e373T he 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.
Case ReportA 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, she was asymptomatic and clinically well. At 20 weeks, she remained asymptomatic but was showing clinical evidence of hemodynamic decompensation with raised jugular venous pressure and echocardiographic evidence of worsening right ventricular (RV) dilation with high RV pressure. There was severe pulmonary regurgitation and significant RVOT obstruction with Doppler evidence of RV hypertension. Obtaining a good-quality Doppler signal across the RVOT was difficult but was at least 3 m/s. There was evidence of fetal growth retardation from an obstetric/fetal well-being assessment. At 23 weeks, the otherwise healthy female fetus had an estimated weight of 480 g (expected mean weight at 24 weeks is 600 g).The clinical view was that the patient was at risk of sudden hemodynamic decompensation or life-threatening arrhythmia. There was concern that the pregnancy might not be sustained long enough for the fetus to reach a viable maturity.The management options available were to let the pregnancy continue, deliver the baby at the earliest possible gestation, surgically replace the conduit, or imp...