Marked Enlargement of the Right Pulmonary Artery 19 Years After Waterston Operation

A 31-year-old woman who underwent the Waterston operation 19 years previously for a single ventricle and severe pulmonary stenosis is described. Her exercise tolerance and hematocrit were unchanged postoperatively, but the the continuous murmur disappeared. Doppler echocardiography showed blood flow from the ascending aorta to the markedly enlarged right pulmonary artery across the Waterston anastomosis in all cardiac cycles. The development of pulmonary hypertension and a muffling effect of the markedly enlarged pulmonary artery may have had roles in the disappearance of the continuous murmur despite the patient's relatively good condition.