20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries.

BACKGROUND: We have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments. OBJECTIVES: The purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs. METHODS: We reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021. RESULTS: During the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death. CONCLUSIONS: In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve.

BACKGROUND: Various bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus on whether a particular valve is best for this application. Recently, the Inspiris Resilia valve (Edwards Lifesciences Inc) was approved for aortic valve replacement, and surgeons have begun using it for PVR. There is limited evidence on the performance of the Inspiris valve compared with other valves in the pulmonary position. METHODS: This study reviewed all patients who underwent PVR with a size 19- to 29-mm Inspiris valve or Mosaic valve (Medtronic Inc) from 2007 to 2022 at Lucile Packard Children's Hospital Stanford (Palo Alto, CA). Midterm outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient ≥36 mm Hg, and freedom from reintervention. RESULTS: A total of 225 consecutive patients who underwent PVR with a size 19- to 29-mm Mosaic (n = 163) or Inspiris (n = 62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On univariable and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient ≥36 mm Hg. CONCLUSIONS: Early and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to that of other bioprosthetic valves used for PVR.