhythmias, whereas another patient of the same age with TOF may have had palliative shunting followed by a transannular patch repair resulting in severe pulmonary regurgitation (PR) with right ventricular (RV) enlargement, biventricular dysfunction, and ventricular tachycardia. To categorize disease severity in CHD in a more comprehensive way, the writing committee developed an ACHD Anatomic and Physiological (AP) classification system (Tables 3 and 4) that incorporates the previously described CHD anatomic variables as well as physiological variables, many of which have prognostic value in patients with ACHD.

Table 3. Physiological Variables as Used in ACHD AP Classification

VariableDescription
AortopathyAortic enlargement is common in some types of CHD and after some repairs. Aortic enlargement may be progressive over a lifetime. There is no universally accepted threshold for repair, nor is the role of indexing to body size clearly defined in adults, as is done in pediatric populations. For purposes of categorization and timing of follow-up imagingS2.2-2–S2.2-4:
 Mild aortic enlargement is defined as maximum diameter 3.5–3.9 cm
 Moderate aortic enlargement is defined as maximum diameter 4.0–4.9 cm
 Severe aortic enlargement is defined as maximum diameter ≥5.0 cm
ArrhythmiaArrhythmias are very common in patients with ACHD and may be both the cause and consequence of deteriorating hemodynamics, valvular dysfunction, or ventricular dysfunction. Arrhythmias are associated with symptoms, outcomes, and prognosis,S2.2-5–S2.2-8 thus are categorized based on presence and response to treatment.
 No arrhythmia: No documented clinically relevant atrial or ventricular tachyarrhythmias
Arrhythmia not requiring treatment: Bradyarrhythmia, atrial or ventricular tachyarrhythmia not requiring antiarrhythmic therapy, cardioversion, or ablation
 Arrhythmia controlled with therapy:
  Bradyarrhythmia requiring pacemaker implantation
  Atrial or ventricular tachyarrhythmia requiring antiarrhythmic therapy, cardioversion, or ablation
  AF and controlled ventricular response
  Patients with an ICD
 Refractory arrhythmias:
  Atrial or ventricular tachyarrhythmia currently unresponsive to or refractory to antiarrhythmic therapy or ablation
Concomitant VHDSeverity defined according to the 2014 VHD guideline.S2.2-2
 Mild VHD
 Moderate VHD
 Severe VHD
End-organ dysfunctionClinical and/or laboratory evidence of end-organ dysfunctionS2.2-9–S2.2-11 including:
 Renal (kidney)
 Hepatic (liver)
 Pulmonary (lung)
Exercise capacityPatients with ACHD are often asymptomatic notwithstanding exercise limitations demonstrated as diminished exercise capacity when evaluated objectively.S2.2-12–S2.2-14 Thus, assessment of both subjective and objective exercise capacity is important (see NYHA classification system below). Exercise capacity is associated with prognosis.S2.2-15–S2.2-17
 Abnormal objective cardiac limitation to exercise is defined as an exercise maximum ventilatory equivalent of oxygen below the range expected for the specific CHD anatomic diagnosis.S2.2-18
 Expected norms for CPET values should take into account age, sex, and underlying congenital diagnosis. Published studies with institution-specific norms can be used as guides, bearing in mind variability among institutional norms and ranges.
Hypoxemia/hypoxia/cyanosisSee Section 3.16. for detailed definition of cyanosis.
 Hypoxemia is defined as oxygen saturation measured by pulse oximetry at rest ≤90%.
 Severe hypoxemia is defined as oxygen saturation at rest <85%.
 In patients with normal or high hemoglobin concentrations, severe hypoxemia will be associated with visible cyanosis (which requires ≥5 g/L desaturated hemoglobin to be appreciated).
 The terms cyanosis and hypoxemia (or hypoxia) are sometimes used interchangeably. Such interchangeability would not apply; however, in the presence of anemia, severe hypoxemia can be present without visible cyanosis.
NYHA functional classification systemS2.2-19ClassFunctional Capacity
IPatients with cardiac disease but resulting in no limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain.
IIPatients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in fatigue, palpitation, dyspnea, or anginal pain.
IIIPatients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes fatigue, palpitation, dyspnea, or anginal pain.
IVPatients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of HF or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort increases.
Pulmonary hypertensionPulmonary hypertension is a broad term that encompasses pulmonary arterial hypertension, which is pulmonary hypertension with increased pulmonary vascular resistance. This document defines PH and PAH as they are used in the field of pulmonary hypertension.
Pulmonary hypertension is defined as:
 Mean PA pressure by right heart catheterization ≥25 mm Hg.
PAH is defined as:
 Mean PA pressure by right heart catheterization ≥25 mm Hg and a pulmonary capillary wedge pressure ≤15 mm Hg and pulmonary vascular resistance ≥3 Wood unitsS2.2-20
Shunt (hemodynamically significant shunt)An intracardiac shunt is hemodynamically significant if:
There is evidence of chamber enlargement distal to the shunt
And/or evidence of sustained Qp:Qs ≥1.5:1
An intracardiac shunt not meeting these criteria would be described as small or trivial
Venous and arterial stenosisAortic recoarctation after CoA repair
Supravalvular aortic obstruction
Venous baffle obstruction
Supravalvular pulmonary stenosis
Branch PA stenosis
Stenosis of cavopulmonary connection
Pulmonary vein stenosis

ACHD indicates adult congenital heart disease; AF, atrial fibrillation; AP, anatomic and physiological; CHD, congenital heart disease; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise test; HF, heart failure; ICD, implantable cardioverter-defibrillator; NYHA, New York Heart Association; PA, pulmonary artery; PAH, pulmonary arterial hypertension; Qp:Qs, pulmonary–systemic blood flow ratio; and VHD, valvular heart disease.

Table 4. ACHD AP Classification(CHD Anatomy + Physiological Stage = ACHD AP Classification)

CHD Anatomy*
I: Simple
 Native disease
  Isolated small ASD
  Isolated small VSD
  Mild isolated pulmonic stenosis
 Repaired conditions
  Previously ligated or occluded ductus arteriosus
Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber enlargement
  Repaired VSD without significant residual shunt or chamber enlargement
II: Moderate Complexity
 Repaired or unrepaired conditions
  Aorto-left ventricular fistula
  Anomalous pulmonary venous connection, partial or total
  Anomalous coronary artery arising from the pulmonary artery
  Anomalous aortic origin of a coronary artery from the opposite sinus
  AVSD (partial or complete, including primum ASD)
  Congenital aortic valve disease
  Congenital mitral valve disease
  Coarctation of the aorta
  Ebstein anomaly (disease spectrum includes mild, moderate, and severe variations)
  Infundibular right ventricular outflow obstruction
  Ostium primum ASD
  Moderate and large unrepaired secundum ASD
  Moderate and large persistently patent ductus arteriosus
  Pulmonary valve regurgitation (moderate or greater)
  Pulmonary valve stenosis (moderate or greater)
  Peripheral pulmonary stenosis
  Sinus of Valsalva fistula/aneurysm
  Sinus venosus defect
  Subvalvar aortic stenosis (excluding HCM; HCM not addressed in these guidelines)
  Supravalvar aortic stenosis
  Straddling atrioventricular valve
  Repaired tetralogy of Fallot
  VSD with associated abnormality and/or moderate or greater shunt
III: Great Complexity (or Complex)
 Cyanotic congenital heart defect (unrepaired or palliated, all forms)
 Double-outlet ventricle
 Fontan procedure
 Interrupted aortic arch
 Mitral atresia
 Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left heart, any other anatomic abnormality with a functionally single ventricle)
 Pulmonary atresia (all forms)
 TGA (classic or d-TGA; CCTGA or l-TGA)
 Truncus arteriosus
 Other abnormalities of atrioventricular and ventriculoarterial connection (ie, crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)
Physiological Stage
 A
  NYHA FC I symptoms
  No hemodynamic or anatomic sequelae
  No arrhythmias
  Normal exercise capacity
  Normal renal/hepatic/pulmonary function
 B
  NYHA FC II symptoms
  Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular enlargement, mild ventricular dysfunction)
  Mild valvular disease
  Trivial or small shunt (not hemodynamically significant)
  Arrhythmia not requiring treatment
  Abnormal objective cardiac limitation to exercise
 C
  NYHA FC III symptoms
  Significant (moderate or greater) valvular disease; moderate or greater ventricular dysfunction (systemic, pulmonic, or both)
  Moderate aortic enlargement
  Venous or arterial stenosis
  Mild or moderate hypoxemia/cyanosis
  Hemodynamically significant shunt
  Arrhythmias controlled with treatment
  Pulmonary hypertension (less than severe)
  End-organ dysfunction responsive to therapy
 D
  NYHA FC IV symptoms
  Severe aortic enlargement
  Arrhythmias refractory to treatment
  Severe hypoxemia (almost always associated with cyanosis)
  Severe pulmonary hypertension
  Eisenmenger syndrome
  Refractory end-organ dysfunction

*This list is not meant to be comprehensive; other conditions may be important in individual patients.

ACHD indicates adult congenital heart disease; AP, anatomic and physiological; ASD, atrial septal defect; AVSD, atrioventricular septal defect; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; d-TGA, dextro-transposition of the great arteries; FC, functional class; HCM, hypertrophic cardiomyopathy; l-TGA, levo-transposition of the great arteries; NYHA, New York Heart Association; TGA, transposition of the great arteries; and VSD, ventricular septal defect.

2.3. The ACHD AP Classification

The ACHD AP classification (Tables 3 and 4), newly elaborated in this guideline, is intended to capture the complexity of ACHD anatomy and physiology, which are not always correlated. Certain anatomic abnormalities of clinical importance are shared across diagnoses (eg, aortic enlargement), which may be found in patients with BAV, coarctation of the aorta (CoA), transposition of the great arteries, and TOF, amongst others. In every patient, anatomic and physiological variables should be considered. In using Tables 3 and 4, a patient should be classified based on the “highest” relevant anatomic or physiological feature. For example, a normotensive patient with repaired CoA, normal exercise capacity, and normal end-organ function would be ACHD AP classification IIA, whereas an otherwise similar patient with ascending aortic diameter of 4.0 cm would be ACHD AP classification IIB, and if moderate aortic stenosis were also present, the ACHD AP classification would be IIC.

Patients with ACHD may have baseline exercise limitations, cyanosis, end-organ dysfunction, or other clinically important comorbidities related to their CHD. They are also at risk of HF, arrhythmias, sudden cardiac death (SCD), and development or progression of cardiac symptoms such as dyspnea, chest pain, and exercise intolerance. Concomitant valvular disease or aortic pathology may be present. There are growing data regarding the prognostic implications of these variables in patients with ACHD, but not the abundance of data available for patients with acquired heart disease.S2.3-1–S2.3-16

The variables forming part of the ACHD AP classification (Table 3) were selected because data exist suggesting their importance in prognosis, management, or quality of life. As new data become available, we expect changes in the relative weights attributed to the components of the ACHD AP classification and perhaps new components, resulting in a scheme that ever more precisely tracks overall severity of disease and need for more or less intensive follow-up and management.

