BACKGROUND Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy and a proposed substrate for arrhythmias and heart failure. In animal models, profibrotic genetic pathways are activated early, before hypertrophic remodeling. Data showing early profibrotic responses to sarcomere-gene mutations in patients with hypertrophic cardiomyopathy are lacking. METHODS We used echocardiography...

Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM) diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, car...

imaging, and its surgical management. Am J Cardiol 2013;111:1677–1679. 19. Harrigan CJ, Peters DC, Gibson CM, Maron BJ, Manning WJ, Maron MS, Appelbaum E. Hypertrophic cardiomyopathy: quantification of late gadolinium enhancement with contrast-enhanced cardiovascular MR imaging. Radiology 2011;258:128–133. 20. Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 yea...

prevalence of apical hypertrophic cardiomyopathy in Japan and the United States. Am J Cardiol 2003;92:1183–6. 21. Olivotto I, Cecchi F, Casey SA, Dolara A, Traverse JH, Maron BJ et al. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 2001;104:2517–24. 22. Kubo T, Kitaoka H, Okawa M, Hirota T, Hayato K, Yamasaki N et al. Clinical impact of atrial f...

An 8-year-old girl died suddenly without prior symptoms. Post-mortem examination identified both systemic group A streptococcal infection and hypertrophic cardiomyopathy. She had no history of cardiac symptoms and was not in a high-risk group for sudden death due to hypertrophic cardiomyopathy. We believe the disseminated but asymptomatic group A streptococcal infection precipitated her early d...

OBJECTIVE To investigate changes in the alignment of the sarcomeric filaments in hypertrophic cardiomyopathy and the effects of cardiac beta myosin heavy chain (beta-MHC) mutation on the sarcomeric ultrastructure. DESIGN A retrospective analysis. PATIENTS Endomyocardial biopsy samples were examined by transmission electron microscopy in seven patients with hypertrophic cardiomyopathy and be...

OBJECTIVES This study was undertaken to differentiate hypertrophic cardiomyopathy from hypertensive hypertrophy using a newly developed M-mode format integrated backscatter imaging system capable of calibrating myocardial integrated backscatter with the power of Doppler signals from the blood. BACKGROUND Myocardial integrated ultrasound backscatter changes in patients with hypertrophic cardio...

Hypertrophic cardiomyopathy occurs as an autosomal dominant familial disorder or as a sporadic disease without familial involvement. While missense mutations in the beta cardiac myosin heavy chain (MHC) gene account for approximately half of all cases of familial hypertrophic cardiomyopathy, the molecular causes of sporadic hypertrophic cardiomyopathy are unknown. To determine whether beta card...

Apical hypertrophic cardiomyopathy is an uncommon variant of non-obstructive hypertrophic cardiomyopathy with low prevalence outside East Asia. A case is reported of a non-Asian (European) 51 year old man with characteristic ECG and morphological changes of apical hypertrophic cardiomyopathy. Although the patient underwent catheterisation three years previously because of suggested coronary isc...

Transmural myocardial infarction in the absence of epicardial coronary arterial narrowing is fairly common at necropsy in patients with hypertrophic cardiomyopathy. Although difficult to diagnose clinically in patients with hypertrophic cardiomyopathy, acute myocardial infarction occasionally has a typical presentation, as in the patient presented in this report. More often, however, transmural...