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Aims Differentiating cardiac amyloidosis (CA) subtypes is important considering the significantly different therapies for transthyretin (ATTR)-CA and light chain (AL)-CA. Therefore, an echocardiographic method to distinguish ATTR-CA from AL-CA would provide significant value. We assessed a novel echocardiographic pixel intensity method to quantify myocardial calcification to differentiate ATTR-CA from phenocopies of CA and from AL-CA, specifically. Methods and results 167 patients with ATTR-CA (n = 53), AL-CA (n = 32), hypertrophic cardiomyopathy (n = 37), and advanced chronic kidney disease (n = 45) were retrospectively evaluated. The septal reflectivity ratio (SRR) was measured as the average pixel intensity of the visible anterior septal wall divided by the average pixel intensity of the visible posterior lateral wall. SRR and other myocardial strain-based echocardiographic measures were evaluated with receiver operator characteristic analysis to evaluate accuracy in distinguishing ATTR-CA from AL-CA and other forms of left ventricular hypertrophy. Mean SRR was significantly higher in the ATTR-CA cohort compared to the other cohorts (P < 0.001). SRR demonstrated the largest area under the curve (AUC) (0.91, P < 0.001) for distinguishing ATTR from all other cohorts and specifically for distinguishing ATTR-CA from AL-CA (AUC = 0.90, P < 0.001, specificity 96%, and sensitivity 63%). There was excellent inter- and intra-operator reproducibility with an ICC of 0.91 (P < 0.001) and 0.89 (P < 0.001), respectively. Conclusion The SRR is a reproducible and robust parameter for differentiating ATTR-CA from other phenocopies of CA and specifically ATTR-CA from AL-CA. [ABSTRACT FROM AUTHOR]

Background: Visual assessment of the left atrial appendage (LAA) by echocardiography for the presence of thrombus is inherently qualitative. However, whether quantitative assessments can provide increased value over qualitative assessment has not been thoroughly examined. Methods: One hundred and thirty‐eight patients (mean age 59 ± 13 years, 70% male) undergoing transesophageal echocardiography prior to pulmonary vein isolation or electrical cardioversion were retrospectively studied. LAA were examined by two expert readers and identified as thrombus, sludge, spontaneous echocardiograph contrast, or normal. LAA were then separately examined to calculate a gain‐independent ratio between the average pixel density of the LAA cavity and that of the LAA wall (C/W ratio). Results: C/W ratio was significantly related with qualitative LAA analysis (p < 0.0001) and with thromboembolic events (OR 1.60, 95% CI 1.095–2.347, p = 0.02). The C/W ratio (AUC 0.73, 95% CI 0.60–0.86) was a reliable predictor for future thromboembolic events when compared to expert reader LAA assessment (Expert Reader 1 AUC = 0.72, 95% CI 0.53–0.90; Expert Reader 2 AUC = 0.69). Conclusions: The C/W ratio may be a complementary method to adjudicate thromboembolic risk in patients with AF that is readily quantifiable at time of TEE. [ABSTRACT FROM AUTHOR]

Introduction: Unicuspid aortic valve (UAV) is a rare disease typically diagnosed by surgical inspection. This study assessed the natural history and outcomes of pathologically confirmed UAV. Methods: The institutional databases (echocardiographic, pathology) and medical record were searched for demographic, echocardiographic and outcome data of all UAV diagnoses. Only UAVs pathologically confirmed post-surgery or at autopsy were included. Results: 81 patients (mean±SD age, 29 ±18 years (range 0-81); male sex, 62%) were identified with pathologically confirmed UAV (83% post-surgery, 17% at autopsy). 67% had ascending aorta dilatation. Aortic stenosis (AS) or aortic regurgitation (AR) of any degree, mixed AR/AS, pure AS, and pure AR was present in 77%, 74%, 58%, 21 %, and 18% of patients, respectively (Table 1). Additional abnormalities included patent foramen ovale (5%), patent ductus arteriosus (5%) and coarctation (4%). At follow up (median 9.9 years) 67 (83%) and 34 (42%) patients had aortic valve surgery (mean ±SD age at surgery, 37 ± 14 years (range 0.1-81); 85% aortic valve replacement (AVR), 4% Ross procedure, 4% heart transplant, 6% unknown type) and ascending aorta surgery (mean ±SD age at aorta surgery, 43 ± 11 years (range 19-67); 82% aortic graft, 9% aortic repair, 9% Ross procedure), respectively (Table 1). 13% of patients had valvotomy prior to surgery. Mean ±SD time to AVR and aorta surgery from diagnosis was 3 ± 9 years and 8 ± 13 years, respectively. Surgical indications were AS (44%), mixed AS/AR (31%), AR (12%) and ascending aortic dilatation (7%). AVR alone and AVR with aorta surgery had similar outcomes (post-surgical survival 100% and 92% for both at 5 and 10 years, respectively (p = 0.32)). Conclusions: To our knowledge this is the largest and most comprehensive natural history and outcomes study of pathologically confirmed UAV. AS and AR were common in UAV and AS was the most common surgery indication. AVR alone and AVR with aorta surgery had similar outcomes. [ABSTRACT FROM AUTHOR]

