Ebstein’s Anomaly, Left Ventricular Noncompaction and Gerbode-Like Defect Triad (Fetal Diagnosis and Neonatal Course)

Ebstein’s anomaly is characterized by the apical displacement of the septal and posterior leaflets of the tricuspid valve with atrialization of the right ventricle (RV). It is commonly associated with other heart defects including left ventricular noncompaction. We describe a case of prenatally diagnosed Ebstein’s anomaly in association with left ventricular noncompaction and a septal defect between the left ventricle and the atrialized portion of the RV (Gerbode-like defect). The patient underwent a modified Blalock−Taussig shunt followed by Glenn procedure because of severe RV hypoplasia and RV outflow tract obstruction. The patient tolerated both procedures and is doing clinically well in anticipation of Fontan procedure for single ventricle palliation.

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

Case Reports in Cardiology ◽

10.1155/2015/854236 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Michael McGee ◽

Luke Warner ◽

Nicholas Collins

Keyword(s):

Sudden Cardiac Death ◽

Cardiac Death ◽

Septal Defect ◽

Left Ventricular ◽

Ebstein’S Anomaly ◽

Left Ventricular Noncompaction ◽

Ventricular Noncompaction ◽

Ebstein's Anomaly ◽

Apical Displacement

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. Echocardiography is often used to diagnose LVNC; however, magnetic resonance (MR) imaging offers superior characterization of the myocardium. We report a case of sudden cardiac death in a patient with Ebstein’s anomaly with unrecognized LVNC noted on post mortem examination with screening documenting the presence of LVNC in one of the patient’s twin sons.

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A rare case of Ebstein’s anomaly with left ventricular noncompaction

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492318791421 ◽

2018 ◽

Vol 27 (3) ◽

pp. 208-209

Author(s):

Christos Tourmousoglou ◽

Harilaos Bogossian ◽

Vlasis Ninios ◽

Elias Ninios

Keyword(s):

Rare Case ◽

Systolic Function ◽

Left Ventricular ◽

Ebstein’S Anomaly ◽

Left Ventricular Noncompaction ◽

Ventricular Noncompaction ◽

Ebstein's Anomaly ◽

Congenital Cardiac Disease ◽

Left Ventricular Noncompaction Cardiomyopathy ◽

Apical Displacement

Ebstein’s anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein’s anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function.

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