LEFT VENTRICULAR NONCOMPACTION WITH EBSTEIN ANOMALY SECONDARY TO MYH7 MUTATION

Mutations in the sarcomere gene MYH7 in Ebstein anomaly.

BACKGROUND Ebstein anomaly is a rare congenital heart malformation characterized by adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium. An association between Ebstein anomaly with left ventricular noncompaction (LVNC) and mutations in MYH7 encoding β-myosin heavy chain has been shown; in this report, we have screened for MYH7 mutations in a cohort...

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

Novel mutation in exon 14 of the sarcomere gene MYH7 in familial left ventricular noncompaction with bicuspid aortic valve.

A 49-year-old man with bicuspid aortic valve (BAV) and mild-to-moderate aortic valvular stenosis was referred for investigation of a dilated cardiomyopathy. At the time of presentation, he was physically active, New York Heart Association class I. Physical examination revealed a grade III/ VI harsh crescendo-decrescendo murmur at right upper sternal border radiating to both carotids. His 12-lea...

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

Ebstein's anomaly is a congenital disorder characterized by apical displacement of the septal leaflet of the tricuspid valve. Ebstein's anomaly may be seen in association with other cardiac conditions, including patent foramen ovale, atrial septal defect, and left ventricular noncompaction (LVNC). LVNC is characterized by increased trabeculation within the left ventricular apex. Echocardiograph...

Left Ventricular Noncompaction

Ma Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinica...