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To determine the differences in left atrial (LA) function and geometry assessed by cardiac magnetic resonance (CMR) between transthyretin (ATTR) and immunoglobulin light chain (AL) cardiac amyloidosis (CA). We performed a retrospective analysis of 54 consecutive patients (68.5% male, mean age 67 ± 11 years) with confirmed CA (24 ATTR, 30 AL) who underwent comprehensive CMR examinations. LA structural and functional assessment including LA volume, LA sphericity index, and LA strain parameters were compared between both subtypes. In addition, 15 age-matched controls were compared to all groups. Patients with ATTR-CA were older (73 ± 9 vs. 62 ± 10 years, p < 0.001) and more likely to be male (83.3% vs. 56.7%, p = 0.036) when compared to AL-CA. No significant difference existed in LA maximum volume and LA sphericity index between ATTR-CA and AL-CA. LA minimum volumes were larger in ATTR-CA when compared with AL-CA. There was a significant difference in LA function with worse strain values in ATTR vs AL: left atrial reservoir [7.4 (6.3–12.8) in ATTR vs. 13.8 (6.90–24.8) in AL, p = 0.017] and booster strains [3.6 (2.6–5.5) in ATTR vs. 5.2 (3.6–12.1) in AL, p = 0.039]. After adjusting for age, LA reservoir remained significantly lower in ATTR-CA compared to AL-CA (p = 0.03), but not LA booster (p = 0.16). We demonstrate novel differences in LA function between ATTR-CA and AL-CA despite similar LA geometry. Our findings of more impaired LA function in ATTR may offer insight into higher AF burden in these patients.

Speckle tracking echocardiography (STE), and more recently, cardiovascular magnetic resonance myocardial feature tracking (CMR-FT) provides insight into all phases of atrial function. The aim of our study was to compare all phases of RA strain using CMR-FT and STE and also assess the relationship between RA and LA strain. A total of 61 healthy volunteers with mean age of 45 ± 13 years had adequate tracking for analysis on CMR-FT and 2D-STE. Females had larger RA reservoir strain (39 ± 15% vs. 32 ± 13%, p = 0.046) and conduit strain (26 ± 12% vs. 20 ± 9%, p = 0.03) when compared to males, but was not the case with booster strain (14 ± 7% vs. 12 ± 6%, p = 0.45). In comparison with STE derived strain, the RA reservoir and conduit strain were not significantly different between CMR-FT and the three echocardiography gating methods (p > 0.05 for all). Noticeably, there were no significant differences in strain and strain rate between RA and LA function using CMR-FT (p > 0.05 for all). RA strain and strain rate using CMR-FT had fair and good intra- and inter-observer reproducibility and had superior reproducibility compared to STE derived strain.

Phenotypic expression of lamin AC-dilated cardiomyopathy (LMNA-DCM) and cardiac sarcoidosis (CS) at times presents with similar clinical findings, including arrhythmia, conduction abnormalities, and heart failure. Lamin AC-dilated cardiomyopathy is one of the most common causes of genetic DCM with an autosomal dominant inheritance pattern. Contrastingly, a smaller proportion of sarcoid cases present with isolated cardiac involvement, highlighting the intricate nature of this systemic disease. This paper presents three cases highlighting potential pitfalls in the clinical diagnosis and management of laminopathies. Also highlighted is the need for extreme caution when endomyocardial biopsies are equivocal and there is sole reliance on cardiac imaging to establish diagnostic criteria. In addition, this case series highlights the imperative role of genetic testing during the initial workup for establishing the accurate etiology in the setting of cardiomyopathy. The strikingly similar clinical characteristics of LMNA-DCM and CS underscore the necessity for a robust diagnostic approach, including early integration of genetic testing, as this approach holds great promise for improving outcomes and the quality of life for those grappling with these challenging cardiac conditions. Innovative strategies, including comprehensive advanced imaging protocols and precision medicine, promise to advance our comprehension and management of both disease entities.

