Ebstein’s Anomaly, Possible Newly Implicated Drug Aetiology? A Case Report

Barakat A. Animasahun*, Omotola A. Ajayi, Faith O. Lawani and Elizabeth A. Disu

Ebstein’s Anomaly, Possible Newly Implicated Drug Aetiology? A Case Report

A striking aspect of Ebstein’s anomaly is its varying clinical presentation based on the age at which it is detected. Its mode of presentation ranges from hydrops fetalis in utero and cyanosis in the newborn, a murmur or heart failure in an infant, an arrhythmia in an adolescent to incidental detection of an ASD in the elderly, all based on the degree of affectation of the tricuspid valve
and other associated cardiac defects.

In this case report, we describe Ebstein’s anomaly in a Nigerian neonate, highlighting its spectrum of presentation across all age groups, highlighting the presentation, known etiology and its prognosis. Permission was granted by the mother to publish this case report.

Appearance, pulse, grimace, activity and respiration (APGAR) scores were 4 and 8 at the first and fifth-minutes respectively. Her weight was 2,670 g. She however remained centrally cyanosed  despite regular respiratory excursions and administration of supplemental oxygen. Oxygen saturation was 66% in room air and 76% on intranasal oxygen.

Ebstein’s anomaly was first described by Wilhelm Ebstein in 1866. Its characterized by malformation and downward displacement of the insertion of the septal and posterior leaflets of the tricuspid valve into the right ventricle. This valve displacement results in an enlarged right atrium due to the “atrialized” portion of the right ventricle and accompanying tricuspid regurgitation. The right ventricle is usually small and dysfunctional. The left ventricular size is usually normal, but it may be compressed by a dilated right ventricle. The left ventricle is also dysfunctional and maybe fibrotic.

Heart Res Open J. 2021; 8(1): 18-20. doi: 10.17140/HROJ-8-158