Abstract

The number of women of childbearing age who have congenital heart disease is increasing.1 A similar proportion of these women can be expected to attempt pregnancy as women in general.2 Although most of these women can be expected to tolerate pregnancy well, particularly if the pregnancy is carefully planned following appropriate clinical evaluation, not all pregnancies are planned, and some will occur in women with unsuitable hemodynamics. We report the case of a 21-year-old woman born with pulmonary atresia and a ventricular septal defect (VSD), ultimately palliated by a fenestrated closure of VSD and a valved homograft conduit placement between the right ventricle and the main pulmonary artery at the age of 3 years, who presented at 16 weeks gestation with a severely degenerate conduit. A 21-year-old woman had been born with pulmonary atresia and a VSD. She was initially palliated with bilateral Blalock-Taussig shunts (classical left and modified right) in the neonatal period because her pulmonary artery confluence was hypoplastic. At 3 years of age, she underwent reparative surgery consisting of closure of the VSD with a fenestrated patch and the placement of a 19-mm homograft valved conduit between the right ventricular outflow tract (RVOT) and the pulmonary artery bifurcation, thereby stimulating growth of the hypoplastic pulmonary artery confluence. The bilateral Blalock-Taussig shunts were ligated. At the age of 18 years, both of her pulmonary arteries were stented because of distorted and stenotic branch pulmonary arteries. The fenestration of the VSD patch was closed interventionally by the use of a muscular VSD closure device. At 20 years of age, there was clinical and echocardiographic evidence of degeneration of the homograft conduit, and she was advised against pregnancy pending further evaluation. Approximately 6 months later, she presented at 16 weeks gestation of an unplanned pregnancy. At this stage, …

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