Global myocardial work (GMW) is an emerging method to characterize left ventricle (LV) function with potential advantages over both ejection fraction and global longitudinal strain (GLS). We aimed to determine the feasibility and reproducibility for echocardiographic-derived GMW in a healthy pediatric population; establish normal reference values; and investigate the influence of age, gender, and other clinical factor on normal reference ranges. We prospectively enrolled 212 individuals (median age of 9 years; interquartile range, 6 to 12 years, 112 female). Global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency (GWE) were measured from LV pressure–strain loops. Quantification of GMW was performed using a GE Vivid E95 system and available software package (Echopac V.203, GE). The mean LV EF was 64 ± 3% with GLS of -21.3 ± 1.5%. GWI was 1688 ± 219 mmHg% with mean GWE of 96.5 ± 1.4%. The GCW was 1959 ± 207 mmHg%, and the mean GWW of 61.1 ± 30.9 mmHg%. No significant difference was found in MW indices across age group and gender ( p  > 0.05 for all). There were significant correlations between both GWI and GCW with GLS and systolic blood pressure ( p  < 0.001), but not with GWE and GWW. Linear regression model revealed that GWI and GCW were more closely correlated with systolic blood pressure than GLS. LV MW indices had good intra-observer and inter-observer reproducibility. This study establishes both the feasibility and reference ranges for non-invasive echocardiographic indices of GMW in healthy children. Myocardial work appears to be a complementary modality to assess LV performance in children.

Background The survival outcome for primary cardiac malignant tumors (PMCTs) based on race has yet to be fully elucidated in previously published literature. This study aimed to address the general long‐term outcome and survival rate differences in PMCTs among African Americans and Caucasian populations. Methods The 18 cancer registries database from the Surveillance, Epidemiology, and End Results (SEER) Program from 1975 to 2016 were utilized. Ninety‐four African American (AA) and 647 Caucasian (CAU) patients from the SEER registry were available for survival analysis. The log‐rank test was used to compare the difference in mortality between two populations and presented by the Kaplan–Meier curves. A multivariate Cox proportional hazards regression was used to determine the independent predictors of all‐cause mortality. Results The overall 30‐day, 1‐year, and 5‐year survival rates were 74%, 44.3%, and 16.6%, respectively, with a median survival of 10 months. There was no significant difference in survival rate between the two races (p‐value = 0.55). The 1‐year survival rate improved significantly during the study timeline in the AA population (13.3% during 1975–1998, 40.9% during 1999–2004, 50% during 2005–2010, and 59.7% during 2011–2016, p‐value = 0.0064). Age of diagnosis, type of tumor, disease stage, and chemotherapy administration are the main factors that predict survival outcomes of PMCT patients. Interactive nomogram was developed based on significant predictors. Conclusions PMCTs have remained one of the most lethal diseases with poor survival outcome. Survival rate improved during the timeline in AA patients, but in general, racial differences in survival outcome were not observed. Survival rate improved during the timeline in AA patients, but in general, racial differences in survival outcome were not observed. Interactive nomogram was developed based on significant predictors: age of diagnosis, type of tumor, disease stage, and chemotherapy administration.

Aim The objective of this study was to investigate the prognostic importance of right ventricular dysfunction (RVD) and tricuspid regurgitation (TR) in patients with moderate–severe functional mitral regurgitation (FMR) receiving MitraClip procedure. RVD and TR grade are associated with cardiovascular mortality in the general population and other cardiovascular diseases. However, there are limited data from observational studies on the prognostic significance of RVD and TR in FMR receiving MitraClip procedure. Methods and results A systemic review and meta‐analysis were performed using MEDLINE, Scopus, and Embase to assess the prognostic value of RVD and TR grade for mortality in patients with functional mitral regurgitation (FMR) receiving MitraClip procedure. Hazard ratios were extracted from multivariate models reporting on the association of RVD and TR with mortality and described as pooled estimates with 95% confidence intervals. A total of eight non‐randomized studies met the inclusion criteria with seven studies having at least 12 months follow‐up with a mean follow‐up of 20.9 months. Among the aforementioned studies, a total of 1112 patients (71.5% being male) were eligible for being included in our meta‐analysis with an overall mortality rate of 28.4% (n = 316). Of the enrolled patients, RVD was present in 46.1% and moderate–severe TR in 29.2%. RVD was significantly associated with mortality compared to normal RV function (HR, 1.79, 95% CI, 1.39–2.31, P < 0.001, I2 = 0). Patients with moderate–severe TR showed increased risk of mortality compared with those in the none‐mild TR group (HR, 1.61. 95% CI, 1.11–2.33, P = 0.01, I2 = 14). Conclusions This meta‐analysis demonstrates the prognostic importance of RVD and TR grade in predicting all‐cause mortality in patients with significant FMR. RV function and TR parameters may therefore be useful in the risk stratification of patients with significant FMR undergoing MitraClip procedure.

