Vol 16, No 7 | Circulation: Cardiovascular Imaging

In This Issue of the Journal

Robert J. Gropler

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 521–522,

originally publishedJuly 18, 2023,

https://doi.org/10.1161/CIRCIMAGING.123.015829

Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 523–535,

originally publishedJuly 11, 2023,

https://doi.org/10.1161/CIRCIMAGING.123.015259

Abstract Export Citations PDF/EPUB

BACKGROUND:
Apo AI amyloidosis (AApoAI) and Apo AIV amyloidosis (AApoAIV) are rare but increasingly recognized causes of cardiac amyloidosis (CA). We sought to define the cardiac phenotype in AApoAI and AApoAIV using multimodality imaging.

METHODS:
We identified all patients with AApoAI and AApoAIV assessed at our center between 2000 and 2021, and 2 cohorts of patients with immunoglobulin light-chain amyloidosis (AL) and transthyretin amyloidosis matched for age, sex, and cardiac involvement.

RESULTS:
Forty-five patients had AApoAI, 13 (29%) of whom had cardiac involvement, 32 (71%) renal involvement, 28 (62%) splenic involvement, 27 (60%) hepatic involvement, and 7 (16%) laryngeal involvement. AApoAI-CA commonly presented with heart failure (n=8, 62%) or dysphonia (n=7, 54%). The Arg173Pro variant universally caused cardiac and laryngeal involvement (n=7, 100%). AApoAI-CA was associated with right-sided involvement, with a thicker right ventricular free wall (8.6±1.9 versus 6.3±1.3 mm versus 7.7±1.2 mm, P=0.004), greater incidence of tricuspid stenosis (4 [31%] versus 0 [0%] versus 0 [0%], P=0.012) and tricuspid regurgitation (6 [46%] versus 1 [8%] versus 2 [15%], P=0.048) than AL-CA and transthyretin CA. Twenty-one patients had AApoAIV, and cardiac involvement was more common than in AApoAI (15 [71%] versus 13 [29%], P=0.001). AApoAIV-CA most commonly presented with heart failure (n=12, 80%), and a lower median estimated glomerular filtration rate than AL-CA and transthyretin CA (36 mL/[min·1.73 m²] versus 65 mL/[min·1.73 m²] versus 63 mL/[min·1.73 m²], P<0.001). All AApoAIV-CA patients had classical CA features on echocardiography/cardiac magnetic resonance, including an apical-sparing strain pattern, which was less common in AApoAI-CA (15 [100%] versus 7 [54%], P=0.003), whereas cardiac uptake on bone scintigraphy was less common in AApoAIV-CA than AApoAI-CA (all grade 1) (14% versus 82%, P<0.001). Patients with AApoAI and AApoAIV had a good prognosis (median survival >172 and >30 months, respectively), and a lower risk of mortality than matched patients with AL-amyloidosis (AL versus AApoAI: hazard ratio, 4.54 [95% CI, 2.02–10.14]; P<0.001; AL versus AApoAIV: hazard ratio, 3.07 [95% CI, 1.27–7.44]; P=0.013).

CONCLUSIONS:
Dysphonia, multisystem involvement, or right-sided cardiac disease should raise suspicion of AApoAI-CA. AApoAIV-CA presents most commonly with heart failure and always displays classical CA imaging features, mimicking common forms of CA. Both AApoAI and AApoAIV are associated with a good prognosis and a lower risk of mortality than matched patients with AL-amyloidosis. Graphical Abstract

Lack of Incremental Prognostic Value of Pericoronary Adipose Tissue Computed Tomography Attenuation Beyond Coronary Artery Disease Reporting and Data System for Major Adverse Cardiovascular Events in Patients With Acute Chest Pain

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 536–544,

originally publishedJune 29, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.015120

Abstract Export Citations PDF/EPUB

BACKGROUND:
Pericoronary adipose tissue (PCAT) and Coronary Artery Disease Reporting and Data System (CAD-RADS) category had prognostic values for major adverse cardiovascular events (MACEs). However, little is known about the difference between CAD-RADS and PCAT computed tomography (CT) attenuation for predicting MACEs. This study was to compare the prognostic value of PCAT and CAD-RADS for MACEs in patients with acute chest pain.

