Key result
Staged surgical palliation successfully treats complex triad of Ebstein anomaly, LVNC, and Gerbode defect.
Why the study?
Ebstein's anomaly is commonly associated with other heart defects including left ventricular noncompaction and rare septal defects such as Gerbode-like defects, but detailed clinical courses are not well described.
Case Report (n=1)
Describes a rare case of a patient with Ebstein's anomaly, left ventricular noncompaction, and a Gerbode-like defect successfully managed with single ventricle palliation.
Answers are grounded in this paper and cite their sources.
Hypothesis-generating for single-ventricle palliation in this rare triad; prospective data needed before broader adoption.
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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Mehdi et al. (2021) conducted a case report in Ebstein's anomaly, left ventricular noncompaction, and Gerbode-like defect (n=1). Modified Blalock-Taussig shunt followed by Glenn procedure was evaluated. A patient with a triad of Ebstein's anomaly, left ventricular noncompaction, and a Gerbode-like defect successfully underwent a modified Blalock-Taussig shunt and subsequent Glenn procedure.
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