Similar to the New York Heart Association (NYHA) classification of functional status, patients may move from one ACHD AP classification to another over time. If clinical status worsens, the classification will change to a higher severity group, but improvement in status, for example after an intervention such as valve replacement or control of arrhythmia, can result in change to a lower severity classification. Such movement among classes is unlike the AHA HF A to D classification,S2.3-17 in which patients move in only one direction. This ACHD AP classification is used throughout this document, particularly when considering follow-up visits and need for testing. As the ACHD AP classification worsens because of changes in physiology (eg, development of arrhythmias, HF, end-organ disease), the nature and frequency of recommended follow-up visits and testing will also change, adapting to the patient's changing circumstance instead of depending solely on a description of anatomic disease, which may not adequately discriminate physiological changes that alter severity over time.

Some patients with ACHD may have substantial acquired comorbidities unrelated to CHD, and as a consequence, their follow-up strategies might be more appropriately be based on other existing guidelines for acquired heart disease. For example, an 80-year-old patient who has a small atrial septal defect (ASD), but whose symptoms are related to diastolic HF, chronic kidney disease caused by hypertension and diabetes mellitus, and moderate aortic stenosis is well-suited to be followed according to existing guidelines for those diseases, rather than according to the ACHD AP classification for the ASD. Nevertheless, the added hemodynamic complexity brought by the ASD must be kept in mind.

Throughout this document, the ACHD AP classification is used to help guide resource utilization, including ACHD consultation and routine diagnostic studies.

3. General Principles

See Online Data Supplements 1 and 2 for additional data supporting this section.

3.1. ACHD Program

Patients with complex CHD have generally better outcomes when cared for in an integrated, collaborative, and multidisciplinary program.S3.1-1 Many medical issues in patients with ACHD involve cardiac sequelae, and the diagnosis and management may require cardiac anesthesiologists, electrophysiologists, and interventional cardiologists; imaging services such as cardiovascular magnetic resonance (CMR)/cardiac computed tomography (CCT); and pulmonary hypertension services with expertise in ACHD (Table 5). Appropriate specialty care must be available to address pregnancy, acquired cardiovascular disease, and acute noncardiac illness complicating CHD, management of which is frequently more complicated in patients with ACHD.

Table 5. Key Personnel and Services Recommended for ACHD Programs

Personnel
 ACHD board-eligible/board-certified cardiologists
 Congenital cardiac surgeons
 Nurses/physician assistants/nurse practitioners
 Cardiac anesthesiologists with CHD training/expertise
 Multidisciplinary teams:
  High-risk obstetrics
  Pulmonary hypertension
  HF/transplant
  Genetics
  Hepatology
  Cardiac pathology
  Rehabilitation services
  Social services
  Psychological services
  Financial counselors
Services
 Echocardiography, including TEE and intraoperative TEE*
 CHD diagnostic and interventional catheterization*
 CHD electrophysiology/pacing/ICD implantation*:
  Exercise testing
  Echocardiographic
  Radionuclide
  Cardiopulmonary
 Cardiac imaging/radiology*:
  CMR
  CCT
  Nuclear medicine
 Information technology:
  Data collection
  Database support
  Quality assessment review/protocols

*These modalities must be supervised/performed and interpreted by clinicians with expertise and/or training in CHD.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CHD, congenital heart disease; CMR, cardiovascular magnetic resonance; HF, heart failure; ICD, implantable cardioverter-defibrillator; and TEE, transesophageal echocardiography.

Although individual providers may be community-based affiliates, ACHD programs are inpatient, outpatient, and hospital-based with staffing and expertise available on-site or accessible when needed (Table 5).

3.2. Access to Care

Recommendation for Access to Care

Referenced studies that support the recommendation are summarized in Online Data Supplement 3.

3.3. Delivery of Care

Recommendations for Delivery of Care

Referenced studies that support recommendations are summarized in Online Data Supplements 3, 4, and 5.

Table 6 addresses delivery of care where circumstances of ACHD expertise may improve patient outcomes.

Table 6. Delivery of Care: Circumstances Where ACHD Expertise May Improve Outcomes

CircumstancePossible SolutionRationaleExample
Care of patients in the lowest ACHD AP classification (IA)*1. Face-to-face consultation with an ACHD cardiologist.1. Patients in ACHD AP classification IA* are likely to be asymptomatic and not require frequent routine congenital cardiac care.Patients with small VSDs are thought to have excellent long-term survival, although complications (double-chamber RV, IE, aortic valve prolapse and aortic regurgitation) may manifest in adulthood; consequently, patients with small VSDs warrant lifelong follow-up.S3.3-4
2. Collaborative care planning between an ACHD patient’s general cardiologist or primary care provider and an ACHD cardiologist.2. The very long-term outcomes of patients with ACHD AP classification IA* lesions have not been well described, although available data suggest that patients with simple CHD have higher cardiac mortality in long-term follow-up than age-matched controls.S3.3-3
3. Consultation with an ACHD cardiologist should help to accurately assess the patient’s ACHD AP class, provide information regarding potential long-term outcomes, and reinforce signs and symptoms that should prompt further evaluation.
Cardiac imaging of patients with ACHDImaging studies should be performed and interpreted by individuals with expertise in CHD imaging.1. The complexity and variability of lesions, repairs, and sequelae in CHD constrain the use of standard protocols and sequences and often require modification of plans during acquisition of images, as well as specialized skills in interpretation. Thus, CHD expertise is helpful for optimal quality and interpretation of cardiac imaging studies.Although imaging of a patient with TOF may seem straightforward because many have familiar chamber and great vessel relationships, there are nuances to echocardiographic imaging of RV size and function, PR severity, and/or location of right ventricular outflow tract obstruction that affect clinical care and are thus best carried out by sonographers and echocardiographers with appropriate expertise. Similarly, expertise in congenital CMR is important in evaluating patients with TOF, as RV volumes and function are key components in evaluation for timing of pulmonary valve replacement.S3.3-7,S3.3-8
2. Use of a multimodality cardiac imaging approach can be used for patients with ACHD, accounting for patient-specific considerations, strengths and weaknesses of each modality, institutional resources, and expertise.
3. ACHD programs need a dedicated CMR service, and CMR expertise is integral to an ACHD program, as is expertise in ACHD CCT.S3.3-5,S3.3-6
Electrophysiological care of patients with ACHDPerform procedures in electrophysiology laboratories equipped for 3D mapping and ablation and involve specialists experienced in the management of arrhythmias in patients with ACHD.Examples of diagnostic questions best answered by electrophysiological study: a) evaluation of the conduction system in cases of suspected postsurgical conduction abnormalities b) evaluation of syncope c) diagnosis of the mechanism of supraventricular tachycardia or wide complex tachycardia d) programmed ventricular stimulation particularly in patients following repair of TOF and its variants (Section 4.4.1.) as well as preoperative assessment of arrhythmia substrates that may be amenable to operative intervention, such as an atrial maze procedure for atrial arrhythmias. The latter procedure is commonly used at the time of conversion of atriopulmonary connection Fontan, and may also be useful in other forms of repaired CHD with postoperative atrial arrhythmias such as TOF.Bradyarrhythmia and tachyarrhythmias are common in TGA with atrial switch patients and may seem “straightforward,” but the altered anatomy adds complexity to the procedures and emphasizes the need for specialized equipment and expertise to ensure the best chance for procedural success. For example: 1) pacemaker placement in a patient with TGA with atrial switch can be challenging because of the altered atrial anatomy and interatrial baffle that will necessitate placement of an atrial lead in the anatomic left atrium, often scarred such that tissue amenable to pacing is difficult to find; and 2) atrial flutter is a common arrhythmia in TGA with atrial switch, but the flutter circuit may be on the systemic side of the interatrial baffle and thus may require baffle puncture or retrograde approach to effectively ablate the circuit.
Diagnostic and interventional cardiac procedures, including electrophysiology procedures1. Perform procedure in a hospital with cardiologists, anesthesiologists, surgeons, and other providers with expertise in the management of patients with ACHD.1. Patients with ACHD often have complex underlying cardiac anatomy and physiology.In patients with CHD, the presence of anatomic and physiological complexity from the specific defect or surgical palliation, may change the overall care plan and procedural decision-making. Procedures that may seem straightforward, such as pacemaker implantation or ASD closure, may be more complex when accounting for the nuances imparted by CHD.
2. The data obtained and the interventions performed during ACHD cardiac procedures are difficult to sort out without specialized knowledge of the CHD.
2. Consultation with providers with ACHD expertise may be substituted if the procedure is urgent such that timely transfer is not feasible.3. An ACHD program has additional resources such as cardiac anesthesia, congenital cardiac surgery, and specialty cardiac imaging, should the need for those services arise during or after the procedure.
Administration of anesthesia for invasive procedures in patients with ACHD AP classification IB-D, IIA-D, and IIIA-D*1. Performed by, or in collaboration with, an anesthesiologist with expertise in the management of patients with ACHD.1. ACHD-specific issues need to be addressed when considering anesthesia, including underlying cardiac physiology and hemodynamics, and the effects of anesthetic medications and ventilation strategies.The application of anesthesia for laparoscopic procedures can be especially challenging in Fontan patients. Significant cardiovascular and respiratory alterations may occur as a result of increased intra-abdominal pressure and decreased venous return. Abdominal insufflation may lead to lower preload and hypotension, while at the same time elevating systemic vascular resistance and compromising cardiac output. Elevations in pulmonary vascular resistance attributable to hypercarbia can be caused by either direct carbon dioxide absorption or hypoventilation.S3.3-11
2. If clinical urgency precludes transfer, consultation with an anesthesiologist with ACHD expertise would be of benefit to on-site providers who do not have ACHD expertise.2. Many patients with ACHD have had surgeries in the past, which may have created or identified airway or vascular access concerns. Patients with ACHD can also have underlying restrictive and/or obstructive lung disease that should be considered.S3.3-9,S3.3-10
Patients with ACHD and pulmonary hypertension1. Consultation with experts in pulmonary hypertension and ACHD to assist in the interpretation of diagnostic and invasive studies and determine the best course of management.1. PAH imparts a poor prognosis compared with CHD without PAH. Because of the complexity of PAH in the setting of CHD, patients with ACHD benefit from the expertise of both ACHD providers and pulmonary hypertension providers.S3.3-12–S3.3-20Management of PAH in patients with shunts can be difficult. For example, in patients for whom PAH treatment is expected to allow subsequent closure of a shunt, cohort series demonstrate progression of pulmonary vascular resistance or late mortality if defects with associated pulmonary vascular resistance elevation beyond 2.5 Wood units (>4 Wood units×m2) or Qp:Qs >3 were closed.S3.3-21,S3.3-22 The utility of acute administration of pulmonary vasodilator therapy as a marker of reversibility of pulmonary vascular resistance remains uncertain. “Treat-to-repair” strategies involving use of PAH therapies to bring pulmonary vascular resistance into a range where repair can be considered have been applied, but the utility of such strategies also remains uncertain.

*See Tables 3 and 4 for details on the ACHD AP classification system.

3D indicates 3-dimensional; ACHD, adult congenital heart disease; AP, anatomic and physiological; ASD, atrial septal defect; CCT, cardiac computed tomography; CHD, congenital heart disease; CMR, cardiovascular magnetic resonance; IE, infective endocarditis; PAH, pulmonary arterial hypertension; PR, pulmonary regurgitation; Qp:Qs, pulmonary–systemic blood flow ratio; RV, right ventricle; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.

3.4. Evaluation of Suspected and Known CHD

3.4.1. Electrocardiogram

See also Table 7.