METHODS:
Between January 2010 and December 2021, all consecutive emergency patients with acute chest pain referred for coronary computed tomography angiography were enrolled in this retrospective study. MACEs included unstable angina requiring hospitalization, coronary revascularization, nonfatal myocardial infarction, and all-cause death. Patients’ clinical characteristics, CAD-RADS, and PCAT CT attenuation were used to evaluate risk factors of MACEs using multivariable Cox regression analysis.

RESULTS:
A total of 1313 patients were evaluated (mean age, 57.13±12.57 years; 782 men). During a median follow-up of 38 months, 142 of the 1313 patients (10.81%) experienced MACEs. Multivariable Cox regression analysis showed that CAD-RADS categories 2, 3, 4, 5 (hazard ratio range, 2.286–8.325; all P<0.005) and right coronary artery PCAT CT attenuation (hazard ratio, 1.033; P=0.006) were independent predictors of MACEs after adjusting for clinical risk factors. The C statistics revealed that CAD-RADS improved risk stratification compared with PCAT CT alone (C-index, 0.760 versus 0.712; P=0.036). However, the benefit of right coronary artery PCAT CT attenuation combined with CAD-RADS was not significant compared with CAD-RADS alone (0.777 versus 0.760; P=0.129).

CONCLUSIONS:
Right coronary artery PCAT CT attenuation and CAD-RADS were independent predictors of MACEs. However, no incremental prognostic value of right coronary artery PCAT CT attenuation beyond CAD-RADS was detected for MACEs in patients with acute chest pain. Graphical Abstract

Impact of Diabetes on Myocardial Fibrosis in Patients With Hypertension: The REMODEL Study

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 545–553,

originally publishedJuly 11, 2023,

https://doi.org/10.1161/CIRCIMAGING.123.015051

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BACKGROUND:
Compared with patients with hypertension only, those with hypertension and diabetes (HTN/DM) have worse prognosis. We aimed to characterize morphological differences between hypertension and HTN/DM using cardiovascular magnetic resonance; and compare differentially expressed proteins associated with myocardial fibrosis using high throughput multiplex assays.

METHODS:
Asymptomatic patients underwent cardiovascular magnetic resonance: 438 patients with hypertension (60±8 years; 59% males) and 167 age- and sex-matched patients with HTN/DM (60±10 years; 64% males). Replacement myocardial fibrosis was defined as nonischemic late gadolinium enhancement on cardiovascular magnetic resonance. Extracellular volume fraction was used as a marker of diffuse myocardial fibrosis. A total of 184 serum proteins (Olink Target Cardiovascular Disease II and III panels) were measured to identify unique signatures associated with myocardial fibrosis in all patients.

RESULTS:
Despite similar left ventricular mass (P=0.344) and systolic blood pressure (P=0.086), patients with HTN/DM had increased concentricity and worse multidirectional strain (P<0.001 for comparison of all strain measures) compared to hypertension only. Replacement myocardial fibrosis was present in 28% of patients with HTN/DM compared to 16% of those with hypertension (P<0.001). NT-proBNP (N-terminal pro-B-type natriuretic peptide) was the only protein differentially upregulated in hypertension patients with replacement myocardial fibrosis and independently associated with extracellular volume. In patients with HTN/DM, GDF-15 (growth differentiation factor 15) was independently associated with replacement myocardial fibrosis and extracellular volume. Ingenuity Pathway Analysis demonstrated a strong association between increased inflammatory response/immune cell trafficking and myocardial fibrosis in patients with HTN/DM.

CONCLUSIONS:
Adverse cardiac remodeling was observed in patients with HTN/DM. The novel proteomic signatures and associated biological activities of increased immune and inflammatory response may partly explain these observations. Graphical Abstract

Predictors of Major Adverse Cardiovascular Events in Patients With Moderate Aortic Stenosis: Implications for Aortic Valve Replacement

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 557–565,

originally publishedJune 29, 2023,

https://doi.org/10.1161/CIRCIMAGING.123.015475

Abstract Export Citations PDF/EPUB

BACKGROUND:
Although the prognosis and management of severe aortic stenosis has been extensively studied, the risk stratification and outcomes of patients with moderate aortic stenosis remain elusive.

