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  • Title: Early and late results in pulmonary atresia and intact ventricular septum.
    Author: Hawkins JA, Thorne JK, Boucek MM, Orsmond GS, Ruttenberg HD, Veasy LG, McGough EC.
    Journal: J Thorac Cardiovasc Surg; 1990 Oct; 100(4):492-7. PubMed ID: 1699087.
    Abstract:
    We examined the early and late results of operations in 29 consecutive neonates with pulmonary atresia and intact ventricular septum treated from 1980 to 1988. Transventricular pulmonary valvotomy and central aorta-pulmonary artery shunting were performed in 19 of 22 infants who had a patent infundibulum. Pulmonary valvotomy alone was performed in 3 of the 22 infants with a patent infundibulum, but 2 of these required subsequent systemic-pulmonary artery shunts. Primary shunting was used to palliate 7 infants who had absent infundibular portions of the right ventricle and a very diminutive right ventricular cavity. Tricuspid valve excision and atrial septectomy were also performed in 5 of these 7 infants to decompress large fistulous communications between the right ventricule and coronary artery. Two early deaths (2/29, 6.9%) occurred overall. Both were in infants who had a very small right ventricle. Definitive operation has been accomplished in 16 patients; 13 have had closure of residual interatrial communications and shunt ligation with no deaths, and 3 have undergone modified Fontan repair with 1 death. Actuarial survival rate for the entire group, including operative deaths, is 86% at 5 years. The technique of transventricular pulmonary valvotomy and systemic-pulmonary artery shunting offers a reliable means of palliating neonates with pulmonary atresia and intact ventricular septum and obtains good late right ventricular growth. Systemic-pulmonary shunting, tricuspid valvectomy, and atrial septectomy may offer a means of reducing or obliterating right ventricular-coronary artery fistulas.
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