Aims: Differentiating cardiac amyloidosis (CA) subtypes is important considering the significantly different therapies for transthyretin (ATTR)-CA and light chain (AL)-CA. Therefore, an echocardiographic method to distinguish ATTR-CA from AL-CA would provide significant value. We assessed a novel echocardiographic pixel intensity method to quantify myocardial calcification to differentiate ATTR-CA from phenocopies of CA and from AL-CA, specifically., Methods and Results: 167 patients with ATTR-CA (n = 53), AL-CA (n = 32), hypertrophic cardiomyopathy (n = 37), and advanced chronic kidney disease (n = 45) were retrospectively evaluated. The septal reflectivity ratio (SRR) was measured as the average pixel intensity of the visible anterior septal wall divided by the average pixel intensity of the visible posterior lateral wall. SRR and other myocardial strain-based echocardiographic measures were evaluated with receiver operator characteristic analysis to evaluate accuracy in distinguishing ATTR-CA from AL-CA and other forms of left ventricular hypertrophy. Mean SRR was significantly higher in the ATTR-CA cohort compared to the other cohorts (P < 0.001). SRR demonstrated the largest area under the curve (AUC) (0.91, P < 0.001) for distinguishing ATTR from all other cohorts and specifically for distinguishing ATTR-CA from AL-CA (AUC = 0.90, P < 0.001, specificity 96%, and sensitivity 63%). There was excellent inter- and intra-operator reproducibility with an ICC of 0.91 (P < 0.001) and 0.89 (P < 0.001), respectively., Conclusion: The SRR is a reproducible and robust parameter for differentiating ATTR-CA from other phenocopies of CA and specifically ATTR-CA from AL-CA., Competing Interests: Conflict of interests: V.A. discloses speaking honoraria from Abbott Laboratories. A.N. discloses speaking honoraria from Abbott Laboratories and Edwards Lifesciences. J.T. discloses consulting roles with Abbott Laboratories, Caption Health, GE Health care, and egnite. R.W. discloses consulting role with Ionetix Corporation. R.W. assisted in the approval of final manuscript. S.S. discloses speaking, advising, or consulting roles with American Medical Association, Aria CV, Inc., Axon Therapies Inc., Bayer AG, Boehringer Ingelheim, Boehringer Ingelheim GmbH, Boston Scientific Corporation (includes Guidant Corporation), Edwards Lifesciences, LLC, Heart Failure Society of America, Inc. (HFSA), IMARA Inc., Impulse Dynamics, Ionis Pharmaceuticals, Inc., Kikaku International (USA) Corporation, Merck Sharp & Dohme Corporation, Novartis, Novo Nordisk A/S, Pri-Med Institute, LLC, Regeneron Pharmaceuticals, Inc., Rivus Pharmaceuticals, Roche Diagnostics Corporation, Shifamed LLC, Tenaya Therapeutics, The Lynx Group (TLG), Total CME, (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Objective: In the United States, racial minorities are underrepresented among patients receiving transcatheter aortic valve replacement (TAVR) and data regarding their outcomes is limited. Global longitudinal strain (GLS) is a measure left ventricular function and has independently predicted outcomes after TAVR. The aim of this study is to assess changes in GLS after TAVR according to race and factors predicting these changes., Methods: Electronic medical records of patients undergoing TAVR at the University of Illinois, Chicago and Jesse Brown Veteran's Administration Medical Center (Chicago, Illinois) from January 2017-February 2020 were reviewed retrospectively. The most recent transthoracic echocardiogram (TTE) prior to TAVR and the TTE 1-month post-procedure were used to determine GLS. Patients were included if both a pre- and post-procedure study were present and TTE images were of sufficient quality to process strain imaging., Results: A total of 103 patients (average age 76 ± 12 years, 80% male, 42% white) were included. At 1-month post-TAVR, GLS improved for all races: white (-2.7 ± 3.5%, P<0.001), African-American (-2.8 ± 3.3%, P<0.001), and Hispanic (-2.0 ± 2.1%, P<0.001). There were no differences in the degree of improvement among races (P=0.62). Baseline GLS was negatively correlated with changes in GLS overall (r=-0.44, P<0.001). Baseline aortic valve area (cm 2 ) was positively correlated with changes in GLS (r=0.2, P=0.036)., Conclusions: This study demonstrated that GLS improved after TAVR independent of race with similar degrees of change across races. Baseline GLS and aortic valve area predicted strain improvement after TAVR, which suggests that those with more impaired LV function may benefit most from the procedure., Competing Interests: None., (AJCD Copyright © 2021.)

Coronary artery anomalies are relatively rare (approximately 1% on CTA). We present two exceedingly rare cases, as well as the first reported case of anomalous retro-aortic coronary arteries diagnosed with cardiac CTA and angiography.