Pulmonary hypertension after Blalock-Taussig anastomosis

A patient with Fallot's tetralogy who had bilateral subclavian-pulmonary anastomoses at 12 years of age was found to have severe pulmonary hypertension 8 years later. The pressure and vascular resistance in the pulmonary circulation were equal to the systemic circulation. The pulmonary stenosis was severe, and virtually all the pulmonary blood flow occurred via the anastomoses. Despite severe pulmonary stenosis, no systolic pressure gradient was demonstrated across the right ventricular outflow tract, and the angiocardiographic picture simulated that of pulmonary atresia. Systolic and diastolic murmurs at the upper left sternal border were present, simulating the usual subclavian-pulmonary anastomotic murmur but probably arising from aortic insufficiency. Hemoptysis and gross enlargement of the pulmonary artery were clinical clues pointing to the true situation, which was revealed by cardiac catheterization. Repeat study 3 years later showed slight clinical and hemodynamic improvement.