Timing of Intervention and Long-Term Outcomes in Mild Congenital Heart Disease: A 35-Year Study From the Pediatric Cardiac Care Consortium.

BACKGROUND

Mild congenital heart diseases such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and pulmonary valve stenosis constitute a significant public health problem. Understanding long-term outcomes after interventions for mild congenital heart disease is essential to inform lifelong care.

METHODS

We queried the Pediatric Cardiac Care Consortium, a multicenter US-based registry of patients with congenital heart disease interventions, for patients undergoing surgical or transcatheter procedures for atrial septal defect, ventricular septal defect, patent ductus arteriosus, and pulmonary valve stenosis; 17 407 patients were included. Statistical methods included survival analysis using Kaplan-Meier plots, multivariable Cox proportional hazards models for risk factors for death and standardized mortality ratios.

RESULTS

There were 115 in-hospital deaths (0.7%), with the remaining patients included in the postdischarge. The 35-year post-discharge survival ranged from 95.5% for pulmonary valve stenosis to 92.1% for ventricular septal defect. Survival patterns differed by lesion and age at intervention, with intervention at 1≤5 years generally associated with more favorable outcomes. Lower weight-for-age at intervention was associated with an increased hazard of death across all shunt lesions but not for pulmonary valve stenosis. Compared with the general population, mortality risk remained elevated for up to 18 to 20 years after intervention.

CONCLUSIONS

Long-term survival after intervention for mild congenital heart disease is excellent; yet lesion-specific risks persist for decades. Age and weight-for-age at intervention are associated with survival, together with excess mortality beyond childhood underscore the need for lifelong, lesion-tailored surveillance in this growing population.