Primary Versus Staged Repair in Neonates With Pulmonary Atresia and Ventricular Septal Defect

Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis

“…Forty-eight studies were included in the full text-screening, of which 44 were excluded as they were not meeting the inclusion criteria. Finally, 4 studies [1,2,8,20] were deemed appropriate for inclusion in the final meta-analysis and summarised in the PRISMA flow chart (Fig. 1).…”

“…Pulmonary atresia with ventricular septal defect (PAVSD) is a congenital cardiac anomaly characterised by the absence of continuity between the right ventricle and pulmonary arteries, together with the presence of a malaligned ventricular septal defect (VSD), due to the anterior deviation of the infundibular septum [1]. It affects between 4.2 and 10 per 100,000 live births [2]. Pulmonary atresia can also be associated with intact ventricular septum, major aortopulmonary collateral arteries (MAPCAs), or other congenital heart diseases [3].…”

From Other Journals: A Review of Recent Articles by Our Editorial Team

“…3). Twenty five-year cumulative incidence of coronary Guariento et al [5] Autologous mitochondrial transplantation for cardiogenic shock in pediatric patients following ischemia-reperfusion injury 24 patients included (MT, n = 10; Control, n = 14) over a span of 16 years Markers of systemic inflammatory response and organ function measured 1 day before and 7 days following revascularization did not differ between groups Successful separation from ECMO MT group (n = 8, 80%) Control group (n = 4, 29%) (= 0.02) Median circumferential strain immediately following IRI but before therapy was not significantly different between groups Immediately following separation from ECMO, ventricular strain was significantly better in the MT group (− 23.0%; range, − 20.0% to − 28.8%) compared with the Control group (− 16.8%; range, − 13.0% to − 18.4%) (P = 0.03) Median time to functional recovery after revascularization was significantly shorter in the MT group (2 days vs 9 days; P = .02) Cox regression analysis showed higher composite estimated risk of cardiovascular events in the Control group (hazard ratio, 4.6; 95% confidence interval, 1.0 to 20.9; P = 0.04) MT was associated with successful separation from ECMO and enhanced ventricular strain in patients requiring postcardiotomy ECMO for severe refractory cardiogenic shock after IRI Kim et al [6] Primary versus staged repair in neonates with pulmonary atresia and ventricular septal defect Comparison of initial palliation with BTS followed by second stage repair versus primary biventricular repair 66 neonates (BTS group: n [30, 45.5%) or primary biventricular repair (pBVR group: n [36, 54.5%) Overall mean follow-up duration was 7.51 ± 4.35 years 10-year overall survival was 84.8% (94.4% for pBVR vs 75.7% for BTS, P [0 .032) BTS group, 2 early and 6 interstage mortalities, and the pBVR group had no early and 2 late mortalities In univariable analysis, genetic or extracardiac anomalies were a risk factor for mortality (HR, 5.56; P [0.038) After achieving BVR, the pBVR group underwent significantly more frequent right ventricle outflow tract reinterventions (P < .001) at a much earlier period (P [0.017) compared with the BTS group In neonates with ductal-dependent pulmonary atresia and ventricular septal defect, the primary BVR approach provides an excellent survival rate, but the burden of right ventricle outflow tract reintervention is heavy. The staged approach with BTS promotes pulmonary artery growth, but hospital and interstage mortality are significant artery reoperation was 8% for coronary anomaly patients and 0.9% for normal coronary anatomy patients, P = 0.0143.…”

“…Clinical outcomes in pediatric patients remain poor, with the highest mortality rates or need for orthotopic heart transplantation (OHT) reported among patients requiring ECMO support. The authors have previously demonstrated that intramyocardial transplantation (MT) of autologous mitochondria is safe and associated with recovery of myocardial function [ 6 ].…”

“…With these findings, the authors concluded that MT was associated with successful separation from ECMO and enhanced ventricular strain in patients with postcardiotomy cardiogenic shock needing ECMO [ 6 ].…”