METHODS:
This study included 674 patients from the Cleveland Clinic Health System with moderate aortic stenosis (aortic valve area, 1–1.5 cm²; mean gradient, 20–40 mm Hg; and peak velocity <4 m/s) and an NT-proBNP (N-terminal pro-B-type natriuretic peptide) level within 3 months of index diagnosis. The primary outcome of major adverse cardiovascular events (defined as the composite outcome of progression to severe aortic stenosis requiring aortic valve replacement, heart failure hospitalization, or death) was extracted from the electronic medical record.

RESULTS:
The mean age was 75.3±12 years, and 57% were men. During a median follow-up of 316 days, the composite end point occurred in 305 patients. There were 132 (19.6%) deaths, 144 (21.4%) heart failure hospitalizations, and 114 (16.9%) patients underwent aortic valve replacement. Elevated NT-proBNP (1.41 [95% CI, 1.01–1.95]; P=0.048), diabetes (1.46 [95% CI, 1.08–1.96]; P=0.01), elevated averaged mitral valve E/e′ ratio (hazard ratio, 1.57 [95% CI, 1.18–2.10]; P<0.01), and presence atrial fibrillation at the time of index echocardiogram (hazard ratio, 1.83 [95% CI, 1.15–2.91]; P=0.01) were each independently associated with an increased hazard for the composite outcome and when taken collectively, each of these factors incrementally increased risk.

CONCLUSIONS:
These results further elucidate the relatively poor short-medium term outcomes and risk stratification of patients with moderate aortic stenosis, supporting randomized trials assessing the efficacy of transcatheter aortic valve replacement in this population. Graphical Abstract

Association of Tricuspid Regurgitation With Outcome in Acute Heart Failure

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 566–576,

originally publishedJune 29, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.014988

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BACKGROUND:
Tricuspid regurgitation (TR) is common in chronic heart failure (HF) and is associated with negative prognosis. However, evidence on prognostic implications of TR in acute HF is lacking. We sought to investigate the association between TR and mortality and the interaction with pulmonary hypertension (PH) in patients admitted for acute HF.

METHODS:
We enrolled 1176 consecutive patients with a primary diagnosis of acute HF and with available noninvasive estimation of TR and pulmonary arterial systolic pressure.

RESULTS:
Moderate-severe TR was present in 352 patients (29.9%) and was associated with older age and more comorbidities. The prevalence of PH (ie, pulmonary arterial systolic pressure >40 mm Hg), right ventricular dysfunction, and mitral regurgitation was higher in moderate-severe TR. At 1 year, 184 (15.6%) patients died. Moderate-severe TR was associated with higher 1-year mortality risk after adjustment for other echocardiographic parameters (pulmonary arterial systolic pressure, left ventricle ejection fraction, right ventricular dysfunction, mitral regurgitation, left and right atrial indexed volumes; hazard ratio, 1.718; P=0.009), and the association with outcome was maintained when clinical variables (eg, natriuretic peptides, serum creatinine and urea, systolic blood pressure, atrial fibrillation) were added to the multivariable model (hazard ratio, 1.761; P=0.024). The association between moderate-severe TR and outcome was consistent in patients with versus without PH, with versus without right ventricular dysfunction, and with versus without left ventricle ejection fraction <50%. Patients with coexistent moderate-severe TR and PH had 3-fold higher 1-year mortality risk compared with patients with no TR or PH (hazard ratio, 3.024; P<0.001).

CONCLUSIONS:
In patients hospitalized for acute HF, the severity of TR is associated with 1-year survival, regardless of the presence of PH. The coexistence of moderate-severe TR and estimated PH was associated with a further increase in mortality risk. Our data must be interpreted in the context of potential underestimation of pulmonary arterial systolic pressure in patients with severe TR. Graphical Abstract

Diagnostic Performance and Safety of a Novel Ferumoxytol-Enhanced Coronary Magnetic Resonance Angiography

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 580–590,

originally publishedJuly 18, 2023,

https://doi.org/10.1161/CIRCIMAGING.123.015404

Abstract Export Citations PDF/EPUB

BACKGROUND:
Currently, noninvasive arteriography for the diagnosis of coronary artery disease is clinically limited to the computed tomography scanning, where patients have to be exposed to the radiation and risks associated with iodinated contrast. We aimed to investigate the diagnostic performance and safety of a novel ferumoxytol-enhanced coronary magnetic resonance angiography (CMRA) in patients with suspected coronary artery disease.