Table 7. Use of ECGs in ACHD Evaluation

Identification of sinus bradycardia or junctional rhythm in patients at risk of sinus node dysfunction (especially after the Mustard, Senning, Glenn, or Fontan procedure)
Identification of clinically inapparent intra-atrial re-entry tachycardia in patients who have had atriotomy
Identification of atrioventricular block in patients at risk for progression of atrioventricular conduction system disease (especially CCTGA)
Evaluation of rhythm in patients with pacemakers
Measurement of QRS duration in patients after repair of TOF and as part of CRT evaluation
Preoperatively to compare with postoperative ECGs in patients undergoing heart surgery and noncardiac surgery
Postoperatively to identify arrhythmias (eg, atrial ectopic tachycardia, atrial flutter, AF, junctional ectopic tachycardia, atrioventricular block)
Diagnosis of Wolff-Parkinson-White Syndrome in patients with Ebstein anomaly
Initial evaluation of suspected acute coronary syndromes

ACHD indicates adult congenital heart disease; AF, atrial fibrillation; CCTGA, congenitally corrected transposition of the great arteries; CRT, cardiac resynchronization therapy; ECG, electrocardiogram; and TOF, tetralogy of Fallot.

Recommendations for Electrocardiogram

3.4.2. Ionizing Radiation Principles

Recommendation for Ionizing Radiation Principles

Referenced studies that support the recommendation are summarized in Online Data Supplement 6.

3.4.3. Echocardiography

Recommendations for Echocardiography

Referenced studies that support recommendations are summarized in Online Data Supplement 7.

3.4.4. CMR Imaging

See also Tables 8 and 9.

Table 8. Circumstances Where CMR, CCT, TEE, and/or Cardiac Catheterization May be Superior to TTE

Assessment of RV size and function in repaired TOF, systemic right ventricles, and other conditions associated with RV volume and pressure overloadS3.4.4-1,S3.4.4-3
Identification of anomalous pulmonary venous connectionsS3.4.4-11
Serial assessment of thoracic aortic aneurysms, especially when the dilation might extend beyond the echocardiographic windowsS3.4.4-7
Accurate assessment of PA pressure and pulmonary vascular resistance
Assessment for recoarctation of the aorta
Sinus venosus defects
Vascular rings
Evaluation of coronary anomalies
Quantification of valvular regurgitation

CCT indicates cardiac computed tomography; CMR, cardiovascular magnetic resonance; PA, pulmonary artery; RV, right ventricular; TEE, transesophageal echocardiography; and TOF, tetralogy of Fallot.

Table 9. Comparison of Imaging Modalities Useful in ACHD Evaluation

Radiation ExposureRelative CostVentricular Volumes/FunctionValvular Structure/FunctionCoronary Anatomy and CourseExtracardiac Vascular Anatomy
EchocardiographyNo$++++++/-+/-
CMRNo$$+++++++*+++
CCTYes$$+*+++++++
Cardiac catheterizationYes$$++++++++

$ indicates less expensive; $$, more expensive; +/-, possible value; +, good; ++, very good; and +++, excellent.

*In specific gated imaging protocols.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; and CMR, cardiovascular magnetic resonance.

Recommendations for CMR Imaging

Referenced studies that support recommendations are summarized in Online Data Supplement 8.

3.4.5. Cardiac Computed Tomography

Recommendation for Cardiac Computed Tomography

Referenced studies that support the recommendation are summarized in Online Data Supplement 9.

3.4.6. Cardiac Catheterization

Recommendations for Cardiac Catheterization

Referenced studies that support recommendations are summarized in Online Data Supplement 10.

3.4.7. Exercise Testing

Recommendations for Exercise Testing

Referenced studies that support recommendations are summarized in Online Data Supplement 11.

3.5. Transition Education

Recommendation for Transition Education

Referenced studies that support the recommendation are summarized in Online Data Supplement 12.

3.6. Exercise and Sports

Recommendations for Exercise and Sports

Referenced studies that support recommendations are summarized in Online Data Supplement 13.

3.7. Mental Health and Neurodevelopmental Issues

Recommendations for Mental Health and Neurodevelopmental Issues

Referenced studies that support recommendations are summarized in Online Data Supplement 14.

3.8. Endocarditis Prevention

Patients with ACHD have an increased risk of developing infective endocarditis (IE).S3.8-1,S3.8-2 The most common pathogens responsible for IE include Streptococcus viridans, Staphylococcus species, and Enterococcus species. Despite advances in antimicrobial therapy and surgical techniques, IE remains a condition associated with significant morbidity and mortality. Numerous guidelines are available with recommendations on the prevention and diagnosis of IE.S3.8-3–S3.8-5 These guidelines include consistent descriptions of the patients at highest risk of adverse effects from endocarditis. Antibiotic prophylaxis continues to be recommended for patients with high-risk characteristics, which are often found in patients with ACHD.S3.8-2 These patients include:

  • Those with previous IE;

  • Patients with prosthetic valves (biological and mechanical, surgical and transcatheter);

  • Patients within 6 months of placement of prosthetic material;

  • Patients with residual intracardiac shunts at the site of or adjacent to previous repair with prosthetic material or devices; or

  • Patients with uncorrected cyanotic heart disease.

See Online Data Supplement 15 for referenced studies.

3.9. Concomitant Syndromes

See also Table 10.

Table 10. Underlying Genetic Syndromes Commonly Associated With CHDS3.9-3,S3.9-4

SyndromeGenetic AbnormalityClinical FeaturesCommon Cardiac Findings
DiGeorge syndrome (velocardiofacial syndrome)22q11.2 deletionThymic and parathyroid hypoplasia, immunodeficiency, low-set ears, hypocalcemia, speech and learning disorders, renal anomalies, psychiatric diseaseIAA type B, aortic arch anomalies, truncus arteriosus, TOF
25%–75% have CHD, depending on age studiedS3.9-5,S3.9-6
Down syndromeTrisomy 21Developmental disability, characteristic facial features, hypotonia, palmar creaseASD, VSD, AVSD, TOF
40%–50% have CHD
Holt–Oram syndromeS3.9-7TBX5Upper limb skeletal abnormalitiesASD, VSD, MV disease
75% have CHD
Klinefelter syndrome47 XXYTall stature, hypoplastic testes, delayed puberty, developmental disabilityPDA, ASD, MV prolapse
50% have CHD
Noonan syndromeS3.9-8PTPN11, KRAS, SOS1 RAF1, NRAS, BRAF, MAP2K1Facial anomalies, webbed neck, chest deformity, short stature, lymphatic abnormalities, bleeding abnormalitiesPS, ASD, HCM
80% have CHD
Turner syndrome45XShort stature, webbed neck, lymphedema, primary amenorrheaCoarctation, BAV, aortic stenosis, hypoplastic left heart, ascending aortopathy
30% have CHD
Risk of aortic dissection
Williams syndrome7q11.23 deletionElfin face, social personality, hearing loss, developmental delay, infantile hypercalcemiaSupravalvar aortic stenosis, peripheral PS
50%–80% have CHD

ASD indicates atrial septal defect; AVSD, atrioventricular septal defect; BAV, bicuspid aortic valve; CHD, congenital heart disease; HCM, hypertrophic cardiomyopathy; IAA, interrupted aortic arch; MV, mitral valve; PDA, patent ductus arteriosus; PS, pulmonary stenosis; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.

Recommendation for Concomitant Syndromes

Referenced studies that support the recommendation are summarized in Online Data Supplement 16.

3.10. Noncardiac Medical Issues

Recommendation for Noncardiac Medical Issues

Referenced studies that support the recommendation are summarized in Online Data Supplement 18.

3.11. Noncardiac Surgery

See also Table 11.

Table 11. ACHD Management Issues for Noncardiac Surgery

Clarify CHD diagnosis
 Clarify prior procedures, residua, sequelae, and current status, including ACHD AP classification
 Be aware that history obtained from only the patient and family may be faulty or incomplete
 Obtain and review old records to ensure accurate understanding of past procedures and clinical course
 Complete additional investigations required to define ACHD AP classification
 Develop management strategies to minimize risk and optimize outcome
Factors associated with increased risk of perioperative morbidity and mortalityS3.12-12.:
 Cyanosis
 Congestive HF
 Poor general health
 Younger age
 Pulmonary hypertension
 Operations on the respiratory and nervous systems
 Complex CHD
 Urgent/emergency procedures
Issues to consider:
 Endocarditis prophylaxis
 Complications related to underlying hemodynamics
 Abnormal venous and/or arterial anatomy affecting venous and arterial access
 Persistent shunts
 Valvular disease
 Arrhythmias, including bradyarrhythmias
 Erythrocytosis
 Pulmonary vascular disease
 Meticulous line care (also consider air filters for intravenous lines) to reduce risk of paradoxic embolus in patients who are cyanotic because of right-to-left shunts
 Adjustment of anticoagulant volume in tubes for some blood work in cyanotic patients
 Prevention of venous thrombosis
 Monitoring of renal and liver function
 Periprocedure anticoagulation
 Possible need for nonconventional drug dosing
 Increased prevalence of hepatitis C infection because of prior procedures and remote blood transfusions
 Developmental disability

ACHD indicates adult congenital heart disease; AP, anatomic and physiological; CHD, congenital heart disease; and HF, heart failure.

Recommendations for Noncardiac Surgery

Referenced studies that support recommendations are summarized in Online Data Supplement 18.

3.12. Pregnancy, Reproduction, and Sexual Health

3.12.1. Pregnancy

Recommendations for Pregnancy

Referenced studies that support recommendations are summarized in Online Data Supplement 19.

3.12.2. Contraception

Recommendations for Contraception

Referenced studies that support recommendations are summarized in Online Data Supplement 20.

3.13. Heart Failure and Transplant

3.13.1. Heart Failure

Recommendation for Heart Failure

Referenced studies that support the recommendation are summarized in Online Data Supplement 22.

3.13.2. Heart Transplant

Because of the prevalence of HF among patients with CHD, heart transplantation is increasingly being considered as a therapeutic option. Data on proper timing of transplantation are limited, particularly for individual lesions. Larger studies based on transplant databases do not allow for analysis based on the type of CHD.S3.13.2-1–S3.13.2-4 Currently, patients with ACHD may have fewer mechanical circulatory devices (eg, ventricular-assist devices), which may lower their listing status and hence potential for organ receipt.S3.13.2-1,S3.13.2-2,S3.13.2-4–S3.13.2-7

Although specific criteria for timing of referral for transplantation are desirable, universal recommendations cannot be made based on current data. Generally, published data show that immediate and early posttransplantation risk is higher in ACHD than in acquired heart disease because of increased perioperative mortality.S3.13.2-2 However, once beyond the perioperative period, patients with ACHD do as well as or better than those with acquired heart disease, with expected 10-year survival equivalent to or better than that of patients without ACHD.S3.13.2-2-4.,S3.13.2-6,S3.13.2-7 Risks for poor outcomes include single ventricle anatomy, anatomic complexity, protein-losing enteropathy, or high titers of panel reactive antibodies.S3.13.2-8,S3.13.2-9 The current allocation system puts patients with ACHD at a disadvantage. Rather than priority dictated by the usual accepted risk markers, patients with ACHD are often listed by “exception,” a process that requires the clinician to argue that the patient warrants higher priority than would be evident by applying the used risk markers. There is also significant mortality for patients with ACHD while on the waitlist.S3.13.2-10,S3.13.2-11 Surgical alternatives to transplantation exist for some patients with CHD (eg, valve replacement, shunt closure), but these patients are at high risk of perioperative mortality.S3.13.2-12 Ideally, providers will consider early referral to a transplant center with expertise in ACHD transplantation when transplantation becomes a relevant clinical consideration. Additionally, it is advisable to consider options for transplantation or ventricular assist device as a backup before other high-risk surgery is pursued.

