心室中隔欠損を伴わない肺動脈閉鎖症

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.

BACKGROUND: The timing of patent ductus arteriosus (PDA) closure after valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) remains controversial. CASE SUMMARY: A 23-day-old neonate with PA-IVS and tripartite right ventricle underwent valvotomy. Immediate PDA closure was subsequently performed because systemic diastolic pressure remained low despite adequate antegrade pulmonary flow, suggesting clinically significant ductal runoff with risk of systemic hypoperfusion. Given the patient's impaired renal function, the PDA closure was conducted using zero-contrast technique under minimal fluoroscopic guidance. DISCUSSION: Concomitant valvotomy and PDA closure may be considered in selected PA-IVS patients when antegrade pulmonary flow is adequate but ductus arteriosus remains hemodynamically significant. TAKE-HOME MESSAGE: Timing of ductal occlusion in PA-IVS requires individualized hemodynamic judgment, with zero-contrast PDA closure strategy as feasible alternative in renal dysfunction patients.

Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) associated with multiple large, coronary artery fistula represents a rare and complex congenital heart defect. The presence of Right Ventricular Dependent Coronary Circulation (RVDCC) critically influences management and prognosis. We report the case of a newborn with PA/IVS, extensive coronary fistulae involving both the right and left coronary arteries. A staged, stepwise approach was undertaken to exclude RVDCC, followed by transcatheter closure of the coronary fistulae and ballon perforation of the pulmonary valve. This sequential approach facilitated gradual right ventricular decompression and the establishment of antegrade pulmonary blood flow while minimizing the risk of coronary ischemia. Definitive biventricular circulation was subsequently achieved with placement of a right ventricle-to-pulmonary artery (RV-PA) conduit, resulting in right ventricular pressures decreasing to approximately onehalfsystemic levels.This case demonstrates the feasibility and effectiveness of a staged, multidisciplinary hybrid approach in carefully selected patients with PA/IVS and complex coronary artery fistulae. It underscores the importance of comprehensive coronary assessment and individualized management in optimizing outcomes in this challenging congenital cardiac anomaly.

IMAGES: Pulmonary atresia with intact ventricular septum and right ventricle (RV)-to-coronary fistula were assessed using intraoperative transesophageal echocardiography (TEE). CASE SUMMARY: A neonate with pulmonary atresia with intact ventricular septum and extensive RV-coronary communications underwent surgery. Intraoperative TEE showed retrograde right coronary flow; temporary fistula clamping restored antegrade flow without ventricular dysfunction, permitting safe ligation, tricuspid valve plasty, and RV-pulmonary artery reconstruction. TAKE-HOME MESSAGES: Intraoperative TEE can confirm coronary perfusion in real time during fistula test clamping. It helps determine whether RV decompression can be performed safely.

OBJECTIVE: The aim of this study was to analyze the echocardiographic characteristics, intrauterine progression, postnatal outcomes, and follow-up results of fetuses diagnosed with critical pulmonary stenosis with an intact ventricular septum (CPS/IVS) and pulmonary atresia with an intact ventricular septum (PA/IVS) using echocardiography. METHODS: A retrospective cohort study was conducted on fetuses with isolated PA/IVS and CPS/IVS, collecting both morphological and hemodynamic echocardiographic data and following them postoperatively. RESULTS: A total of 48 cases were included in the study. On the initial ultrasound, eight cases were diagnosed with PA/IVS and 40 cases with CPS/IVS. The right ventricle/left ventricle long axis (RV/LV) ratio, right ventricle long axis (RV-L) z-score, and pulmonary artery/aorta (PA/AO) ratio for PA/IVS were significantly lower than those for CPS/IVS (RV/LV P < 0.001; RV-L P = 0.002; PA/AO P = 0.003). Severe tricuspid regurgitation (TR) was predominant in both PA/IVS and CPS/IVS, with six and 28 cases, respectively. A significant increase in RV-L, tricuspid valve (TV) annulus, and pulmonary valve (PV) was observed with gestational age and fetal growth. In an exploratory analysis, earlier gestational age at first ultrasound was associated with neonatal intervention (hypothesis-generating due to small sample size). Post-surgical follow-up showed improvements in RV/LV ratio, tricuspid valve/mitral valve (TV/MV) ratio, and PV z-score (P < 0.05), suggesting favorable short-term echocardiographic postoperative outcomes. CONCLUSION: In this cohort in which all infants achieved biventricular circulation, PA/IVS fetuses showed more compromised RV and PV annular development than CPS/IVS. Serial echocardiography demonstrated measurable growth of RV/TV/PV with gestation, while postnatal and post-intervention follow-up showed improved RV/LV ratio and PV z-scores. These findings support the descriptive value of echocardiography for longitudinal assessment and perioperative follow-up within an integrated management pathway.