Abstract

Absent pulmonary valve syndrome is a rare anomaly and its combination with a complete atrioventricular septal defect and double-outlet right ventricle is even rarer which has never been reported in the literature as an antenatal diagnosis. We report a case of a 21-week fetus diagnosed having this rare combination of lesions. The main purpose of reporting this case is to highlight the prognostic implications for the fetus with this complex heart defect. There is a high risk of fetal heart failure and a higher risk of associated extracardiac and chromosomal anomalies. Unfortunately, the pregnancy was terminated, and hence, further antenatal course and postnatal details could not be evaluated.

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