The original Fontan operation was employed for patients with tricuspid atresia, but its application has been extended to a broad spectrum of congenital cardiac defects with a functional or anatomical single ventricle. From 1989 to 1995, 35 patients (23 males, 12 females) underwent a modified Fontan procedure; their age ranged from 14 months to 15 years (mean 5.1 a). Indications for operation were the following: D(S)ILV = 16, TA = 9, MA = 3, TGA with straddling AV-valve = 2, DORV = 4 and criss-cross heart = 1. The same surgical technique was utilized in all patients with redirection of the systemic venous return by means of a bidirectional cavopulmonary anastomosis and an intraatrial baffle, adaptable to all the various forms of underlying anatomy. Associated anomalies such as dextrocardia, coarctation, anomalous systemic or pulmonary venous return, subaortic stenosis and situs anomalies were present in 26%, 9%, 26%, 9%, and 20%, respectively. 12 patients were presumed to be at high risk and underwent a two-stage procedure (n = 7 bidirectional Glenn preceding the definitive repair and n = 5 a fenestration of the intraatrial baffle). Risk factors in our group of patients were: age under 2 years, abnormal systemic venous drainage, stenotic pulmonary arteries, PVR over 2 Wood units, mPAP over 15 mmHg, AV-valve incompetence, subaortic obstruction and ventricular dysfunction. Two patients had failure of the total cavo-pulmonary connection necessitating the take-down to a bidirectional Glenn anastomosis in the early postoperative period, with one death. Hospital mortality was 24% (n = 8). One child did not survive complications following the fenestration closure 4 months postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)