Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy; and among them, 25% present dynamic left ventricular outflow tract obstruction. Apical HCM is unusual. Echocardiography has been the first imaging method for patients with suspected HCM, but its shortcomings in evaluating the apex are well known. We present a 56-year-old woman with a history of left hemitho...

Background Late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) is a common finding in patients with hypertrophic cardiomyopathy (HCM), and its extent correlates with clinical parameters such as ventricular tachycardia and sudden cardiac death. While LGE has been shown to reflect focal replacement fibrosis, recently optimized T1 mapping techniques have allowed for the quantific...

OBJECTIVES The purpose of this study was to determine the prevalence, clinical significance, and genetic basis of hypertrophic cardiomyopathy (HCM) with "restrictive phenotype" characterized by restrictive filling and minimal or no left ventricular hypertrophy. BACKGROUND Hypertrophic cardiomyopathy is a heterogeneous myocardial disorder with a broad spectrum of clinical presentation and morp...

Mutations in myosin heavy chain (MyHC) can cause hypertrophic cardiomyopathy (HCM) that is characterized by hypertrophy, histopathology, contractile dysfunction, and sudden death. The signaling pathways involved in the pathology of HCM have not been elucidated, and an unresolved question is whether blocking hypertrophic growth in HCM may be maladaptive or beneficial. To address these questions,...

Obstructive hypertrophic cardiomyopathy (HCM) during pregnancy is associated with significant maternal and perinatal morbidity. Physiological changes can induce complications: sudden death, heart failure arrhythmia involving the fetal prognosis. Women HCM generally tolerate well. The risk however higher in women who are symptomatic before or those severe left ventricular outflow tract obstructi...

BACKGROUND Restrictive cardiomyopathy (RCM) has been associated with poor prognosis in childhood. The goal of the present analysis was to use the Pediatric Cardiomyopathy Registry to analyze outcomes of childhood RCM, with a focus on the impact of phenotype comparing pure RCM with cases that have additional features of hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS We analyzed the Ped...

Background Patients with hypertrophic cardiomyopathy (HCM) represent a heterogeneous group at risk of death and other cardiac events, necessitating further effective risk stratification. Late gadolinium enhancement (LGE) on cardiac MR (CMR) represents fibrosis and has emerged as a possible risk predictor of hard events in several small studies. We sought to evaluate the prognostic utility of LG...

Background Surgical myectomy is regarded as a gold standard treatment for Hypertrophic Cardiomyopathy (HCM) and may be conducted using different approaches, depending on the location of the thickened heart muscle, to widen the outflow tract and thereby reduce outflow drag to favorably alter the natural course of HCM. We present a novel approach for patient-specific visualization of precise loca...

Background In hypertrophic cardiomyopathy (HCM) assessment of late gadolinium enhancement (LGE) is increasingly important as LGE has been demonstrated to be an adverse prognosticator. Manual delineation of LGE after visual assessment is labour-intensive. Automatic quantification is limited by its dependence on signal/noise ratio and varying definition of LGE. Our aim is to assess inter-individu...

Background Interest has emerged in the evaluation of mitral valve morphology in patients with Hypertrophic Cardiomyopathy (HCM) and its contribution to left ventricular outflow tract obstruction (LVOTO). Specifically, elongation of the anterior mitral valve leaflet (AMVL) appears to be part of phenotypic expression in HCM and has been associated with severity of LVOT obstruction. However, the i...