Introduction Long QT syndrome (LQTS) is an arrhythmogenic disorder that presents with QT interval prolongation and high risk for onset of ventricular arrhythmias and sudden death. It is a genetically heterogeneous condition involving at least 17 different genetic loci. ,…
Introduction Andersen-Tawil syndrome (ATS) is a clinically pleiotropic disorder resulting from mutations in an ion channel gene that modulates the most terminal portion of cardiac repolarization. ATS is fundamentally a disorder of ventricular repolarization. Although initially classified as long QT…
Long QT Syndrome Congenital long QT syndrome (LQTS), a leading cause of sudden death in the young, is characterized by a prolongation of the QT interval in the standard electrocardiogram (ECG) and by syncopal episodes, which often result in cardiac…
In 1992, a manuscript titled “Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographical syndrome” was published in the Journal of the American College of Cardiology. This publication described eight individuals with…
Introduction Malignant mitral valve prolapse (MVP) syndrome has been recently described in a subset of patients who may be at greater risk for sudden cardiac death (SCD), made up of young women with mostly bileaflet MVP, midsystolic click, biphasic or…
Takotsubo cardiomyopathy (TC), also known as apical ballooning syndrome or stress cardiomyopathy, is a reversible cause of left ventricular (LV) dysfunction that is frequently precipitated by an emotionally or physically stressful event. Dote and colleagues first reported the occurrence of…
Acknowledgments J.A.T. was funded, in part, by the National Institutes of Health (NIH), National Heart Lung, and Blood Institute (NHLBI) with grants RO1 HL53392 and RO1 HL087000, for the Pediatric Cardiomyopathy Registry and Pediatric Cardiomyopathy Specimen Repository, RO1 HL151438, and…
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome that is characterized by exercise or emotional stress–induced bidirectional or polymorphic ventricular tachyarrhythmias in patients with a normal electrocardiogram (ECG) at rest and in the absence of structural heart disease.…
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy that is characterized by ventricular arrhythmias, an increased risk for sudden cardiac death (SCD), and abnormalities of right ventricular (RV) structure and function. Although structural involvement of the RV predominates, it…
Hypertrophic cardiomyopathy (HCM) is a common genetic cardiac disease with global distribution and heterogeneous morphologic expression and natural history. , In the current cardiovascular treatment era, HCM is associated with very low disease-related mortality with an expectation for normal longevity…