Pulmonary artery stenosis in hybrid single-ventricle palliation: High incidence of left pulmonary artery intervention.

Abstract

Pulmonary artery growth is an important determinant of outcome in single-ventricle strategies. Higher rates of pulmonary artery intervention have been reported with hybrid-based palliation when compared with Norwood palliation. We performed a retrospective review of pulmonary artery growth and clinical outcomes in patients undergoing hybrid-based single-ventricle palliation. The stage I hybrid procedure was performed in 72 patients between 2004 and 2012, of whom 54 were on a Fontan palliative pathway. Thirty-four infants completed stage II, and 20 infants underwent the Fontan operation. The mean diameters of the right pulmonary artery (5.6 ± 1.9 mm) and left pulmonary artery (5.6 ± 2.1 mm) were similar before stage II. After stage II, the right and left pulmonary artery diameters were 8.5 ± 2.1 mm and 5.8 ± 1.3 mm, respectively (P < .001), and after the Fontan operation, these were 8.8 ± 2.0 mm and 6.4 ± 1.1 mm, respectively (P = .002). The mean right pulmonary artery z score was normal throughout, but the left pulmonary artery did not maintain a normal size. The cumulative pulmonary artery intervention rate was 50% at any time after stage II. Fifteen interventions (88%) were performed after stage II (35% during the same hospitalization, 71% <60 days). The most intervened site was the midsection of the left pulmonary artery (41%). Initial pulmonary artery intervention was balloon dilation in 59% of patients and stent implantation in 41% of patients. Half of patients with initial balloon dilation required reintervention. There is significant risk of left pulmonary artery compromise after the second stage of hybrid palliation associated with a high intervention rate.