Case Report of a 33-year-old with Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy

Abstract

Ebstein malformation of tricuspid valve is a congenital disease of tricuspid valve with associated right ventricular cardiomyopathy. Hypertrophic cardiomyopathy is a form of inherited left ventricular cardiomyopathy caused by sarcomeric protein gene mutations with inherent risks of sudden cardiac death. Here we report a rare case with co-occurrence of Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy in a young patient.

A clinical case of a combination of Ebstein’s anomaly and genetically determined cardiomyopathy in a child who required a heart transplantation in childhood

“…Большинство случаев АЭ являются спорадическими, семейные формы встречаются достаточно редко [2]. Также в литературе описаны случаи сочетания АЭ с генетически детерминированными кардиомиопатиями [2,3,7,8], в основном с некомпактным миокардом левого желудочка (ЛЖ), что, по данным Pignatelli и соавторов, встречается в 16,4 % [2, 3,8].…”

Surgical Outcomes of Repairing Ebstein Anomaly: A Single-Center Experience

“…This anomaly is characterized by variable levels of tricuspid valve (TV) septal and posterior leaflet displacement due to the abnormal delamination of the underlying right ventricular (RV) myocardium, resulting in severe tricuspid regurgitation (TR) and abnormal atrialized portion of RV, culminating in right-sided heart failure. Variable degrees of leaflet displacement are accountable for numerous anatomic subtypes of EA (2,3), and clinical presentation ranges from intrauterine or shortly after birth mortality to minimal symptoms in early adulthood. Due to the wide spectrum of clinical and anatomic presentations, finding a uniform management strategy for EA is often challenging (2,3).…”

“…Variable degrees of leaflet displacement are accountable for numerous anatomic subtypes of EA (2,3), and clinical presentation ranges from intrauterine or shortly after birth mortality to minimal symptoms in early adulthood. Due to the wide spectrum of clinical and anatomic presentations, finding a uniform management strategy for EA is often challenging (2,3).…”