METHODS:
Thirty patients, 19 males, with a median age of 63 years old, and 17 with renal insufficiency, who were scheduled for invasive coronary angiography, were enrolled. Ferumoxytol was administered intravenously with a dose of 3 mg/kg during CMRA. Images were acquired with an ECG-triggered, navigator-gated, inversion recovery-prepared 3D fast low-angle shot sequence, and the image quality was assessed by a 4-point scale. Eighteen-segment coronary artery model was adopted to evaluate the visibility of the coronary arteries, and the image quality and stenosis were evaluated in nine segments. The diagnostic performance of CMRA is described as sensitivity, specificity, positive and negative predictive values, and accuracy with the invasive coronary angiography results as reference. The patients’ vital signs were monitored during CMRA, and their hepatic and renal functions were followed up for 3 months to evaluate the safety of ferumoxytol.

RESULTS:
Two hundred fifty-two of the 270 study segments were identified by CMRA, and their quality score reached 3.6±0.7. Referring to the invasive coronary angiography results, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of ferumoxytol-enhanced CMRA reached 100.0%, 66.7%, 92.3%, 100.0%, and 93.3% respectively in patient-based analysis; 91.4%, 90.9%, 86.5%, 94.3%, and 91.1%, respectively in vessel-based analysis; and 92.3%, 96.7%, 83.7%, 98.6%, and 96.0%, respectively in segment-based analysis. No ferumoxytol-related adverse event was observed during the 3-month follow-up.

CONCLUSIONS:
Ferumoxytol-enhanced CMRA demonstrated good diagnostic performance and excellent safety in the diagnosis of significant coronary stenosis, providing an alternative to coronary computed tomography angiography for the diagnosis of coronary artery disease.

REGISTRATION:
URL: https://www.clinicaltrials.gov; Unique identifier: NCT05032937. Graphical Abstract

Righting on the Wall: A Case of Medically Managed Right Ventricular Mural Endocarditis

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 591–593,

originally publishedMay 12, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.014721

Application of 3-Dimensional Fetal Echocardiography in the Detection of Hidden Extended Ventricular Septal Defect

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 594–596,

originally publishedMarch 6, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.014658

Comprehensive Multimodality Imaging of Congenitally Corrected Transposition of the Great Arteries Incidentally Found to Have Coronary Ischemia

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 597–600,

originally publishedApril 24, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.015158

Four-Dimensional Flow Magnetic Resonance Imaging Features of a Platypnea-Orthodeoxia Syndrome Caused by a Patent Foramen Ovale

Circulation: Cardiovascular Imaging. 2023 | Volume 16, Issue 7: 601–603,

originally publishedApril 24, 2023,

https://doi.org/10.1161/CIRCIMAGING.122.014722

Table of Contents

Editor’s Note

In This Issue of the Journal

Original Articles

Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

Lack of Incremental Prognostic Value of Pericoronary Adipose Tissue Computed Tomography Attenuation Beyond Coronary Artery Disease Reporting and Data System for Major Adverse Cardiovascular Events in Patients With Acute Chest Pain

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

Impact of Diabetes on Myocardial Fibrosis in Patients With Hypertension: The REMODEL Study

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

Editorial

Early Appearance of Myocardial Fibrosis in Restrictive Diabetic Cardiomyopathy

Predictors of Major Adverse Cardiovascular Events in Patients With Moderate Aortic Stenosis: Implications for Aortic Valve Replacement

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

Association of Tricuspid Regurgitation With Outcome in Acute Heart Failure

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

Editorial

Importance of Tricuspid Regurgitation—Time to Move on

Diagnostic Performance and Safety of a Novel Ferumoxytol-Enhanced Coronary Magnetic Resonance Angiography

BACKGROUND:

METHODS:

RESULTS:

CONCLUSIONS:

REGISTRATION:

Cardiovascular Images

Righting on the Wall: A Case of Medically Managed Right Ventricular Mural Endocarditis

Application of 3-Dimensional Fetal Echocardiography in the Detection of Hidden Extended Ventricular Septal Defect

Comprehensive Multimodality Imaging of Congenitally Corrected Transposition of the Great Arteries Incidentally Found to Have Coronary Ischemia

Four-Dimensional Flow Magnetic Resonance Imaging Features of a Platypnea-Orthodeoxia Syndrome Caused by a Patent Foramen Ovale

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