Faculty Bibliography

BACKGROUND:Cardiovascular-kidney-metabolic syndrome (CKM) describes the clustering of risk factors and progression of disease at the intersection of metabolic disorders, chronic kidney disease (CKD), and cardiovascular disease (CVD). We aimed to characterize the prevalence of CKM stages and progression of CKM syndrome among diverse U.S. Hispanic/Latino adults. METHODS:The Hispanic Community Health Study/Study of Latinos (HCHS/SOL) is a population-based longitudinal cohort of 16,415 Hispanic/Latino adults aged 18-74 years from four U.S. communities. Participants completed examinations at visit 1 (V1, 2008-2011) and visit 2 (V2, 2014-2017). Data collected included body mass index, waist circumference, glucose tolerance, lipids, blood pressure, metabolic syndrome, CKD, 10-year CVD risk (to define subclinical CVD), and clinical CVD. CKM stages were defined using American Heart Association criteria: stage 0 (no CKM risk factors), stage 1 (excess/dysfunctional adiposity), stage 2 (metabolic risk factors or CKD), stage 3 (subclinical CVD or risk equivalents), and stage 4 (clinical CVD). We estimated the age-standardized prevalence of CKM stages at V1 (n=16,123) and CKM stage progression from V1 to V2 (n=11,178). All analyses were weighted and accounted for the HCHS/SOL complex survey design. RESULTS:At V1, the mean age was 41.1 years, and 52.3% were female. The distribution of CKM stages was: stage 0, 10.6% (standard error (SE): 0.4); stage 1, 26.1% (SE: 0.6); stage 2, 50.6% (SE: 0.6); stage 3, 2.4% (SE: 0.2); and stage 4, 10.3% (SE: 0.3). Over an average follow-up of six years, 10.3% (SE: 0.5) regressed to a lower CKM stage, while 21.5% (SE: 0.7) progressed to a higher CKM stage. CONCLUSIONS:Among diverse U.S. Hispanic/Latino adults, eight in ten had CKM risk factors or subclinical CVD, and one in five experienced CKM progression over six years. These findings highlight the need for targeted, early-stage interventions to prevent CKM progression and improve CKM health in Hispanic/Latino populations.

MR imaging is increasingly used in evaluation of patients with known or suspected hypertrophic cardiomyopathy (HCM), as it provides useful information on cardiac structure, function, and tissue characterization that is complementary to echocardiography. While the adverse effect of left ventricle (LV) outflow tract obstruction on blood flow patterns is well characterized by the midsystolic drop in LV ejection velocities and flow, flow patterns in HCM with mid-LV obstruction, with or without apical aneurysm, are less well characterized. MR imaging can provide additional information on alterations of blood flow patterns in these HCM phenotypes and "paradoxic" flows associated with apical aneurysms.

Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) occurring in 20-25% of patients and associated with significant morbidity including stroke risk and worsening heart failure. Atrial fibrillation in HCM (HCM-AF) arises from disease-specific mechanisms distinct from non-HCM AF including HCM-related atrial structural changes (dilation/ fibrosis), biophysical abnormalities (within ion-channels), and atrial dysfunction that culminate in an HCM specific atrial-myopathy. While the adoption of contemporary AF therapies have been associated with improved clinical outcomes in non-HCM-AF populations, HCM-AF outcomes nonetheless remain worse including lower rates of rhythm control success with catheter ablation and antiarrhythmic medications. In this context, we provide support that HCM-AF is a distinct clinical entity, highlighting gaps in knowledge and care, and represents an area of need for dedicated investigation as it is unclear the effectiveness of applying therapeutics from non-HCM AF populations to HCM-AF.

