心室中隔欠損を伴わない肺動脈閉鎖症

BACKGROUND: Pulmonary atresia with intact ventricular septum management has evolved, with increased use of transcatheter right ventricular decompression, but the impact on contemporary outcomes remains unknown. This study evaluates contemporary national outcomes in patients aged 0 to 18 years. METHODS: Patients with pulmonary atresia with intact ventricular septum undergoing cardiac intervention (2000-2022) in England and Wales were linked to multiple national data sets. Mortality (early and late), reintervention, and hospital stay were described overall and by treatment pathway. RESULTS: Among 488 included patients with pulmonary atresia with intact ventricular septum, 58.8% underwent right ventricular decompression as the first procedure, predominantly transcatheter (84.8%), while 32.4% received stage 1 palliation (arteriopulmonary shunt or ductal stenting), and 8.8% died or were lost to follow-up before either intervention. Overall, 54.5% achieved biventricular (BiV) repair, 26.7% underwent functionally single-ventricle palliation, and 18.8% were noncommitted. Overall group in-hospital mortality was 11.7%. The median follow-up was 8.9 years, with 1- and 5-year mortality of 19.8% (95% CI, 16.2%-23.2%) and 20.8% (95% CI, 17.1%-24.4%), respectively. Stage 1 palliation as the initial therapy had higher 1-year mortality than right ventricular decompression (37.2% [29.0%-44.5%] versus 7.7% [4.5%-10.7%]). Cardiac reintervention was 62.6% (56.1%-68.4%) at 10 years after BiV repair and 32.8% (21.5%-44.4%) at 10 years after palliative stage 3. Median hospital stay was greatest in the first year (35 days), declining to 2 days annually by 10 years, with functionally single-ventricle patients requiring more days in hospital than BiV patients (52 versus 28). CONCLUSIONS: In a contemporary pulmonary atresia with intact ventricular septum cohort, transcatheter right ventricular decompression was the predominant initial intervention. This approach had lower in-hospital mortality compared with older multicenter series, with BiV repair achieved in more than half of the patients. Early mortality after nonantegrade flow augmentation remains high, likely due to nonmodifiable risk factors in severe cases. Long-term survival and hospital stay remain better for those amenable to BiV circulation, reflecting more favorable anatomy compared with those on the single-ventricle pathway, although reintervention is lower in the latter.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare critical congenital heart defect. Although biventricular repair is reserved for those with adequate right ventricular development, significant outcome variability persists even among anatomically similar patients, implicating non-structural determinants of surgical recovery. Gestational age has been recognized as a prognostic factor in various congenital heart diseases, yet its specific impact on short-term outcomes after biventricular repair for PA/IVS remains inadequately characterized. METHODS: This retrospective cohort study enrolled 97 consecutive pediatric patients with PA/IVS who underwent biventricular repair at a single tertiary referral center from 1999 to 2024. Participants were categorized as preterm or term according to standard gestational age criteria. RESULTS: The cohort comprised 21 preterm infants and 76 term infants. Multivariable analysis showed each additional gestational week reduced adverse outcome risk by 45% (OR 0.55, 95% CI 0.41-0.75). Term infants demonstrated significantly lower risk vs. preterm infants (OR 0.14, 95% CI 0.04-0.46). Restricted cubic spline regression revealed a nonlinear threshold effect (P-nonlinear=0.047) with an inflection point at 37.6 weeks (95% CI 36.6-38.7). Beyond this threshold infants had a 92% lower risk compared with those under 37.6 weeks (OR 0.08, 95% CI 0.02-0.42). CONCLUSIONS: This study establishes a gestational age threshold effect for short-term outcomes in PA/IVS patients undergoing biventricular repair. Extending gestation beyond 37.6 weeks significantly improves short-term outcomes in PA/IVS patients undergoing biventricular repair, providing crucial evidence-based guidance for perinatal management.