See Online Data Supplement 23 for referenced studies.

3.14. Palliative Care

Recommendation for Palliative Care

Referenced studies that support the recommendation are summarized in Online Data Supplement 24.

3.15. Cyanosis

See Table 12.

Table 12. Specific Management Practices for Cyanotic CHD

Recording clinical oxygen saturation at rest (>5 min) rather than immediately after effort (eg, walking into a clinic examination room).
Meticulous intravenous care to avoid air or particulate matter, which may include use of air/particulate filters on all intravenous access lines, when feasible, and careful de-airing of all lines.
Cerebral imaging for any new headache or neurologic sign to assess for possible cerebral abscess, hemorrhage, or stroke.
Measurement of serum uric acid and treatment with allopurinol in a patient with a history of gout.
Supplemental oxygen as needed for symptom relief but not to a target oxygen saturation level and not if there is no demonstrable symptomatic benefit.
Avoidance of or cautious use of therapies that may reduce the patient’s hypoxia-mediated drive to ventilation, such as narcotics or, in rare circumstances, excess supplemental oxygen.S3.15-1
Anesthesia by providers with expertise in anesthesia for patients with ACHD for any noncardiac surgery.
Non-estrogen–containing birth control for women of child-bearing potential (intrauterine device may be a preferred option). Avoidance of birth control entirely is not a safe acceptable option.
Patients can travel safely on commercial airlines without undue risk.S3.15-2 Preflight simulation testing or mandated supplemental oxygen are not usually indicated, although adequate hydration and movement during the flight are appropriate.
Measurement of coagulation parameters (eg, activated partial thromboplastin time, international normalized ratio, thrombin time) in a patient with an elevated hematocrit >55% requires adjustment of anticoagulant volume in the blood collection vials to account for reduced plasma volume in the draw.S3.15-3

See Online Data Supplement 25 for referenced studies.

ACHD indicates adult congenital heart disease and CHD, congenital heart disease.

3.16. Pharmacological Therapy for ACHD

Patients with ACHD are commonly excluded from clinical trials, and there are few data to guide pharmacological therapies. Although it may be tempting to extrapolate from management guidelines developed for patients without CHD (eg, HF guidelines),S3.16-1 treatments may not have the same benefit in the heterogeneous population of patients with ACHD and in some cases may cause harm. The evaluation of new symptoms in a patient with ACHD must be tailored to the patient’s anatomy, surgical repair, and physiology. Before considering pharmacological therapies, evaluation for residual shunts, baffle stenosis, valvular or conduit dysfunction, and collateral vessels, any of which may be amenable to interventions, is an important consideration.

The literature documenting pharmacological therapies for patients with ACHD is limited to small studies with limited duration of drug administration and follow-up. Additionally, the endpoints used are often surrogate markers that have not been validated for clinical decision-making, and studies are also often underpowered. However, studies in patients with ACHD do exist and evaluate conventional pharmacological therapy, especially for HF and for arrhythmia, including beta blockers, angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, and aldosterone antagonists, although results vary.S3.16-2–S3.16-9

Pharmacological therapies in patients with ACHD are often directed to specific conditions (ie, beta blockers for arrhythmia treatment). However, there are limited data examining the benefits of beta blockers in specific ACHD populations. Results from a small study indicate that beta-blocker therapy may have potential to improve functional class in patients with a systemic right ventricle and a pacemaker.S3.16-2 Angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers have also been assessed in small studies in specific ACHD populations in which no significant benefit on ventricular function or exercise capacity has been proven.S3.16-6–S3.16-8 Data from 1 small trial with a short follow-up interval in patients with a systemic right ventricle suggest that eplerenone may be associated with reduced myocardial fibrosis, as assessed by imaging.S3.16-3

Some pharmacological therapies affecting the pulmonary vasculature (eg, endothelin-receptor antagonists and phosphodiesterase type-5 [PDE-5] inhibitors) have a beneficial effect on long-term outcomes in patients with Eisenmenger syndrome.S3.16-10 Similarly, there are limited data on the use of pulmonary vasodilator therapy in Fontan patients, in whom the pulmonary vascular resistance may be abnormal.S3.16-11–S3.16-13 Because of the lack of data, clinical recommendations regarding pharmacological therapy for patients with ACHD are unsupported. Individualized care is needed, recognizing the potential benefits and risks of the therapy relative to patient-specific anatomic and physiological issues.

See Online Data Supplement 23 for referenced studies.

4. Specific Lesions

4.1. Shunt Lesions

4.1.1. Atrial Septal Defect

See also Table 13 and Figure 1.

Table 13. ASD: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist36–60246–123–6
ECG36–60241212
TTE36–60241212
Pulse oximetryAs neededAs neededEach visitEach visit
Exercise testAs neededAs needed12–246–12

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Atrial Septal Defect

Referenced studies that support recommendations are summarized in Online Data Supplement 26 and the ERC systematic review report.S4.1.1-1

Figure 1.

Figure 1. Secundum ASD.

*Combination therapy with bosentan and PDE-5 inhibitor if symptomatic improvement does not occur with either alone. ACHD indicates adult congenital heart disease; ASD, atrial septal defect; PAH, pulmonary artery hypertension; PASP, pulmonary artery systolic pressure; PDE-5, phosphodiesterase type-5 inhibitors; PH, pulmonary hypertension; and Qp:Qs, pulmonary–systemic blood flow ratio.

4.1.2. Anomalous Pulmonary Venous Connections

Recommendations for Anomalous Pulmonary Venous Connections

Referenced studies that support recommendations are summarized in Online Data Supplement 27.

4.1.3. Ventricular Septal Defect

See also Table 14 and Figure 2.

Table 14. VSD: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist36246–123–6
ECG36241212
TTE36241212
Pulse oximetryAs neededAs neededEach visitEach visit
Exercise testAs neededAs needed12–246–12

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on the clinical circumstance.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; TTE, transthoracic echocardiogram; and VSD, ventricular septal defect.

Recommendations for Ventricular Septal Defect

Referenced studies that support recommendations are summarized in Online Data Supplement 28.

Figure 2.

Figure 2. Hemodynamically significant ventricular level shunt.

*Combination therapy with bosentan and PDE-5 inhibitor, if symptomatic improvement does not occur with either alone. ACHD indicates adult congenital heart disease; AR, aortic regurgitation; IE, infective endocarditis; LV, left ventricular; PAH, pulmonary artery hypertension; PASP, pulmonary artery systolic pressure; PDE-5, phosphodiesterase type-5 inhibitors; PH, pulmonary hypertension; Qp:Qs, pulmonary–systemic blood flow ratio; and VSD, ventricular septal defect.

4.1.4. Atrioventricular Septal Defect

See also Table 15.

Table 15. AVSD: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24–36246–123–6
ECG24–36241212
TTE24–36241212
Pulse oximetryAs neededAs neededEach visitEach visit
Exercise testAs neededAs needed12–246–12

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; AVSD, atrioventricular septal defect; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Atrioventricular Septal Defect

Referenced studies that support recommendations are summarized in Online Data Supplement 29.

4.1.5. Patent Ductus Arteriosus

See also Table 16.

Table 16. PDA: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist36–60246–123–6
ECG36–60241212
TTE36–60241212
Pulse oximetryAs neededAs neededEach visitEach visit
Exercise testAs neededAs needed12–246–12

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Upper and lower extremity.

‡6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PDA, patent ductus arteriosus; and TTE, transthoracic echocardiogram.

Recommendations for Patent Ductus Arteriosus

Referenced studies that support recommendations are summarized in Online Data Supplement 30.

4.2. Left-Sided Obstructive Lesions

4.2.1. Cor Triatriatum

Recommendations for Cor Triatriatum

Referenced studies that support recommendations are summarized in Online Data Supplement 31.

4.2.2. Congenital Mitral Stenosis

See also Table 17.

Table 17. Congenital Mitral Stenosis: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24246–123–6
ECG24241212
TTE24241212
Exercise testAs needed242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendation for Congenital Mitral Stenosis

Referenced studies that support the recommendation are summarized in Online Data Supplement 32.

4.2.3. Subaortic Stenosis

See also Table 18.

Table 18. Subaortic Stenosis: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24246–123–6
ECG24241212
TTE24241212
Exercise testAs needed242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Subaortic Stenosis

Referenced studies that support recommendations are summarized in Online Data Supplement 33.

4.2.4. Congenital Valvular Aortic Stenosis

See also Table 19.

Table 19. Congenital Aortic Stenosis: Routine Follow-Up and Testing Intervals*

StageFrequency of Echocardiogram
Progressive (Stage B)Every 3–5 y (mild severity, Vmax 2.0–2.9 m/s)
Every 1–2 y (moderate severity, Vmax 3.0–3.9 m/s)
Severe (Stage C)Every 6–12 mo (Vmax >4.0 m/s)
Aortic dilation >4.5 cmEvery 12 mo (echocardiogram, MRI or CT)

*Modified from existing GDMT for valvular heart disease.S4.2.4-5

CT indicates computed tomography; GDMT, guideline-directed management and therapy; MRI, magnetic resonance imaging; and Vmax, maximum velocity.

Recommendations for Congenital Valvular Aortic Stenosis

Referenced studies that support recommendations are summarized in Online Data Supplement 34.

4.2.4.1. Turner Syndrome

Recommendations for Turner Syndrome

Referenced studies that support recommendations are summarized in Online Data Supplement 35.

4.2.5. Supravalvular Aortic Stenosis

See also Table 20.

Table 20. Supravalvular Aortic Stenosis: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24246–123–6
ECG24241212
TTE24241212
CMR/CCT§36–6036–6036–6036–60
Exercise testAs needed242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of aortic anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Supravalvular Aortic Stenosis

Referenced studies that support recommendations are summarized in Online Data Supplement 37.

4.2.6. Coarctation of the Aorta

See also Table 21.

Table 21. CoA: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24246–123–6
ECG24241212
TTE24241212
CMR/CCT§36–6036–6012–2412–24
Exercise test36242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of aortic size and aortic arch/coarctation repair site anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy for coarctation of the aorta; the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on the clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Coarctation of the Aorta

Referenced studies that support recommendations are summarized in Online Data Supplement 38.

4.3. Right-Sided Lesions

4.3.1. Valvular Pulmonary Stenosis

See also Tables 22 and 23.

Table 22. Severity of RVOT Obstruction

MildPeak gradient <36 mm Hg (peak velocity <3 m/s)ModeratePeak gradient 36–64 mm Hg (peak velocity 3–4 m/s)SeverePeak gradient 64 mm Hg (peak velocity >4 m/s); mean gradient >35 mm Hg

Estimations of RV systolic pressure by TR velocity is part of the echocardiographic assessment of RV obstruction, as Doppler measurements across the RV obstruction itself may be unreliable.

