Long-term follow up (10.0yrs on average) is described of 33 patients with coronary aretry fistulae (CAF) without other cardiac disorders. Of 33 patients, nine cases demonstrated some electrocardiographic ischemic changes (group I), and other twenty-four cases showed no significant electrocardiographic changes (group II) clinically. Symptomatic, hemodynamic and angiographic findings showed no significant difference statistically between both groups. Thirty patients of small to moderate left-to-right shunt ratio (<50∼60%) showed hemodynamic and angiographic stability during the 10.0 years retrograde follow-up period on average. Of 33 patients, seventeen cases have underwent surgical closure of CAF since 1973 to 1990. Seven of the seventeen patients had showed significant myocardial ischemic changes before surgical treatments, and five of the seven cases showed definitive improvement of the electrocardiographic changes seemed to be derived from occlusion of the coronary steal blood flow after surgical operations. There were no operative deaths and no appearance of new myocardial ischemia following the surgical repairs. At present since not only it is unclear whether the CAF predisposes correlate coronary arteries to some premature atherosclerosises but also there are several reports of severe late risks of surgical repair of CAF, surgical indication in the patients of CAF with small-to-moderate left-to-right shunt shou ld be limited to the cases with such as definitive myocardial ischemia or evidence of infective endocarditis.

We performed the Senning operation and pulmonary valvotomy in an 11-month-old baby with transposition of the great arteries (TGA) with an intact ventricular septum (IVS), and bicuspid pulmonary valvular stenosis associated with pulmonary hypertension (PH). Preoperative catheterization showed a pressure gradient (PG) between the left ventricle (LV) and main pulmonary artery (MPA) of 35mmHg, mean pulmonary artery pressure (MPAP) of 56mmHg, and pulmonary vascular resistance (PVR) of 11.2unit·m². The pure oxygen inhalation test showed a decrease in MPAP from 56 to 38mmHg, and a decrease in PVR from 11.2 to 5.5 unit·m². We could not perform lung biopsy to determine the surgical indications in terms of PH due to preoperative progressive congestive heart failure in this patient. Postoperative catheterization (28 days after the Senning operation) showed a decrease in PG between the LV and MPA to 8mmHg, and MPAP also decreased to 17mmHg. Two radical operations were possible in this patient. One was the arterial switch operation (ASO), and the other was the atrial switch operation, i. e. the Senning or the Mustard operation. We selected the Senning operation because there was the possibility that the new aortic valve might develop persistent stenosis and regurgitation after ASO and pulmonary valvotomy. The Senning operation may be an alternative in selected patients with TGA with IVS and pulmonary valvular stenosis.