Unguarded Tricuspid Valve and Pulmonary Atresia With Intact Ventricular Septum Complicated With Right Coronary Artery Fistula and Advanced Atrioventricular Block in a Fetus: A Case Report

TL;DR

A rare fetal heart condition called unguarded tricuspid valve was reported in a case where the baby survived in the womb but died shortly after birth.

Contribution

This case report highlights the rare survival of a fetus with severe unguarded tricuspid valve and associated complications.

Findings

The fetus had multiple severe heart defects but did not develop fetal hydrops.

The baby was born at 33 weeks but died on the second day of life.

The case suggests that some fetuses with severe unguarded tricuspid valves may survive the fetal period.

Abstract

The unguarded tricuspid valve is a rare and severe condition. When found in the fetus, they mostly undergo abortion or intrauterine death. The details of the fetal course in such cases are poorly understood. Here, we report a case of an unguarded tricuspid valve detected at 20 weeks of gestation who developed a complete atrioventricular block and survived in utero. The fetus also had pulmonary atresia with intact ventricular septum, Uhl's disease, hypoplastic right ventricle, noncompacted left ventricle, valvular aortic stenosis, and right coronary artery fistula to the right ventricle. Despite this serious condition, the fetal hydrops did not develop. The baby was born at 33 weeks of gestation but died on day two. Our experience suggests that some babies may survive the fetal period even with the severe type of an unguarded tricuspid valve. Hence, efficient fetal and neonatal treatment strategies for fetal unguarded tricuspid valves are crucial.

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