Mavacamten in real-life practice: Initial experience at a hypertrophic cardiomyopathy centre.

Aims: In clinical trials, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved symptoms in patients with symptomatic obstructive hypertrophic cardiomyopathy (oHCM). We aimed to share our real-world experience with the efficacy and safety of mavacamten in this patient population.

Methods And Results: This retrospective, single-centre study included patients with symptomatic oHCM from March 2023 to November 2023.

Clinical Interpretation of Genetic Variants in the Evaluation and Management of Thoracic Aortic Aneurysm and Dissection.

Background: We aimed to elucidate clinical implications of genetic variant interpretation in assessing disease severity and progression in thoracic aortic aneurysm and dissection (TAAD) patients.

Methods: Consecutive TAAD patients with aortic root and/or ascending aortic aneurysms seen between 2011 and 2020 were included. Serial echocardiography, family history of TAAD, and management information were retrospectively collected and analyzed.

Hypertrophic cardiomyopathy in identical twins: a case series.

Background: Hypertrophic cardiomyopathy in identical twins is rare. Cases of hypertrophic cardiomyopathy with homogenous and heterogeneous phenotypes have been described in the literature.

Case Summary: We report a pair of monozygotic twins (Twin A and Twin B) with identical morphological expression of hypertrophic cardiomyopathy.

Implantable cardioverter defibrillators in diabetics: efficacy and safety in patients at risk of sudden cardiac death.

Diabetes mellitus is a major risk factor for arrhythmogenesis and is associated with a two-fold increase in all-cause mortality and a four-fold increase in cardiovascular mortality including sudden cardiac death when compared with nondiabetics. Implantable cardioverter defibrillators (ICD) have been shown to effectively reduce arrhythmic death and all-cause mortality in patients with severe myocardial dysfunction. With a high competing risk of nonarrhythmic cardiac and noncardiac death, survival benefit of ICD in patients with diabetes mellitus could be reduced, but the subanalysis of diabetic patients in randomized clinical trials provides reassurance regarding a similar beneficial survival effect of ICD and cardiac resynchronization therapy in diabetics, as observed in the overall population with advanced heart disease.

Cardiac resynchronization therapy in patients with challenging anatomy due to venous anomalies or adult congenital heart disease.

Background: Cardiac resynchronization therapy (CRT) has proven salutary effects in patients with congestive heart failure, systolic dysfunction, and electromechanical dyssynchrony in the setting of ischemic, nonischemic, and congenital cardiomyopathy. While CRT device implants have become routine in the adult ischemic or nonischemic cardiomyopathy populations, patients with congenital heart disease offer special challenges due to unusual anatomic variations.

Methods: A comprehensive assessment of anatomic abnormalities is essential prior to implant.

Isolated partial anomalous pulmonary venous connection: Diagnostic value of suprasternal color flow imaging and contrast echocardiography.

Isolated partial anomalous pulmonary venous connection is frequently missed even when patients present with mild right ventricular enlargement. We describe the value of imaging from suprasternal window with color flow and ultrasound contrast echocardiography in aiding the diagnosis.