RV indicates right ventricular; RVOT, right ventricular outflow tract; and TR, tricuspid regurgitation.

Table 23. Valvular PS: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist36–60246–123–6
ECG36–60241212
TTE36–60241212
Exercise testAs needed242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PS, pulmonary stenosis; and TTE, transthoracic echocardiogram.

Recommendations for Valvular Pulmonary Stenosis

Referenced studies that support recommendations are summarized in Online Data Supplement 39.

Figure 3.

Figure 3. Isolated PR after repair of PS.

*Significant PR causes RV dilation. If a patient has moderate or greater PR and normal RV size, most likely the estimation of PR severity is inaccurate or there may be restrictive RV physiology, which would warrant further investigation. †Symptoms may include dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; PR, pulmonary regurgitation; PS, pulmonary stenosis; and RV, right ventricular.

4.3.1.1. Isolated PR After Repair of PS

Recommendations for Isolated PR After Repair of Pulmonary Stenosis

4.3.2. Branch and Peripheral Pulmonary Stenosis

See also Table 24.

Table 24. Branch and Peripheral PS: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24–36246–123–6
ECG24–36241212
TTE24–36241212
CMR/CCT§36–6036–6024–3624–36
Exercise test36242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of branch PS. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status and post-stent therapy for peripheral PS; the frequency should be weighed against radiation exposure.

‖6-minute walk test or cardiopulmonary exercise test, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; ECG, electrocardiogram; PS, pulmonary stenosis; and TTE, transthoracic echocardiogram.

Recommendations for Branch and Peripheral PS

Referenced studies that support recommendations are summarized in Online Data Supplement 40.

4.3.3. Double-Chambered Right Ventricle

See also Table 25.

Table 25 Double-Chambered Right Ventricle: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist24–36246–123–6
ECG24–36241212
TTE24–36241212
Exercise testAs needed242412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Double-Chambered Right Ventricle

Referenced studies that support recommendations are summarized in Online Data Supplement 41.

4.3.4. Ebstein Anomaly

See also Table 26.

Table 26. Ebstein Anomaly: Routine and Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12–24126–123–6
ECG12–24121212
CXRAs neededAs needed12–2412–24
TTE12–2412–241212
Pulse oximetry2412Each visitEach visit
Holter monitorAs neededAs needed2412–24
CMR/CCT§603624–3612–24
Exercise test3624–362412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of right ventricular size and function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible; the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; CXR, chest x ray; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Ebstein Anomaly

Referenced studies that support recommendations are summarized in Online Data Supplement 42.

4.3.5. Tetralogy of Fallot

See also Table 27.

Table 27. TOF: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12–24126–123–6
ECG24121212
TTE2412–24126–12
Pulse oximetryAs neededAs neededEach visitEach visit
Holter monitorAs neededAs needed12–2412–24
CMR/CCT§3624–3612–2412–24
Exercise test36–6024–6012–2412–24

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of right ventricular size and function, pulmonary valve function, pulmonary artery anatomy and left heart abnormalities. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate origin and course of the coronary arteries, and cross-sectional imaging status—post-stent therapy. If cardiac CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; TOF, tetralogy of Fallot; and TTE, transthoracic echocardiogram.

Recommendations for TOF

Referenced studies that support recommendations are summarized in Online Data Supplement 43. (See Section 4.3.6. for recommendations regarding evaluation and management of right ventricle–to-PA conduits.)

Figure 4.

Figure 4. Pulmonary valve replacement in patients with TOF repair and PR.

*Symptoms may included dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained. ACHD indicates adult congenital heart disease; HF, heart failure; LV, left ventricular; LVEDV, left ventricular end diastolic volume; PR, pulmonary regurgitation; RV, right ventricular; RVEDV, right ventricular end diastolic volume; RVEDVI, right ventricular end diastolic volume index; RVESVI, right ventricular end systolic volume index; RVOT, right ventricular outflow tract; RVSP, right ventricular systolic pressure; and TOF, tetralogy of Fallot. †See recommendation 6 supporting text in the full text of the “2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease.”P-9

4.3.6. Right Ventricle–to-Pulmonary Artery Conduit

See also Table 28.

Table 28. Right Ventricle–to-PA Conduit: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12–24126–123–6
ECG12–24121212
TTE12–24121212
CMR/CCT§36–6036–6012–2412–24
Exercise testAs neededAs needed12–2412–24

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of right ventricular size and function and valvular function, conduit anatomy and pulmonary artery anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PA, pulmonary artery; and TTE, transthoracic echocardiogram.

Recommendations for Right Ventricle–to-PA Conduit

Referenced studies that support recommendations are summarized in Online Data Supplement 44.

4.4. Complex Lesions

4.4.1. Transposition of the Great Arteries
4.4.1.1. Transposition of the Great Arteries With Atrial Switch

See also Table 29.

Table 29. d-TGA With Atrial Switch: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12126–123–6
ECG12126–126–12
TTE12–2412–241212
Pulse oximetry1212Each visitEach visit
Holter monitor24241212
CMR/CCT§24–362412–2412–24
Exercise test36362412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of ventricular size and function, systemic and venous baffle obstruction and leaks, and valvular function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute exercise test or cardiopulmonary exercise test, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; d-TGA, dextro-transposition of the great arteries; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for d-TGA With Atrial Switch

Referenced studies that support recommendations are summarized in Online Data Supplement 45.

4.4.1.2. Transposition of the Great Arteries With Arterial Switch

See also Table 30.

Table 30. d-TGA With Arterial Switch: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12–24126–123–6
ECG12–2412–24126
TTE12–2412–241212
CMR/CCT§36–6024–3612–2412–24
Exercise test36–6036–6024–3612–24

*See ACHD AP classification Table 4.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of neoaortic size, the origin and proximal course of the coronary arteries, branch pulmonary arteries, ventricular function and valvular function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT or catheterization once to establish knowledge of coronary artery anatomy and then as warranted by clinical condition. CCT may be used if CMR is not feasible and to evaluate coronary artery anatomy and cross-sectional imaging status-post stent therapy. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute exercise test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; d-TGA, dextro-transposition of the great arteries; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for d-TGA With Arterial Switch

Referenced studies that support recommendations are summarized in Online Data Supplement 46.

4.4.1.3. Congenitally Corrected Transposition of the Great Arteries

See also Table 31.

Table 31. CCTGA: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist12126–123–6
ECG12121212
TTE12–24121212
Pulse oximetryAs neededAs neededEach visitEach visit
Holter monitor12–6012–6012–3612
CMR/CCT§36–6036–6012–2412
Exercise test36–6036–6012–2412

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of ventricular size and function and valvular function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CCTGA, congenitally corrected transposition of the great arteries; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Congenitally Corrected Transposition of the Great Arteries

Referenced studies that support recommendations are summarized in Online Data Supplement 47.

4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle)

See also Table 32.

Table 32. Fontan Palliation: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage A* (mo)Physiological Stage B* (mo)Physiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist121263–6
ECG12126–126
TTE12121212
Pulse oximetry1212Each visitEach visit
Holter monitor12121212
CMR/CCT§36242424
Exercise test36241212

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of the long-term sequelae of Fontan palliation: thrombosis, right-to-left shunts (eg, fenestration. intrapulmonary atrioventricular malformation), obstructive lesion, systemic atrioventricular valve dysfunction, ventricular size and function, collateral burden, and branch pulmonary artery obstruction. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status-post stent therapy. CCT with contrast injection in Fontan patients can be misleading; therefore, it should be done only when clinically indicated and when it can be appropriately protocoled and interpreted. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.

‖6-minute walk test or CPET, depending on clinical indication.

ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Fontan Palliation of Single Ventricle Physiology

Referenced studies that support recommendations are summarized in Online Data Supplement 48.

4.4.3. Double Outlet Right Ventricle

Double outlet right ventricle is an anatomic descriptor that includes abnormalities similar to TOF in some patients (when the aorta is closely related to the VSD) and similar to d-TGA with a VSD in others (when the PA is more closely related to the VSD than the aorta). Repairs are predicated on the underlying anatomy and may involve VSD closure with relief of PS, right ventricle–to-PA conduit, or Rastelli-type repair. In severe cases, single-ventricle physiology may be present. Consequently, recommendations for the management of a patient with double outlet right ventricle can generally be inferred in the recommendations for the lesion with the most similar anatomy and physiology (eg, TOF can reasonably be based on the recommendations in Section 4.4.1, recognizing that a patient with double outlet right ventricle is more likely to have residual LVOT obstruction).

4.4.4. Severe PAH and Eisenmenger Syndrome
4.4.4.1. Severe PAH

See also Table 33.

Table 33. Pulmonary Hypertension and Eisenmenger Syndrome: Routine Follow-Up and Testing Intervals

Frequency of Routine Follow-Up and TestingPhysiological Stage C* (mo)Physiological Stage D* (mo)
Outpatient ACHD cardiologist6–123–6
ECG1212
TTE1212
Pulse oximetryEach visitEach visit
CMRAs neededAs needed
Exercise test§6–126–12
Cardiac catheterizationAs neededAs needed

*See Tables 3 and 4 for details on the ACHD AP classification system.

†Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.

‡CMR may be indicated for assessment of right ventricular function and CHD anatomy not clarified with TTE. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.

§6-minute walk test or CPET, depending on clinical indication.

‖Cardiac catheterization should be performed at baseline and as needed.

ACHD indicates adult congenital heart disease; CMR, cardiovascular magnetic resonance; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.

Recommendations for Severe PAH

Referenced studies that support recommendations are summarized in Online Data Supplement 49.

4.4.4.2. Eisenmenger Syndrome

Recommendations for Eisenmenger Syndrome

Referenced studies that support recommendations are summarized in Online Data Supplement 50.

4.4.5. Coronary Anomalies

Table 34.

