[Biventricular repair with a modified Glenn shunt for the hypoplastic right ventricle]

Between 1982 and 1990, 14 patients with small right ventricle underwent biventricular repair with a modified Glenn shunt. The patients consisted of 8 cases of pulmonary atresia and intact ventricular septum, 2 with pulmonary stenosis and intact ventricular septum, 3 with tetralogy of Fallot, 1 with pulmonary atresia and straddling tricuspid valve. Nine patients had one or more prior preliminary palliative procedures. Repairs consisted of a modified Glenn shunt and closure of the intracardiac and extracardiac shunt, with right ventricular outflow reconstruction in 13, and pulmonary valvotomy in 1. In nine patients superior vena cava was not ligated. There was one operative death (7%). Preoperative RVEDV ranged from 19 to 70% of normal with a mean of 36.1% of normal. Preoperative pulmonary resistance ranged from 1.6 to 5.3 unit with a mean of 2.8 unit. Preoperative PA index ranged from 102 to 444 mm2/m2 with a mean of 234.3 mm2/m2. No patients died later. Follow-up 2 to 9 year after operation showed that 9 patients were in the New York Heart Association class I and 4 were in class II. Our experience shows that this procedure can be safely done for patients, who have hypoplastic right ventricle smaller than 40% of normal and are not candidate for Fontan procedure because of high pulmonary vascular resistance and inadequate size of pulmonary artery. In this procedure a modified Glenn shunt without ligation of SVC may effectively reduce the volume overload on the right ventricle.