Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant ventriculo-arterial connexions and a relatively large hypoplastic right ventricle, one year after an atrio-ventricular type of Fontan operation by means of a valveless woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical recovery of the patient. The use of a valved conduit is recommended when an atrioventricular type of Fontan repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial connexions and relatively large hypoplastic right ventricle.

Two infants with aortico-left ventricular tunnel and associated aortic valvular disease were operated upon. The first patient, age 28 months, had successful surgical closure of the anomalous communication. Four years postoperatively he is asymptomatic, but mild aortic regurgitation persists. The second infant, aged 3 months (the youngest reported surgical case), also had severe aortic stenosis (dysplastic valve) and mitral regurgitation. She died, twelve hours after the operation, from intractable left ventricular failure. On the basis of the previously reported cases (22), the clinical profile of the malformation is outlined with special reference to associated aortic valve lesions. Early operation is recommended because of the poor natural history of the condition.

One hundred-five patients (median age 14 months) in whom a PTFE prosthesis was used to create a systemic-pulmonary artery shunt were studied between 1978 and 1980. The prosthesis was mainly used to create a modified Blalock-Taussig anastomosis. Nine patients died in hospital (8.5%, 70% confidence limits: 5.7% to 12.3%). The clinical condition did not appear to be an incremental risk factor, while the young age and the underlying type of disease accounted for the hospital mortality to a large extent. All 96 survivals were available for follow-up information. One graft, which was damaged intraoperatively, occluded 3 months later. The remaining patients have a patent graft 3 months to 36 months (mean 24 months) postoperatively. In 6 patients the patency was assessed angiographically and no damages of the pulmonary artery were observed, therefore this type of palliation is advisable for any patient, particularly in the first year of life.

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Although the occurrence of subpulmonary stenosis resulting from displacement of an aneurysm of the membranous septum is well known, resultant subaortic stenosis has not been well documented. We present an unusual case of acquired pulmonary atresia with restrictive ventricular septal defect and associated severe subaortic resulting from an aneurysm of the membranous septum.

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Fourteen patients younger than two years of age with persistent truncus arteriosus underwent primary repair. Twelve of them were less than 1 year and 4 less than 3 months of age. Intractable heart failure was the indication for surgery in all patients but one who had increased pulmonary vascular resistance. There were 5 hospital and 2 late deaths. Six out of the 7 survivors (median follow-up: 29 months) were symptom-free. The remaining infant who preoperatively had significant truncal valve regurgitation was doing fairly well 2 1/2 years after repair. Our experience suggests that, although the mortality remains high, primary repair for infants with persistent truncus arteriosus is feasible and offers better overall results than does pulmonary artery banding followed by later intracardiac repair. We advise primary repair for all infants with intractable heart failure or increasing pulmonary vascular resistance with or without truncal valve regurgitation. Elective repair is recommended before the age of 2 years to minimize the risk of pulmonary vascular disease.

Two hundred eighteen consecutive patients with tetralogy of Fallot (TOF) underwent surgical treatment under a prospective protocol during a three-year period (January 1978 to December 1980). No patient was refused the operation. Eighty-three patients had palliative operations without hospital deaths (50% less than 1 year of age). One hundred thirty-five had total correction with 5 hospital deaths (3.7%; 70% C.L. = 2.0 to 6.2). Thirty-five of them had had palliation in the first year of life (one hospital death at time of correction). Incremental risk factors were young age (p less than 0.0002), transannular patch (p = 0.13) and primary repair (p = 0.38). Significant stenosis in the pulmonary artery branches were eliminated utilizing an original table of relationship between the diameter of the expected normal pulmonary valve annulus and the calculated diameter of the branches. The immediate post-repair peak systolic pressure ratio between right and left ventricles (Prv/lv) was only 0.39 and the incidence of transannular patches was remarkably low (34%). It is concluded that surgical management of tetralogy of Fallot can be achieved, today, with a very low hospital mortality reserving a two-stage procedure only for small infants (less than 1 year of age). Accurate criteria for the reconstruction of the right ventricle outflow tract (RVOT) can easily allow very low rates of transannular patches and postoperative RV hypertension with an ultimate better preservation of the RV function.