Table 34. Factors That May Relate to the Clinical Importance of AAOCA and Risk of SCD

AgeAAOCA is more commonly invoked as the cause of SCD in patients <35 y of age than in patients >35 y of age, in whom atherosclerotic coronary disease becomes a more prevalent cause. However, death has been attributed to AAOCA in patients of all ages; there does not seem to be an age beyond which the AAOCA may not be relevant, even in the setting of atherosclerotic coronary disease and other concomitant conditions.S4.4.5-1,S4.4.5-2
Anatomy of coronary ostium and proximal coronary courseSlit-like/fish-mouth-shaped orifice, acute angle takeoff, intramural course, interarterial course and hypoplasia of the proximal coronary artery have all been proposed as reasons for symptoms, ischemia and SCD in patients with AAOCA. The slit-like orifice is more commonly seen in anomalous right coronary artery arising from the left sinus. Each of these anatomic findings offers a pathophysiological mechanism for intermittent ischemia, particularly at times of high cardiac output and/or increased aortic wall tension, such as during exercise.S4.4.5-3–S4.4.5-6
Anomalous originLeft coronary artery arising from the right cusp is less common than the right coronary artery arising from the left cusp but is more often found in autopsy series of SCD.S4.4.5-1,S4.4.5-7,S4.4.5-8 This suggests that anomalous origin of the left coronary artery from the right cusp is more likely to cause SCD than anomalous origin of the right coronary artery from the left cusp. This may be due either to anatomic features that make anomalous aortic origin of the left coronary artery prone to coronary compromise or because a larger proportion of myocardium is supplied by the left coronary artery, or both.
ExerciseAutopsy series suggest most patients die during, or in close temporal association with, exercise.S4.4.5-8–S4.4.5-10
IschemiaAutopsy series demonstrate myocardial fibrosis in a significant number of patients whose deaths were attributed to AAOCA, particularly in patients with anomalous left coronary artery arising from the right cusp.S4.4.5-10 Surgical series describe patients with ischemia or MI before surgical repair in the absence of other CAD, suggesting a relation of the coronary anomaly to the ischemia.S4.4.5-11 This suggests that had perfusion imaging been obtained before SCD, ischemia would have been found in such patients.S4.4.5-12,S4.4.5-13 However, other data indicate that a normal stress test does not preclude a SCD event, with the proviso that most of those studies used only stress ECG, rather than the more sensitive and specific modalities of nuclear perfusion imaging or stress echocardiography. In addition, postoperative studies have shown that ischemia may be found after surgical repair in the distribution not supplied by the abnormal coronary artery and may not persist on repeat testing.S4.4.5-14
SymptomsIn autopsy and surgical series, a significant number of patients reported cardiovascular symptoms, including before SCD events.S4.4.5-9,S4.4.5-15–S4.4.5-18 Symptoms are more commonly reported in patients in whom the left coronary artery arises from the right sinus. Surgical series have described improvement in symptoms after surgical repair.S4.4.5-6,S4.4.5-8–S4.4.5-10,S4.4.5-15,S4.4.5-16

AAOCA indicates anomalous aortic origin of the coronary artery; CAD, coronary artery disease; ECG, electrocardiogram; MI, myocardial infarction; and SCD, sudden cardiac death.

4.4.5.1. Anomalous Coronary Artery Evaluation

Table 34.

Recommendations for Anomalous Coronary Artery Evaluation

Referenced studies that support recommendations are summarized in Online Data Supplement 51.

4.4.5.2. Anomalous Aortic Origin of Coronary Artery

Figure 5.

Recommendations for Anomalous Aortic Origin of Coronary Artery

Referenced studies that support recommendations are summarized in Online Data Supplement 51.

Figure 5.

Figure 5. Anomalous aortic origin of the coronary artery.

*Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction.

4.4.5.3. Anomalous Coronary Artery Arising From the PA

Recommendations for Anomalous Coronary Artery Arising From the PA

Referenced studies that support recommendations are summarized in Online Data Supplement 51.

5. Evidence Gaps and Future Directions

Table 35.

Table 35. High-Impact Research Questions in ACHD

General
 PathophysiologyWhat are the mechanisms of heart failure that can be prevented, reversed, or treated?
Why does the systemic right ventricle fail?
Will all patients with Fontan physiology develop clinically important cirrhosis, and how can we prevent this?
Who is at risk of aortic rupture and dilation?
Are patients with manipulated coronary arteries (eg, ASO, Ross repair) at risk of premature coronary artery disease?
What is the impact of radiation exposure on long-term health?
Can we predict who will develop pulmonary hypertension/pulmonary vascular disease?
 Medical and surgical treatmentHow can we modify current CHD surgical procedures to prevent or reduce later development of heart failure and/or arrhythmias?
Which patients with ACHD can use direct oral anticoagulants instead of warfarin?
What is the best algorithm for contraception choices?
Beyond those with severe PAH, which patients will benefit from PAH therapies?
Do patients with ACHD with systemic right ventricles and HF benefit from standard therapies (beta blockers, ACE inhibitors/ARBs, aldosterone antagonist)? Which one(s)?
What medical therapies benefit patients with failing Fontan physiology?
Do asymptomatic patients with ACHD with PAH benefit from PAH-specific therapy?
Who will benefit from ventricular assist devices?
What should be the threshold(s) for aortic aneurysm surgery?
What pacing and resynchronization strategies are of most benefit, and when should they be used?
 Outcomes/risk assessmentWhat criteria should determine transplantation eligibility?
Which patients benefit from primary prevention ICDs?
How can we risk stratify for SCD in patients with systemic right ventricles?
What operative risk score predicts outcomes in ACHD reoperations?
What HF risk score predicts outcomes in patients with ACHD?
Is there a level of exercise where risk exceeds benefit?
What is the rate and/or risk of endocarditis?
 AssessmentWho is at high risk of neurodevelopmental abnormalities and would benefit from neuropsychiatric evaluation and treatment?
Who should be screened for anxiety and depression, what treatment is most effective, and are there differences compared with non-patients with ACHD?
What is the standard protocol for assessing right ventricular size and function by CMR imaging?
Which biomarkers are predictive of mortality and morbidity?
Disease-specific
 Coarctation of the aortaWhich measure of hypertension—resting, exercise, or ambulatory—best predicts outcomes?
Is there an optimal antihypertensive regimen?
What should blood pressure goals be?
How often should patients be screened for thoracic aneurysm?
Should exercise-induced hypertension be treated?
What criteria warrant reintervention in recoarctation?
Is long-term outcome better with medical therapy or catheter intervention for less than severe recoarctation?
Should patients be screened for intracranial aneurysm, and if so, how often?
 Ebstein anomalyWhat is the indication for surgery in the asymptomatic patient?
Who should have a Glenn shunt at the time of tricuspid valve surgery?
Should surgeons attempt tricuspid valve repair or routinely perform replacement in all patients?
 TOFWhat is the optimal timing for pulmonary valve replacement in asymptomatic patients with TOF?
Do pulmonary valve replacement and ventricular tachycardia ablation decrease the risk of SCD?
Who needs a primary prevention ICD, and does this strategy reduce mortality?
Is there a role for PAH therapies in TOF?
Why does left ventricular dysfunction develop?
 TGA/systemic right ventricleWho benefits from ACE inhibitors/ARBs/beta blockers/spironolactone?
Who needs a primary prevention ICD, and does this strategy prevent mortality?
What imaging findings predict mortality/morbidity?
In CCTGA with VSD/PS, does the double switch have better long-term outcomes than VSD closure and left ventricle–to-PA conduit?
When should tricuspid valve replacement be performed?
What is the role of cardiac resynchronization therapy in patients with systemic right ventricle?
 ASOWhat are the long-term outcomes after ASO?
How should the possibility of asymptomatic coronary disease (ostial, compression) and ischemia be assessed?
 Single ventricle/FontanIs warfarin or aspirin beneficial in patients with a Fontan?
Are PAH therapies beneficial?
Is exercise capacity predictive of mortality?
What liver screening is appropriate and at what intervals?
How is protein-losing enteropathy best medically treated?
Why do some patients fail with preserved ejection fraction, whereas other have decreased ejection fraction?
What are the long-term outcomes of hypoplastic left heart syndrome?
What is ideal timing for heart transplantation in single ventricle Fontan patients, and should liver issues prompt earlier transplantation than might be felt necessary from a cardiac perspective?
Which has better long-term outcomes, the Fontan operation or bidirectional Glenn alone?
 Coronary anomaliesDoes surgical intervention in anomalous aortic origin of coronary arteries improve survival?

ACE indicates angiotensin-converting enzyme; ACHD, adult congenital heart disease; ARB, angiotensin-receptor blocker; ASO, arterial switch operation; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; CMR, cardiac magnetic resonance; ICD, implantable cardioverter-defibrillator; HF, heart failure; PA, pulmonary artery; PAH, pulmonary artery hypertension; PS, pulmonary stenosis; SCD, sudden cardiac death; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.

ACC/AHA Task Force Members

Glenn N. Levine, MD, FACC, FAHA, Chair; Patrick T. O’Gara, MD, MACC, FAHA, Chair-Elect; Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair; Nancy M. Albert, PhD, RN, FAHA*; Sana M. Al-Khatib, MD, MHS, FACC, FAHA; Joshua A. Beckman, MD, MS, FAHA; Kim K. Birtcher, PharmD, MS, AACC; Biykem Bozkurt, MD, PhD, FACC, FAHA*; Ralph G. Brindis, MD, MPH, MACC*; Joaquin E. Cigarroa, MD, FACC; Lesley H. Curtis, PhD, FAHA*; Anita Deswal, MD, MPH, FACC, FAHA; Lee A. Fleisher, MD, FACC, FAHA; Federico Gentile, MD, FACC; Samuel S. Gidding, MD, FAHA*; Zachary D. Goldberger, MD, MS, FACC, FAHA; Mark A. Hlatky, MD, FACC; John Ikonomidis, MD, PhD, FAHA; José Joglar, MD, FACC, FAHA; Richard J. Kovacs, MD, FACC, FAHA*; Laura Mauri, MD, MSc, FAHA; E. Magnus Ohman, MD, FACC*; Mariann R. Piano, RN, PhD, FAHA, FAAN; Susan J. Pressler, PhD, RN, FAHA*; Barbara Riegel, PhD, RN, FAHA; Frank W. Sellke, MD, FACC, FAHA*; Win-Kuang Shen, MD, FACC, FAHA*; Duminda N. Wijeysundera, MD, PhD* Former Task Force member; current member during the writing effort.

Presidents and Staff

American College of Cardiology

C. Michael Valentine, MD, FACC, President

Cathleen C. Gates, Interim Chief Executive Officer and Chief Operating Officer

William J. Oetgen, MD, MBA, FACC, Executive Vice President, Science, Education, Quality, and Publishing

MaryAnne Elma, MPH, Senior Director, Science, Education, Quality, and Publishing

Amelia Scholtz, PhD, Publications Manager, Science, Education, Quality, and Publishing

American College of Cardiology/American Heart Association

Katherine A. Sheehan, PhD, Director, Guideline Strategy and Operations

Abdul R. Abdullah, MD, Senior Manager, Guideline Science

American Heart Association

Ivor Benjamin, MD, FAHA, President

Nancy Brown, Chief Executive Officer

Rose Marie Robertson, MD, FAHA, Chief Science and Medical Officer

Gayle R. Whitman, PhD, RN, FAHA, FAAN, Senior Vice President, Office of Science Operations

Prashant Nedungadi, PhD, Science and Medicine Advisor, Office of Science Operations

Jody Hundley, Production and Operations Manager, Scientific Publications, Office of Science Operations

* Former Task Force member; current member during the writing effort.

Footnotes

The American Heart Association requests that this document be cited as follows: Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139:e637–e697. doi: 10.1161/CIR.0000000000000602.

This document was approved by the American College of Cardiology Clinical Policy Approval Committee in May 2018, the American Heart Association Science Advisory and Coordinating Committee in June 2018, and the American Heart Association Executive Committee in July 2018.

The Comprehensive RWI Data Supplement table is available with this article at https://www.ahajournals.org/doi/suppl/10.1161/CIR.0000000000000602.

The online Data Supplement is available with this article at https://www.ahajournals.org/doi/suppl/10.1161/CIR.0000000000000602.

This article has been copublished in the Journal of the American College of Cardiology.

Copies: This document is available on the websites of the American College of Cardiology (www.acc.org) and the American Heart Association (professional.heart.org). A copy of the document is also available at https://professional.heart.org/statements by selecting the “Guidelines & Statements” button. To purchase additional reprints, call 843-216-2533 or e-mail .

The expert peer review of AHA-commissioned documents (eg, scientific statements, clinical practice guidelines, systematic reviews) is conducted by the AHA Office of Science Operations. For more on AHA statements and guidelines development, visit https://professional.heart.org/statements. Select the “Guidelines & Statements” drop-down menu near the top of the webpage, then click “Publication Development.”

Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the express permission of the American Heart Association. Instructions for obtaining permission are located at https://www.heart.org/permissions. A link to the “Copyright Permissions Request Form” appears in the second paragraph (https://www.heart.org/en/about-us/statements-and-policies/copyright-request-form).

https://www.ahajournals.org/journal/circ

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  • 2. Background and Pathophysiology

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  • 2.2. Severity of ACHD

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  • S2.2-4. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for management and quality measures.Ann Thorac Surg. 2013; 95:S1–S66.CrossrefMedlineGoogle Scholar
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  • S2.2-9. Dimopoulos K, Diller G-P, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.Circulation. 2008; 117:2320–28.LinkGoogle Scholar
  • S2.2-10. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease relates to adverse outcome.J Am Coll Cardiol. 2009; 54:2093–100.CrossrefMedlineGoogle Scholar
  • S2.2-11. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease.Eur Heart J. 2010; 31:595–601.CrossrefMedlineGoogle Scholar
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  • 2.3. The ACHD AP Classification

  • S2.3-1. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease.Eur Heart J. 2010; 31:1220–29.CrossrefMedlineGoogle Scholar
  • S2.3-2. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication.Circulation. 2005; 112:828–35.LinkGoogle Scholar
  • S2.3-3. Dimopoulos K, Diller G-P, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.Circulation. 2008; 117:2320–28.LinkGoogle Scholar
  • S2.3-4. Dimopoulos K, Okonko DO, Diller G-P, et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival.Circulation. 2006; 113:2796–802.LinkGoogle Scholar
  • S2.3-5 .Hebson CL, McCabe NM, Elder RW, et al. Hemodynamic phenotype of the failing Fontan in an adult population.Am J Cardiol. 2013; 112:1943–47.CrossrefMedlineGoogle Scholar
  • S2.3-. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122:868–75.LinkGoogle Scholar
  • S2.3-7. Khairy P, Harris L, Landzberg MJ, et al. Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.Circ Arrhythm Electrophysiol. 2008; 1:250–7.LinkGoogle Scholar
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  • S2.3-9. Müller J, Hager A, Diller G-P, et al. Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot.Int J Cardiol. 2015; 196:158–64.CrossrefMedlineGoogle Scholar
  • S2.3-10. Stefanescu A, Macklin EA, Lin E, et al. Usefulness of the Seattle Heart Failure Model to identify adults with congenital heart disease at high risk of poor outcome.Am J Cardiol. 2014; 113:865–70.CrossrefMedlineGoogle Scholar
  • S2.3-11. Giannakoulas G, Dimopoulos K, Engel R, et al. Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors.Am J Cardiol. 2009; 103:1445–50.CrossrefMedlineGoogle Scholar
  • S2.3-12. Giannakoulas G, Dimopoulos K, Bolger AP, et al. Usefulness of natriuretic peptide levels to predict mortality in adults with congenital heart disease.Am J Cardiol. 2010; 105:869–73.CrossrefMedlineGoogle Scholar
  • S2.3-13. Khairy P, Fernandes SM, Mayer JE, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.Circulation. 2008; 117:85–92.LinkGoogle Scholar
  • S2.3-14. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot.Circulation. 2008; 117:363–70.LinkGoogle Scholar
  • S2.3-15. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease relates to adverse outcome.J Am Coll Cardiol. 2009; 54:2093–100.CrossrefMedlineGoogle Scholar
  • S2.3-16. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease.Eur Heart J. 2010; 31:595–601.CrossrefMedlineGoogle Scholar
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  • 3. General Principles

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  • 3.2. Access to Care

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  • 3.3. Delivery of Care

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  • 3.4. Evaluation of Suspected and Known CHD

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  • 3.4.3. Echocardiography

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  • 3.4.4. CMR Imaging

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  • 3.4.5. Cardiac Computed Tomography

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  • 3.4.6. Cardiac Catheterization

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  • 3.4.7. Exercise Testing

  • S3.4.7-1. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication.Circulation. 2005; 112:828–35.LinkGoogle Scholar
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  • 3.5. Transition Education

  • S3.5-1. Mackie AS, Islam S, Magill-Evans J, et al. Healthcare transition for youth with heart disease: a clinical trial.Heart. 2014; 100:1113–18.CrossrefMedlineGoogle Scholar
  • S3.5-2. Goossens E, Van Deyk K, Zupancic N, et al. Effectiveness of structured patient education on the knowledge level of adolescents and adults with congenital heart disease.Eur J Cardiovasc Nurs. 2014; 13:63–70.CrossrefMedlineGoogle Scholar

  • 3.6. Exercise and Sports

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  • S3.6-8. Holloway TM, Chesssex C, Grace SL, et al. A call for adult congenital heart disease patient participation in cardiac rehabilitation.Int J Cardiol. 2011; 150:345–6.CrossrefMedlineGoogle Scholar
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  • 3.7. Mental Health and Neurodevelopmental Issues

  • S3.7-1. Kovacs AH, Saidi AS, Kuhl EA, et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence.Int J Cardiol. 2009; 137:158–64.CrossrefMedlineGoogle Scholar
  • S3.7-2. Kovacs AH, Sears SF, Saidi AS. Biopsychosocial experiences of adults with congenital heart disease: review of the literature.Am Heart J. 2005; 150:193–201.CrossrefMedlineGoogle Scholar
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  • S3.7-7. Karsdorp PA, Everaerd W, Kindt M, et al. Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis.J Pediatr Psychol. 2007; 32:527–41.CrossrefMedlineGoogle Scholar
  • S3.7-8. van der Rijken REA, Maassen BAM, Walk TLM, et al. Outcome after surgical repair of congenital cardiac malformations at school age.Cardiol Young. 2007; 17:64–71.CrossrefMedlineGoogle Scholar
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  • 3.8. Endocarditis Prevention

  • S3.8-1. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines.Circulation. 2014; 129:e521–643.LinkGoogle Scholar
  • S3.8-2. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population.Eur Heart J. 2011; 32:1926–34.CrossrefMedlineGoogle Scholar
  • S3.8-3. Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC)Eur Heart J. 2009; 30:2369–413.CrossrefMedlineGoogle Scholar
  • S3.8-4. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group.Circulation. 2007; 116:1736–54.LinkGoogle Scholar
  • S3.8-5. Allen U. Infective endocarditis: updated guidelines.Paediatr Child Health. 2010; 15:205–12.CrossrefMedlineGoogle Scholar

  • 3.9. Concomitant Syndromes

  • S3.9-1. van Engelen K, Topf A, Keavney BD, et al. 22q11.2 Deletion syndrome is under-recognised in adult patients with tetralogy of Fallot and pulmonary atresia.Heart. 2010; 96:621–4.CrossrefMedlineGoogle Scholar
  • S3.9-2. Fung WLA, Chow EWC, Webb GD, et al. Extracardiac features predicting 22q11.2 deletion syndrome in adult congenital heart disease.Int J Cardiol. 2008; 131:51–8.CrossrefMedlineGoogle Scholar
  • S3.9-3. Lin AE, Basson CT, Goldmuntz E, et al. Adults with genetic syndromes and cardiovascular abnormalities: clinical history and management.Genet Med. 2008; 10:469–94.CrossrefMedlineGoogle Scholar
  • S3.9-4. Richards AA, Garg V. Genetics of congenital heart disease.Curr Cardiol Rev. 2010; 6:91–7.CrossrefMedlineGoogle Scholar
  • S3.9-5. Kitsiou-Tzeli S, Kolialexi A, Fryssira H, et al. Detection of 22q11.2 deletion among 139 patients with Di George/velocardiofacial syndrome features.In Vivo. 2004; 18:603–8.MedlineGoogle Scholar
  • S3.9-6. Bassett AS, Chow EWC, Husted J, et al. Clinical features of 78 adults with 22q11 deletion syndrome.Am J Med Genet A. 2005; 138:307–13.CrossrefMedlineGoogle Scholar
  • S3.9-7. Barisic I, Boban L, Greenlees R, et al. Holt Oram syndrome: a registry-based study in Europe.Orphanet J Rare Dis. 2014; 9:156.CrossrefMedlineGoogle Scholar
  • S3.9-8. Pierpont MEM, Magoulas PL, Adi S, et al. Cardio-facio-cutaneous syndrome: clinical features, diagnosis, and management guidelines.Pediatrics. 2014; 134:e1149–62.CrossrefMedlineGoogle Scholar

  • 3.10. Noncardiac Medical Issues

  • S3.10-1. Wang A, Book WM, McConnell M, et al. Prevalence of hepatitis C infection in adult patients who underwent congenital heart surgery prior to screening in 1992.Am J Cardiol. 2007; 100:1307–09.CrossrefMedlineGoogle Scholar

  • 3.11. Noncardiac Surgery

  • S3.11-1. Ammash NM, Connolly HM, Abel MD, et al. Noncardiac surgery in Eisenmenger syndrome.J Am Coll Cardiol. 1999; 33:222–7.CrossrefMedlineGoogle Scholar
  • S3.11-2. Maxwell BG, Posner KL, Wong JK, et al. Factors contributing to adverse perioperative events in adults with congenital heart disease: a structured analysis of cases from the closed claims project.Congenit Heart Dis. 2015; 10:21–9.CrossrefMedlineGoogle Scholar
  • S3.11-3. Maxwell BG, Wong JK, Lobato RL. Perioperative morbidity and mortality after noncardiac surgery in young adults with congenital or early acquired heart disease: a retrospective cohort analysis of the National Surgical Quality Improvement Program database.Am Surg. 2014; 80:321–6.CrossrefMedlineGoogle Scholar
  • S3.11-4. Maxwell BG, Williams GD, Ramamoorthy C. Knowledge and attitudes of anesthesia providers about noncardiac surgery in adults with congenital heart disease.Congenit Heart Dis. 2014; 9:45–53.CrossrefMedlineGoogle Scholar
  • S3.11-5. Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery.J Cardiothorac Vasc Anesth. 2011; 25:320–34.CrossrefMedlineGoogle Scholar
  • S3.11-6. Maxwell BG, Wong JK, Kin C, et al. Perioperative outcomes of major noncardiac surgery in adults with congenital heart disease.Anesthesiology. 2013; 119:762–9.CrossrefMedlineGoogle Scholar
  • S3.11-7. Maxwell BG, Wong JK, Sheikh AY, et al. Heart transplantation with or without prior mechanical circulatory support in adults with congenital heart disease.Eur J Cardiothorac Surg. 2014; 45:842–6.CrossrefMedlineGoogle Scholar
  • S3.11-8. Rabbitts JA, Groenewald CB, Mauermann WJ, et al. Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation.Paediatr Anaesth. 2013; 23:180–7.CrossrefMedlineGoogle Scholar
  • S3.11-9. Mylotte D, Quenneville SP, Kotowycz MA, et al. Long-term cost-effectiveness of transcatheter versus surgical closure of secundum atrial septal defect in adults.Int J Cardiol. 2014; 172:109–14.CrossrefMedlineGoogle Scholar
  • S3.11-10. Warner MA, Lunn RJ, O’Leary PW, et al. Outcomes of noncardiac surgical procedures in children and adults with congenital heart disease. Mayo Perioperative Outcomes Group.Mayo Clin Proc. 1998; 73:728–34.CrossrefMedlineGoogle Scholar

