Ebstein's Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

Ebstein’s anomaly may be caused by mutations in the sarcomere protein gene MYH7

Ebstein's anomaly is a rare congenital heart malformation characterised by adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium. Associated abnormalities of left ventricular morphology and function including left ventricular noncompaction (LVNC) have been observed. An association between Ebstein's anomaly with LVNC and mutations in the sarcomeric pr...

Ebstein's anomaly with left ventricular noncompaction and bicuspid aortic valve.

Response to letter regarding article, "mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center".

BACKGROUND Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death. METHODS AND RESULTS We retrospectively reviewed all children diagnosed with left ventric...

Ebstein's anomaly.

Ebstein's anomaly is a rare congenital heart disease, accounting for less than 1% of all congenital heart diseases, characterized by a wide clinical, electrocardiographic, echocardiographic, anatomic and prognostic polymorphism. The disease can be fatal since birth or may remain asymptomatic until adulthood, sometimes being associated with septal defects, transposition of great vessels, preexci...

Cardiac 123I-MIBG scintigraphy and arrhythmic risk in left ventricular noncompaction.

Left ventricular noncompaction is an unusual but increasingly recognized cardiomyopathy, the etiology of which is still not definitely established. Clinical presentation includes a wide spectrum of scenarios, including heart failure, thromboembolism and malignant arrhythmias, with half of deaths occurring suddenly. Early detection of LVNC is therefore essential to prevent sudden cardiac death. ...