Results of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect.

The 2 management strategies of neonates born with ductal-dependent pulmonary atresia and ventricular septal defect (PAVSD) include single stage primary biventricular repair (BVR) or staged palliation with modified Blalock-Taussig shunt (BTS) followed by second stage repair. Each approach is associated with specific benefits and drawbacks. We report outcomes of those 2 different strategies. Between 2002 and 2012, 86 neonates with ductal-dependent PAVSD underwent surgery using primary repair (BVR group: n = 28, 33%) or shunt palliation (BTS group: n = 58, 67%). Early and late results were compared between the 2 groups. Median age was 6 days (interquartile range [IQR] 3-17) and median weight was 2.8 kg (IQR 2.5-3.3) with 27 patients (31%) 2.5 kg or less. Associated risk factors such as prematurity 36 weeks or less and genetic or extra-cardiac malformations were present in 30% and 40% of patients, respectively. Hospital mortality occurred in 5 (5.8%) patients (1 [3.6%] for BVR versus 4 [6.9%] for BTS, p = 1.00). Overall 8-year survival was 76.5% (85.5% for BVR versus 72.2% for BTS, p = 0.189). On multivariable analysis, risk factors for mortality were genetic or extra-cardiac malformations (hazard ratio [HR], 2.8 (95% confidence interval [CI] 1.7% to 16.0%), p = 0.036) and postoperative extracorporeal membrane oxygenation (ECMO) (HR, 4.0 [95% CI, 1.1% to 14.4), p = 0.039). Freedom from right ventricular outflow tract reoperation after achievement of repair was 63.2% at 8 years (52.4% for BVR versus 70.2% for BTS, p = 0.170). On multivariable analysis, risk factors for reoperation were the use of conduit (HR, 8.7 [95% CI, 1.1% to 65.8%], p = 0.037) and prematurity (HR, 2.8 [95% CI, 1.1% to 7.2%], p = 0.028). Primary BVR of neonates with ductal-dependent PAVSD is associated with a trend for improved survival due to hospital and interstage mortality with the staged approach. Genetic or extra-cardiac malformations are common and are associated with worse survival.

Alsoufi B ，Mori M ，McCracken C ，Williams E ，Samai C ，Kogon B ，Ferguson E ，Kanter K ... -  《-》

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

The 2 surgical strategies for neonates with ductal-dependent pulmonary atresia and ventricular septal defect are primary biventricular repair (BVR) or initial palliation with a modified Blalock-Taussig shunt (BTS) followed by second stage repair. In this study, we report the combined outcomes from 2 hospitals using different strategies. Between 2004 and 2017, 66 neonates underwent surgery with palliative shunts (BTS group: n = 30, 45.5%) or primary biventricular repair (pBVR group: n = 36, 54.5%). The 2 groups were similar in age, body weight, and Nakata index scores. The overall mean follow-up duration was 7.51 ± 4.35 years, and early and late results were compared between the groups. The 10-year overall survival was 84.8% (94.4% for pBVR vs 75.7% for BTS, P = .032). The BTS group had 2 early and 6 interstage mortalities, and the pBVR group had no early and 2 late mortalities. In the BTS group, the Nakata index score significantly increased during the interstage period (P < .001). In univariable analysis, genetic or extracardiac anomalies were a risk factor for mortality (hazard ratio, 5.56; P = .038). After achieving BVR, the pBVR group underwent significantly more frequent right ventricle outflow tract reinterventions (P < .001) at a much earlier period (P = .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. Genetic and extracardiac anomalies are significant risk factors for mortality.

Kim ER ，Lee CH ，Kim WH ，Lim JH ，Kim YJ ，Min J ，Cho S ，Kwak JG ... -  《-》

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes. SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041]. Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

Alsoufi B ，Gillespie S ，Mori M ，Clabby M ，Kanter K ，Kogon B ... -  《-》

Establishment of right ventricle-pulmonary artery continuity as the first-stage palliation in older infants with pulmonary atresia with ventricular septal defect may be preferable to use of an arterial shunt.

Right ventricle-pulmonary artery (RV-PA) conduit and systemic-to-pulmonary artery (S-PA) shunt in younger infants for the first-stage palliation with pulmonary atresia with ventricular septal defect (PAVSD) obtained good results. However, the pulmonary arteries (PA) grow slow in older infants undergoing an S-PA shunt. We compared the clinical outcomes of the two procedures in older infants with PAVSD. A total of 48 patients with PAVSD underwent the first-stage palliative procedure between January 2010 and July 2012. Patients were divided into the RV-PA group and the S-PA group based on whether they had an RV-PA conduit (n = 24) or an S-PA shunt (n = 24). The early and late outcomes were compared between groups. There was no significant difference in in-hospital mortality, mechanical ventilation time, paediatric intensive care unit stay and hospital stay between groups (all P > 0.05). The RV-PA conduits were associated with better PA growth compared with the S-PA shunts (P < 0.001). The RV-PA group had a higher rate of second-stage biventricular surgery compared with the S-PA group (P = 0.03). The early outcomes among different conduits of the RV-PA conduit were not different (all P > 0.05). A positive correlation was found between the size of conduits and body weight (R(2) = 0.684, P < 0.001). In older infants with PAVSD who underwent the first-stage palliative procedure, early outcomes showed no difference between the RV-PA conduit group and the S-PA shunt group. The RV-PA conduits were associated with better growth of the PA and higher rates of second-stage biventricular repair. Autologous pericardium is a good choice for RV-PA conduits, and there is a correlation between body weight and size of conduit.

Zheng S ，Yang K ，Li K ，Li S ... -  《-》

Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.

Lee WY ，Kang SR ，Im YM ，Yun TJ ... -  《-》