Circulation. 2000 Apr 18;101(15):1826-32.
Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients.
Reddy VM, McElhinney DB, Amin Z, Moore P, Parry AJ, Teitel DF, Hanley FL.
Divisions of Cardiothoracic Surgery, University of California, San Francisco 94143-0118, USA.
BACKGROUND: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients.
METHODS AND RESULTS: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients.
CONCLUSIONS: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.
MAPCAs arrangement in 11 patients with PA-VSD reported in JTCVS 1997 paper from Quebec:
J Thorac Cardiovasc Surg 1997;114:727-737. One-satge midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aorto-pulmonary collaterals. Christo I. Tchervenkov et al.
Group I (pt #1-6) had multi-stage repair:
Group II (pt #7-11) had single-stage repair:
Also see other postings on this subject:
Pre-op evaluation of PAs and MAPCAs
MAPCAs in PA-VSD
Surgery Algorithms for PA-VSD
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