Invited Commentary

Abstract

The National Paediatric Cardiology Quality Improvement Collaborative (NPC-QIC; http://npcqic.org) is a multicenter initiative led by Cincinatti Children’s Hospital and created with the mission of standardizing care and improving outcomes for infants with single-ventricle congenital heart disease. The group began in 2007 by implementing practice change among participating institutions. Since then, outcomes have been monitored and studied through a registry that now encompasses data from more than 2000 patients, and lessons learned from this research have driven further practice changes. Evans and colleagues [1Evans C.F. Sorkin J.D. Abraham D.S. et al.Interstage weight gain is associated with survival after first-stage single-ventricle palliation.Ann Thorac Surg. 2017; 104: 674-680Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar] interrogated the registry to answer the question: Is interstage weight gain after stage 1 palliation associated with improved survival? They identified 1358 patients undergoing non-hybrid stage 1 palliation operated over a 7-year period. An association between weight gain in the interstage period and survival (both overall and transplant-free) was shown in adjusted analysis that was not present in simple univariable analysis. The magnitude of this gain is small but significant and would not have been detected were it not for the quantity of longitudinal follow-up data within the registry. The major limitation of this dataset is that it captures only the fraction of patients who were discharged between stage 1 and stage 2, which is a select subgroup of patients. In addition, the data comes from a group of institutions for whom hypoplastic left heart syndrome is the predominant single ventricle morphology. Finally, the effect of weight gain on the hospital mortality after stage 2 could not be analyzed. These limitations notwithstanding, to our knowledge this is the first study to demonstrate an association between survival and weight gain between stages 1 and 2. Although optimizing weight gain is already a major focus of care in the interstage period, these data demonstrate that we need to do something more for patients who, for whatever reason, are not gaining weight. Aggressive reintervention for arch or shunt obstruction or, more controversially, earlier performance of the stage 2 operation may be beneficial in the subset of patients struggling to gain weight. Although data from the Pediatric Heart Network Infant Single Ventricle trial previously demonstrated higher procedural risk for those undergoing stage 2 at a younger age [2Cnota J.F. Allen K.R. Colan S. et al.Superior cavopulmonary anastomosis timing and outcomes in infants with single ventricle.J Thorac Cardiovasc Surg. 2013; 145: 1288-1296Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar], a recent report from the NPC-QIC registry suggests similar outcomes and less interstage attrition among centers performing the stage 2 operation earlier [3Hill G.D. Rudd N.A. Ghanayem N.S. Hehir D.A. Bartz P.J. Center variability in timing of stage 2 palliation and association with interstage mortality: a report from the National Pediatric Cardiology Quality Improvement Collaborative.Pediatr Cardiol. 2016; 37: 1516-1524Crossref PubMed Scopus (22) Google Scholar]. Interstage Weight Gain Is Associated With Survival After First-Stage Single-Ventricle PalliationThe Annals of Thoracic SurgeryVol. 104Issue 2PreviewLow birth and operative weight have been identified as risk factors for death after first-stage single-ventricle palliation. We hypothesize that weight gain after the first-stage operation is associated with transplant-free interstage survival to admission for the second-stage operation. Full-Text PDF