American Heart Association

cardiomyopathy

Female HCM patients with obstructive physiology undergo myectomy later and have worse diastolic dysfunction but more compliant titin

Contributor: Nick Hawkes

Hypertrophic Cardiomyopathy (HCM) is a genetic condition characterized by unexplained left ventricular hypertrophy often with a wall thickness of greater than or equal to 15mm. it often disproportionately affects the septum and can lead to development of left ventricular outflow obstruction (LVOTO). Myectomy is a targeted surgical reduction in LV mass — thereby reducing LVOTO. Nijenkamp et al analyzed samples obtained during myectomy as well as pre-myectomy echocardiographic markers of diastolic dysfunction in 71 HCM patients. The authors also compared control myocardium to HCM. In this study, female HCM patients presented later, had a higher degree of diastolic dysfunction, a higher amount of fibrosis, more compliant titin, and greater septal thickness when indexed to body surface area (IVSi) compared to males.

Watch out for heart failure in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia

Contributor: Chris Sobowale

Natural history is catching up when arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients develop heart failure.

ARVC/D is an inherited cardiomyopathy in which myocardium is replaced with fibro-fatty tissue.  This is followed by development of right ventricular dysfunction and ventricular arrhythmia.

Why are heart failure specialists seeing more patients with ARVC/D? The patients are living longer. Increased awareness allows timely diagnosis. ICD implantation prevents sudden cardiac death. As a result, ARVC/D patients eventually develop heart failure. Gilotra et al explores the increased prevalence of HF related to ARVC/D and characterizes the evolution to HF.

Concentric left ventricular hypertrophy rarely leads to dilated cardiomyopathy

Contributor: Jennifer Huang

Does concentric left ventricular hypertrophy (LVH) progress to dilated cardiomyopathy (DCM)? It may not be as common as we previously thought and the transition may occur over decades.

1,386 participants of the Dallas Heart Study without baseline LV dilation were included. Ten percent of the participants had baseline LVH (7.2 g/mL0.67  for men and 5.8 g/mL0.67 for women). The study population had a mean age of 44 years, 57% women and 43% black patients. Of note, patients that developed cardiovascular disease during the study period (MI, CABG, PCI, stroke or HF) were not included in the final cohort of 1282. Baseline and follow up cardiac magnetic resonance imaging was performed a median of 7 years after baseline imaging.