Reexamining Interstage Home Monitoring After the Norwood Operation

Survival for children born with congenital heart disease has improved dramatically over the past 3 decades. Of the >35 000 children undergoing congenital heart surgery across the United States each year, >95% now survive to hospital discharge.1 Even for complex lesions such as hypoplastic left heart syndrome that were once uniformly fatal as recently as the early 1980s, early survival is now ≈90% at experienced centers. However, as we have begun to learn more about the longer-term outcomes of these patients in recent years, it has become apparent that, despite these gains in early survival, continued mortality over the mid- and long-term remains a challenge. For example, in the recent Pediatric Heart Network Single Ventricle Reconstruction Trial, which enrolled 549 patients undergoing the Norwood operation (randomly assigned to a right-ventricle-to-pulmonary-artery shunt versus modified Blalock-Taussig shunt), transplant-free survival in the overall cohort was 64% at a mean follow-up of 4.8 years.2 One of the most high-risk periods is known to be the interstage period between discharge from the Norwood operation (stage 1) and stage 2 palliation (bidirectional Glenn or hemi-Fontan operation) typically performed at 4 to 6 months of age. In the Single Ventricle Reconstruction Trial, the interstage mortality rate was 12%.3Article see p 502In 2006, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was formed, and now includes >50 congenital heart programs engaged with patients and families in promoting collaboration across sites and quality improvement activities.4 The first project initiated by the collaborative focused on the interstage period, with a primary goal of reducing interstage mortality and improving quality of life. The key drivers that were deemed necessary to achieve these goals included engaging parents, improving care transitions at Norwood discharge, optimizing growth, and improving coordination among the care team and families.4 Multiple …