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

摘要

Inclusion CriteriaFollow-UpStatistical AnalysisResultsPatients’ Characteristics, Morphologic and Operative DetailsHospital ComplicationsTime-Related Survival After SurgeryRight Ventricular Outflow Tract Reoperations After Biventricular RepairCommentStudy LimitationsConclusionDiscussionReferencesThe 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.MethodsBetween 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.ResultsMedian 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).ConclusionsPrimary 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.Pulmonary atresia and ventricular septal defect (PAVSD) is a congenital cardiac anomaly characterized by the absence of continuity between the right ventricle and pulmonary arteries, together with the presence of a malalignment ventricular septal defect due to the anterior deviation of the infundibular septum [