There is a growing body of literature supporting the utilization of machine learning (ML) to improve diagnosis and prognosis tools of cardiovascular disease. The current study was to investigate the impact that the ML framework may have on the sensitivity of predicting the presence or absence of congenital heart disease (CHD) using fetal echocardiography. A comprehensive fetal echocardiogram including 2D cardiac chamber quantification, valvar assessments, assessment of great vessel morphology, and Doppler-derived blood flow interrogation was recorded. The postnatal echocardiogram was used to ascertain the diagnosis of CHD. A random forest (RF) algorithm with a nested tenfold cross-validation was used to train models for assessing the presence of CHD. The study population was derived from a database of 3910 singleton fetuses with maternal age of 28.8 ± 5.2 years and gestational age at the time of fetal echocardiography of 22.0 weeks (IQR 21–24). The proportion of CHD was 14.1% for the studied cohort confirmed by post-natal echocardiograms. Our proposed RF-based framework provided a sensitivity of 0.85, a specificity of 0.88, a positive predictive value of 0.55 and a negative predictive value of 0.97 to detect the CHD with the mean of mean ROC curves of 0.94 and the mean of mean PR curves of 0.84. Additionally, six first features, including cardiac axis, peak velocity of blood flow across the pulmonic valve, cardiothoracic ratio, pulmonary valvar annulus diameter, right ventricular end-diastolic diameter, and aortic valvar annulus diameter, are essential features that play crucial roles in adding more predictive values to the model in detecting patients with CHD. ML using RF can provide increased sensitivity in prenatal CHD screening with very good performance. The incorporation of ML algorithms into fetal echocardiography may further standardize the assessment for CHD.

We sought to study the mechanics of ventricular contraction in patients with and outward basal movement. Using echocardiographic parasternal long-axis views we retrospectively screened 760 echocardiograms to enroll 50 individuals with late systolic bileaflet prolapse, papillary muscle traction, and outward movement of the sub-annular base and posterior mitral annulus in late systole. Two-dimensional speckle tracking echocardiography was used to analyze the mechanics of contraction. Global and regional longitudinal strain values between the study group and 45 healthy control subjects were compared. The study group’s global strain was lower compared to the control group. We identified a pattern of weak contraction of the inferior and lateral walls in the late systolic bileaflet prolapse group. The weakest segment in the study group was the basal-inferolateral segment (− 15.8% vs. − 21.5%, p < 0.001). There was no relationship between the mitral annular size, degree of leaflet prolapse, or degree of basal weakness. Late systolic bileaflet mitral prolapse and papillary muscle traction are accompanied by an outward movement of the sub-annular base and posterior mitral annulus in late systole. We demonstrated an abnormal contractile pattern in these ventricles characterized by a weaker contraction of the base, most significant at the inferolateral segment. This weakness of contraction may contribute to the outward movement of the base and posterior annulus.

Mitral valve prolapse (MVP) is the most common valvar dysfunction in children. There is emerging evidence that MVP is not always a benign entity, hence identification of underlying mechanisms is pertinent to clinical management. Our group previously identified a ventricular contraction abnormality named end-systolic basal eversion (ESBE) in adults that contributed to MVP. The aim of this study was to evaluate regional circumferential strain in pediatric patients with MVP and ESBE compared to normal controls. Left ventricular circumferential strain was assessed in 16 pediatric patients referred for clinical echocardiographic examination with MVP and ESBE (MVP group) and compared to age-gender-matched healthy subjects. ESBE has been previously described as late systolic bileaflet mitral valve prolapse, papillary traction, and concomitant late systolic outward movement of the basal inferior myocardium. The mean age of the MVP group was 13.8 ± 4.6 year and 75% were female. All patients with MVP and controls had qualitatively normal systolic cardiac function. The MVP group had significantly lower regional strain values for 11/16 of the segments including all 6 basal segments. Importantly, the basal inferior (− 17.02 ± 8.32% vs. − 26.10 ± 3.18, p = 0.001) and basal inferolateral (− 19.53 ± 9.76 vs. − 26.10 ± 3.18, p = 0.03) had the lowest strain values compared to the average of all other segments suggesting weaker contraction in the basal inferior segments. Pediatric patients with MVP and ESBE are subject to a similar left ventricular mechanical dysfunction previously described in adults. ESBE was evident by decreased basal circumferential strain values. These findings denoted weaker contraction which is believed to propagate the late systolic outward movement of the basal ventricular myocardium.