title: Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.
abstract: Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.
mesh_terms: D001011:Aorta / Q000601:surgery; D001807:Blood Vessel Prosthesis; D004374:Ductus Arteriosus, Patent / Q000601:surgery; D006349:Heart Valve Diseases / Q000601:surgery; D006350:Heart Valve Prosthesis; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008722:Methods; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000151:congenital / Q000601:surgery*; D012086:Reoperation; D012189:Retrospective Studies; D014262:Tricuspid Valve Insufficiency / Q000601:surgery