Pulmonary Valve Atresia with Intact Ventricular Septum and Severe Aortic Stenosis

Excerpt

A 19-year-old at 16 weeks of gestation was referred for evaluation our institution. The fetal echocardiogram showed pulmonary atresia with intact ventricular septum. Figures 1 and 2 show the most relevant aspects of postnatal echo study. Pulmonary atresia (Fig. 1, arrow) with a hypoplastic right ventricle and tricuspid valve, coronary sinusoids, and a patent ductus arteriosus were evident. Surprisingly, severe aortic valve stenosis (Fig. 2, arrow) and a dilated and hypertrophied left ventricle were also noted. The maximum, aortic pressure gradient measured by continuous-wave Doppler was 45–50 mmHg. The infant received prostaglandin E1, and a pulmonary valvulotomy, a systemic–pulmonary shunt, and an aortic valvulotomy were planned. Unfortunately, the infant died after an episode of irreversible low cardiac output. Autopsy confirmed the echocardiographic findings. Severe left ventricular hypertrophy (Fig. 3, dark arrow) and a thickened aortic valve (Fig. 3, light arrows) were noted on macroscopic pathology.

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