  • 3.12. Pregnancy, Reproduction, and Sexual Health

  • S3.12.1-1. Balint OH, Siu SC, Mason J, et al. Cardiac outcomes after pregnancy in women with congenital heart disease.Heart. 2010; 96:1656–61.CrossrefMedlineGoogle Scholar
  • S3.12.1-2. Roos-Hesselink JW, Ruys TPE, Stein JI, et al. Outcome of pregnancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology.Eur Heart J. 2013; 34:657–65.CrossrefMedlineGoogle Scholar
  • S3.12.1-3. Ouyang DW, Khairy P, Fernandes SM, et al. Obstetric outcomes in pregnant women with congenital heart disease.Int J Cardiol. 2010; 144:195–9.CrossrefMedlineGoogle Scholar
  • S3.12.1-4. Egidy Assenza G, Cassater D, Landzberg M, et al. The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot.Int J Cardiol. 2013; 168:1847–52.CrossrefMedlineGoogle Scholar
  • S3.12.1-5. Chan WS, Anand S, Ginsberg JS. Anticoagulation of pregnant women with mechanical heart valves: a systematic review of the literature.Arch Intern Med. 2000; 160:191–6.CrossrefMedlineGoogle Scholar
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  • S3.12.1-7. Siu SC, Sermer M, Colman JM, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease.Circulation. 2001; 104:515–21.LinkGoogle Scholar
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  • S3.12.1-9. Gill HK, Splitt M, Sharland GK, et al. Patterns of recurrence of congenital heart disease: an analysis of 6640 consecutive pregnancies evaluated by detailed fetal echocardiography.J Am Coll Cardiol. 2003; 42:923–9.CrossrefMedlineGoogle Scholar
  • S3.12.1-10. Ohuchi H, Tanabe Y, Kamiya C, et al. Cardiopulmonary variables during exercise predict pregnancy outcome in women with congenital heart disease.Circ J. 2013; 77:470–6.CrossrefMedlineGoogle Scholar
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  • S3.12.1-13. Holland BJ, Myers JA, Woods CR. Prenatal diagnosis of critical congenital heart disease reduces risk of death from cardiovascular compromise prior to planned neonatal cardiac surgery: a meta-analysis.Ultrasound Obstet Gynecol. 2015; 45:631–8.CrossrefMedlineGoogle Scholar

  • 3.12.2. Contraception

  • S3.12.2-1. Kovacs AH, Harrison JL, Colman JM, et al. Pregnancy and contraception in congenital heart disease: what women are not told.J Am Coll Cardiol. 2008; 52:577–8.CrossrefMedlineGoogle Scholar
  • S3.12.2-2. Vigl M, Kaemmerer M, Seifert-Klauss V, et al. Contraception in women with congenital heart disease.Am J Cardiol. 2010; 106:1317–21.CrossrefMedlineGoogle Scholar
  • S3.12.2-3. Kaemmerer M, Vigl M, Seifert-Klauss V, et al. Counseling reproductive health issues in women with congenital heart disease.Clin Res Cardiol. 2012; 101:901–7.CrossrefMedlineGoogle Scholar
  • S3.12.2-4. Lidegaard Ø, Løkkegaard E, Svendsen AL, et al. Hormonal contraception and risk of venous thromboembolism: national follow-up study.BMJ. 2009; 339:b2890.CrossrefMedlineGoogle Scholar
  • S3.12.2-5. Pijuan-Domènech A, Baró-Mariné F, Rojas-Torrijos M, et al. Usefulness of progesterone-only components for contraception in patients with congenital heart disease.Am J Cardiol. 2013; 112:590–3.CrossrefMedlineGoogle Scholar

  • 3.13. Heart Failure and Transplant

  • S3.13.1-1. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease.Eur Heart J. 2010; 31:1220–29.CrossrefMedlineGoogle Scholar
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  • S3.13.1-3. Oechslin EN, Harrison DA, Connelly MS, et al. Mode of death in adults with congenital heart disease.Am J Cardiol. 2000; 86:1111–16.CrossrefMedlineGoogle Scholar
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  • 3.13.2. Heart Transplant

  • S3.13.2-1. Everitt MD, Donaldson AE, Stehlik J, et al. Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS).J Heart Lung Transplant. 2011; 30:395–401.CrossrefMedlineGoogle Scholar
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  • S3.13.2-6. Goerler H, Simon A, Gohrbandt B, et al. Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience.Eur J Cardiothorac Surg. 2007; 32:926–31.CrossrefMedlineGoogle Scholar
  • S3.13.2-7. Burchill LJ, Edwards LB, Dipchand AI, et al. Impact of adult congenital heart disease on survival and mortality after heart transplantation.J Heart Lung Transplant. 2014; 33:1157–63.CrossrefMedlineGoogle Scholar
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  • S3.13.2-9. Seddio F, Gorislavets N, Iacovoni A, et al. Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience.Eur J Cardiothorac Surg. 2013; 43:605–11.CrossrefMedlineGoogle Scholar
  • S3.13.2-10. Alshawabkeh LI, Hu N, Carter KD, et al. Wait-list outcomes for adults with congenital heart disease listed for heart transplantation in the US.J Am Coll Cardiol. 2016; 68:908–17.CrossrefMedlineGoogle Scholar
  • S3.13.2-11. Krishnamurthy Y, Cooper LB, Lu D, et al. Trends and outcomes of patients with adult congenital heart disease and pulmonary hypertension listed for orthotopic heart transplantation in the United States.J Heart Lung Transplant. 2016; 35:619–24.CrossrefMedlineGoogle Scholar
  • S3.13.2-12. Harper AR, Crossland DS, Perri G, et al. Is alternative cardiac surgery an option in adults with congenital heart disease referred for thoracic organ transplantation?Eur J Cardiothorac Surg. 2013; 43:344–51.CrossrefMedlineGoogle Scholar

  • 3.14. Palliative Care

  • S3.14-1. Greutmann M, Tobler D, Colman JM, et al. Facilitators of and barriers to advance care planning in adult congenital heart disease.Congenit Heart Dis. 2013; 8:281–8.CrossrefMedlineGoogle Scholar
  • S3.14-2. Tobler D, Greutmann M, Colman JM, et al. Knowledge of and preference for advance care planning by adults with congenital heart disease.Am J Cardiol. 2012; 109:1797–800.CrossrefMedlineGoogle Scholar
  • S3.14-3. Tobler D, Greutmann M, Colman JM, et al. End-of-life in adults with congenital heart disease: a call for early communication.Int J Cardiol. 2012; 155:383–7.CrossrefMedlineGoogle Scholar

  • 3.15. Cyanosis

  • S3.15-1. Broberg CS, Van Woerkom RC, Swallow E, et al. Lung function and gas exchange in Eisenmenger syndrome and their impact on exercise capacity and survival.Int J Cardiol. 2014; 171:73–7.CrossrefMedlineGoogle Scholar
  • S3.15-2. Broberg CS, Uebing A, Cuomo L, et al. Adult patients with Eisenmenger syndrome report flying safely on commercial airlines.Heart. 2007; 93:1599–603.CrossrefMedlineGoogle Scholar
  • S3.15-3. Perloff JK, Child JS, Aboulhosn J. Congenital Heart Disease in Adults. 3rd ed. Philadelphia, PA: Saunders/Elsevier, 2009.Google Scholar

  • 3.16. Pharmacological Therapy for ACHD

  • S3.16-1. Yancy CW, Jessup M, Bozkurt B, et al. 2017 ACC/AHA/HFSA focused update of the 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Failure Society of America.Circulation. 2017; 136:e137–61.LinkGoogle Scholar
  • S3.16-2. Doughan ARK, McConnell ME, Book WM. Effect of beta blockers (carvedilol or metoprolol XL) in patients with transposition of great arteries and dysfunction of the systemic right ventricle.Am J Cardiol. 2007; 99:704–6.CrossrefMedlineGoogle Scholar
  • S3.16-3. Dos L, Pujadas S, Estruch M, et al. Eplerenone in systemic right ventricle: double blind randomized clinical trial. The EVEDES Study.Int J Cardiol. 2013; 168:5167–73.CrossrefMedlineGoogle Scholar
  • S3.16-4. van der Bom T, Winter MM, Bouma BJ, et al. Effect of valsartan on systemic right ventricular function: a double-blind, randomized, placebo-controlled pilot trial.Circulation. 2013; 127:322–30.LinkGoogle Scholar
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  • S3.16-6. Babu-Narayan SV, Uebing A, Davlouros PA, et al. Randomised trial of ramipril in repaired tetralogy of Fallot and pulmonary regurgitation: the APPROPRIATE study (Ace inhibitors for Potential PRevention Of the deleterious effects of Pulmonary Regurgitation In Adults with repaired TEtralogy of Fallot)Int J Cardiol. 2012; 154:299–305.CrossrefMedlineGoogle Scholar
  • S3.16-7. Lester SJ, McElhinney DB, Viloria E, et al. Effects of losartan in patients with a systemically functioning morphologic right ventricle after atrial repair of transposition of the great arteries.Am J Cardiol. 2001; 88:1314–16.CrossrefMedlineGoogle Scholar
  • S3.16-8. Hechter SJ, Fredriksen PM, Liu P, et al. Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure.Am J Cardiol. 2001; 87:660–663; A11.CrossrefMedlineGoogle Scholar
  • S3.16-9. Therrien J, Provost Y, Harrison J, et al. Effect of angiotensin receptor blockade on systemic right ventricular function and size: a small, randomized, placebo-controlled study.Int J Cardiol. 2008; 129:187–92.CrossrefMedlineGoogle Scholar
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  • S3.16-12. Hebert A, Mikkelsen UR, Thilen U, et al. Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study.Circulation. 2014; 130:2021–30.LinkGoogle Scholar
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  • 4. Specific Lesions

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  • 4.1.2. Anomalous Pulmonary Venous Connections

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  • 4.1.3. Ventricular Septal Defect

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  • 4.1.4. Atrioventricular Septal Defect

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  • 4.1.5. Patent Ductus Arteriosus

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  • 4.2. Left-Sided Obstructive Lesions

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  • 4.2.2. Congenital Mitral Stenosis

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  • 4.2.3. Subaortic Stenosis

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  • 4.2.4. Congenital Valvular Aortic Stenosis

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  • 4.2.4.1. Turner Syndrome

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  • 4.2.5. Supravalvular Aortic Stenosis

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  • 4.2.6. Coarctation of the Aorta

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  • 4.3. Right-Sided Lesions

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  • 4.3.2. Branch and Peripheral Pulmonary Stenosis

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  • 4.3.3. Double-Chambered Right Ventricle

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  • 4.3.4. Ebstein Anomaly

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  • 4.3.5. Tetralogy of Fallot

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  • 4.3.6. Right Ventricle–to-Pulmonary Artery Conduit

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  • 4.4. Complex Lesions

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  • 4.4.1.2. Transposition of the Great Arteries With Arterial Switch

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  • 4.4.1.3. Congenitally Corrected Transposition of the Great Arteries

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  • 4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle)

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  • 4.4.4. Severe PAH and Eisenmenger Syndrome

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  • S4.4.4.1-3. Duffels MGJ, Engelf