Transplant-Free Survival After Intervention for Mild Congenital Heart Disease: Long-Term Outcomes from the PCCC Open Access

Perlow, Gabriel (Spring 2019)

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Mild congenital heart diseases [CHDs] such as patent ductus arteriosus [PDA], atrial septal defect/patent foramen ovale [ASD], mild pulmonary stenosis [PS], and ventricular septal defect [VSD] have, historically, been considered “cured” after percutaneous or surgical intervention. Studies with sufficient participation and duration to describe the long-term outcomes after surgical or percutaneous intervention are lacking.


The Pediatric Cardiac Care Consortium database was queried for US patients with intervention for any combination of mild CHDs before 21 years. Patients with additional cardiac comorbidities, prematurity, or inadequate identifies for death certificate linkage. Outcomes included transplant, death, and cause of death. Product-limit survival analysis was performed for time to transplant or death among those surviving first intervention. Standardized mortality ratios [SMRs] with the general population were calculated using the CDC WONDER vital statistics database. Cox Proportional Hazards models were generated to assess risk factors for transplant or death.


The cohort for survival analysis included 14,861 patients. Survival at 25 years after first intervention was greater than 97 % for all defects. Survival was significantly greater for PS and ASD than VSD (log-rank p < 0.0001). SMRs were significantly greater than 1 until 18 years after first intervention. The presence of extracardiac comorbidities, being underweight, male sex, and younger age at first surgery are associated with greater risk for transplant or death during follow-up. Cardiovascular- and CHD-related causes of death were most frequent among those in younger age groups at death.


While transplant-free survival after intervention for mild CHDs is excellent, risk for death remains elevated over the general population up to 18 years after first intervention. Extracardiac comorbidities confer significantly greater risk, along with several biometric factors. This study provides pediatric cardiologists with long-term data for advising patients and families, and prompts a deeper analysis of causes of death in mild CHD.

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