Hawe, Anthony

Five patients have had the common features of pulmonary atresia or severe stenosis associated with a septal defect and a coronary artery-to-pulmonary artery (CA-PA) fistula. Four had pulmonary valvular atresia, and one had severe pulmonary stenosis. In all five, the CA-PA fistula contributed the principal component of the pulmonary blood flow. Cyanosis, continuous murmur, right ventricular hypertrophy, and decreased or normal pulmonary vascularity were frequent clinical manifestations, but angiocardiography was required to establish the diagnosis. The fistula was a side-to-side communication between the left coronary artery and the main pulmonary artery in each of the five patients. The dilated proximal coronary artery connecting the aortic root with the pulmonary artery gave a unique angiocardiographic appearance distinguishing it from truncus arteriosus or aorticopulmonary window. It is suggested that this unique angiocardiographic appearance be called an "aorticopulmonary tunnel." The surgical repair of this anomaly is closely related to that for severe tetralogy of Fallot and should involve a comparable risk and achieve a comparable result.

Postoperative embolism occurred in 35 of 546 patients traced for 2 to 15 years after surgical repair of uncomplicated ASD. A total of 58 embolic episodes occurred, resulting in the death of 11 of these 35 patients. The incidence of embolism was not significantly related to the technique used for surgical repair. Six of the nine patients who had had a preoperative embolism also had an embolism in the postoperative period. The most significant factors associated with an increased risk of postoperative embolism were increased age of the patient, atrial fibrillation, and pulmonary hypertension, all of which are interrelated: seventy-seven per cent (27/35) of patients having a postoperative embolism had either atrial fibrillation or pulmonary hypertension, and 77% (27/35) of patients with postoperative embolism were more than 40 years old at the time of the first embolism. Because actuarial analysis suggests that 30% of patients more than 40 years of age who have atrial fibrillation after repair of ASD may have an embolism within 10 years postoperatively, continuation of anticoagulation therapy indefinitely in these patients is suggested. The highest risk of embolism occurs in the early postoperative period, suggesting the advisability of anticoagulation therapy for all adult patients during convalescence.