Anatomy of the Pulmonary Circulation and Outcomes in Tetralogy of Fallot With Major Systemic-to-Pulmonary Collaterals and Unilateral Pulmonary Artery of Ductal Origin.

BACKGROUND: In patients with tetralogy of Fallot (TOF) and multiple aortopulmonary collaterals (MAPCAs), the presence of a ductus arteriosus (DA) supplying all flow to 1 lung and MAPCAs providing all flow to the other lung is a distinct phenotype that has not been well characterized.

AIMS: To characterize the anatomy of the pulmonary circulation and report long-term surgical outcomes in patients with this subtype of TOF/MAPCAs.

METHODS: From our institutional experience with TOF/MAPCAs, we ascertained patients with unilateral ductal origin of a PA who underwent surgery from 11/01 through 4/25. The study was limited to patients who (1) had no prior surgery or a shunt only without modification of MAPCAs or PAs, and (2) had adequate preoperative angiography. The anatomy of the PAs and MAPCAs was characterized in detail from preoperative angiography, and outcomes were assessed.

RESULTS: During the study period, 880 patients with TOF and MAPCAs underwent surgery, 61 (6.9%) in whom the native anatomy comprised a DA to 1 lung and MAPCAs to the contralateral lung. The study cohort consisted of 42 eligible patients. The DA supplied the left PA in 39 patients (91%), but there was no association with aortic arch laterality. MAPCAs to the contralateral lung connected to a fully (n = 12) or partially (n = 9) arborizing central PA in 21 patients. MAPCA origins were similar to the broader TOF/MAPCAs population, except that MAPCAs arising from a coronary artery were more common (14% vs. 5%, p = 0.019). The DA had been stented prior to surgery in 18 patients. At the first surgery (median age 5.1 months), 74% of patients underwent complete repair, and all but 1 ultimately achieved complete repair. During a median follow-up of 6.2 years (2.6, 13.0), 6 patients died. Ten-year transplant-free survival was 86% ± 6%. Freedom from any PA reintervention was 63% ± 9% at 5 years and 59% ± 9% at 10 years.

CONCLUSIONS: The presence of with a DA supplying all flow to 1 normally arborizing PA, almost always the left PA, and MAPCAs providing all flow to the other lung, is a distinct anatomic subset of TOF/MAPCAs. In our practice, stenting of the DA in the neonatal period is the most appropriate therapy, typically followed by PA reconstruction and repair in early infancy, depending on the anatomy of the MAPCAs to the contralateral lung. Outcomes in these patients are generally excellent.