Pulmonary atresia and intact ventricular septum. Definitive repair in the neonatal period.
Pulmonary atresia with an intact ventricular septum and a small right ventricle is associated with high mortality and lacks a consensus surgical approach. The results of operations in eight of eleven patients with either pulmonary atresia and an intact ventricular septum or critical pulmonary stenosis, hypoplastic right ventricle, and intact ventricular septum, who were operated on between 1983 and 1989, are presented. Definitive correction was performed via a right ventricular transannular patch with prolonged postoperative prostaglandin E1 infusion. Limiting conditions in using this approach were (1) severely hypoplastic right ventricle, (2) massive tricuspid regurgitation, or (3) right ventricle-dependent coronary artery blood supply. Eleven neonates had the aforementioned diagnoses; eight underwent definitive repair, five successfully. Successful outcome, up to 5 postoperative years, was achieved if the tricuspid valve diameter was greater than or equal to 0.75 cm, or if the tricuspid/mitral valve ratio was greater than or equal to 0.70. Other significant predictors of success were a tripartite right ventricle (p less than 0.006), lack of sinusoids (p less than 0.05), the ratio of the right ventricular internal and external diameters greater than or equal to 0.73 (p less than 0.05), and some contractility (p less than 0.04). Thus we choose a right ventricular transannular patch with long-term prostaglandin E1 infusion for patients with pulmonary atresia and intact ventricular septum if (1) they have a tricuspid valve diameter of 0.75 cm or 70% of the mitral valve size, (2) they have a tripartite right ventricle, (3) they exhibit some right ventricular contractility, (4) they do not have marked tricuspid valve insufficiency, and (5) the coronary arteries do not fill primarily from sinusoids.
Section of Pediatric Cardiology, Medical College of Georgia, Augusta 30912.
F M
FM
McCaffrey
L
L
Leatherbury
H V
HV
Moore
D000527:Alprostadil / Q000627:therapeutic use; D015150:Echocardiography, Doppler; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000254:growth & development; D006801:Humans; D007231:Infant, Newborn; D011183:Postoperative Complications; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery
D000527:Alprostadil