Early experience with the two patch technique for complete atrioventricular septal defect.

A total of 12 cases underwent repair of complete atrioventricular (AV) septal defect utilising the two-patch technique. There were 4 males and 8 females. The mean age at repair was 9.6 months (range, 3 to 49 months). The average weight was 5.4 kg (range, 3.5 to 13 kg). Five had associated patent ductus arteriosus. A Gore-Tex patch and glutaraldehyde preserved pericardium was utilised for the ventricular and atrial portions of the defects respectively in all patients. Four of these were done under hypothermic circulatory arrest. There were no intra-operative deaths. Early mortality comprised of 2 patients (2/12, 16.6%). One due to a pulmonary hypertensive crisis and the other to septicemia. The mean duration of ventilatory support was 62 hours (range, 24 to 192 hours). The mean duration of inotropic support was 57 hours (range, 24 to 192 hours). Primary repair of AV septal defects using the two-path of technique can be accomplished with a low early mortality in carefully selected patients.

Early experience in the surgical treatment of the univentricular heart.

Eighteen patients under went surgical treatment of univentricular hearts between April 1989 till October 1992. All 18 had palliative operations in the form of: a modified Blalock-Taussig shunt (5), pulmonary artery banding (2), bidirectional Glenn shunts (2), conventional Fontan repair (1) and total cavo pulmonary connection (8). There were 11 males and 7 females. The mean age at operation was 4.5 years (range, 1 month to 24 years). The average weight at operation was 12.2 kg (range 2 to 43 kg). Associated anomalies included: transposition of the great arteries (4), pulmonary stenosis (9), pulmonary atresia (1), mitral atresia (1), atrioventricular septal defect (1) and patent ductus arteriosus (3). There were no intra operative deaths. There were 5 early deaths (27.7%). Causes of death were due to: blockage of a modified Blalock-Taussig shunt (1), respiratory failure (1), persistent ventricular arrhythmias (1) and low output syndrome (2). Treatment in the form of a systemic to pulmonary arterial shunt or an operation based on the Fontan principle may offer an initial palliative or definitive correction for this condition.

Total cavopulmonary connection--early results.

Presently total cavopulmonary connection is used as an alternative in complex congenital disorders requiring atriopulmonary connection. From January 1989 till November, 1992, a total of 18 cases underwent total cavopulmonary connection. There were 8 males and 10 females. The mean age at operation was 92 months (range 5 to 504 months). The average weight was 19.6 kg (range 5.25 to 42 kg). Diagnoses included: tricuspid atresia (6); univentricular heart (7); pulmonary atresia with intact interventricular septum (1); double outlet right ventricle, pulmonary stenosis with uncommitted ventricular septal defect (2); corrected transposition of the great arteries with multiple ventricular septal defects (1) and complete atrioventricular septal defect with double outlet right ventricle (1). The mean duration of ventilatory and inotropic support was 127 hours (range 12 to 528 hours) and 140 hours (range 24 to 528 hours) respectively. The average duration of hospital stay was 17.9 days (range 5 to 30 days). There was no intra-operative mortality. Early mortality was 22.2% (4/18). Pre-operative diagnoses of patients who died were: tricuspid atresia (1), univentricular heart (1), complete atrio-ventricular septal defect with double outlet right ventricle (1) and double outlet right ventricle, pulmonary stenosis with uncommitted ventricular septal defect (1). The causes of death were: low-output syndrome (3) and septicemia (1). Although these initial results with total cavopulmonary connection suggest a lower early mortality compared to other modifications of the Fontan principle, long-term follow-up would be necessary to assess the late implications of this procedure.