UNLABELLED:Hypertrophic cardiomyopathy (HCM) is the most common monogenic cardiac disease, affecting approximately 0.2% of the general population. Despite its prevalence, it remains significantly underdiagnosed clinically. Surgical management of obstructive HCM has advanced extensively, particularly in understanding the mitral valve's contribution to left ventricular outflow tract obstruction (LVOTO). Historically, LVOTO was attributed mainly to septal hypertrophy and treated through isolated septal myectomy. However, contemporary insights highlight the importance of mitral leaflet elongation and abnormal papillary muscle dynamics in this pathology. Mitral leaflet shortening, either through horizontal plication or direct excision (ReLex), alongside the release of abnormal papillary muscle attachments, has emerged as a complementary strategy to standard septal myectomy. These combined approaches have demonstrated improved surgical outcomes, including reduced mitral regurgitation, alleviation of LVOTO, and excellent mid-term survival. This review synthesizes current evidence and clinical experiences, providing insights into mitral leaflet shortening's role and the comprehensive surgical strategies for managing obstructive HCM. SUPPLEMENTARY INFORMATION/UNASSIGNED:The online version contains supplementary material available at 10.1007/s12055-025-02051-1.

OBJECTIVE/UNASSIGNED:During septal myectomy, once the heart is arrested and drained of blood on cardiopulmonary bypass, transesophageal echocardiography can no longer assess septal thickness. In the present study, we evaluated the effectiveness of on-pump intracardiac echocardiography (OPIE) for real-time intraoperative septal thickness assessment in patients with preoperative thickness ≤2.0 cm. Our hypothesis was that OPIE measurements would be conconcordant with the pre- and postcardiopulmonary bypass transesophageal echocardiography measurements that are at present the primary operative guides. METHODS/UNASSIGNED:We retrospectively reviewed patients with hypertrophic cardiomyopathy and septal thickness ≤2.0 cm on transthoracic echocardiography who underwent septal myectomy from July 2017 to July 2024. The OPIE probe was introduced into the left-ventricular chamber during cardioplegic arrest, with repeated measurements to assess the depth and adequacy of resection. Septal thickness was evaluated pre-myectomy using transthoracic echocardiography, cardiac magnetic resonance imaging, transesophageal echocardiography, and OPIE. Lin's concordance correlation coefficients and Bland-Altman analyses were used to evaluate agreement between modalities. RESULTS/UNASSIGNED:A total of 220 patients were included with preoperative thickness ≤2.0, 56 of whom underwent myectomy with OPIE guidance. Preresection transesophageal echocardiography and OPIE demonstrated the strongest agreement of all the imaging modalities (Lin's concordance correlation coefficient, 0.81; 95% CI, 0.72-0.88), with minimal bias (-0.73) and the narrowest limits of agreement (-3.76, +2.31]. OPIE-derived resection thickness estimates were tightly clustered. In the OPIE cohort, there was 1 ventricular septal defect (1.8%) and no 30-day mortality. CONCLUSIONS/UNASSIGNED:OPIE is a reliable tool for intraoperative assessment of septal thickness, particularly in patients with mild hypertrophy.

BACKGROUND:We present the case of a 74-year-old woman diagnosed with obstructive hypertrophic cardiomyopathy. CASE SUMMARY/METHODS:Amyloidosis was initially considered because she was genotype positive in the transthyretin gene. However, because of 2 negative 99m technetium pyrophosphate radionuclide scans, this diagnosis was considered unlikely, and endomyocardial biopsy was deferred. She had an adverse response to all attempted medical therapies for her left ventricular outflow tract obstruction and ultimately underwent surgical myectomy. Surgical pathology revealed transthyretin (TTR) amyloidosis. DISCUSSION/CONCLUSIONS:This case highlights the limitations of diagnostic testing and reinforces the consideration of more invasive procedures to determine the true underlying cause of disease. This helps clinicians provide the most advanced level of treatment available. TAKE-HOME MESSAGES/CONCLUSIONS:TTR amyloidosis can mimic hypertrophic cardiomyopathy with left ventricular outflow tract obstruction. 99m Technetium pyrophosphate scans are useful to investigate the presence of TTR amyloid, but if suspicion persists despite negative testing, it is reasonable to perform an endomyocardial biopsy.