Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium)

• Published in: The American journal of cardiology Vol. 124; no. 5; pp. 795 - 802
• Main Authors: Oster, Matthew E., McCracken, Courtney, Kiener, Alexander, Aylward, Brandon, Cory, Melinda, Hunting, John, Kochilas, Lazaros K.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-09-2019; Elsevier Limited
• Subjects: Adolescent; Adult; Age; Age Factors; Analysis of Variance; Angioplasty; Aortic Coarctation - diagnostic imaging; Aortic Coarctation - mortality; Aortic Coarctation - surgery; Cardiac Surgical Procedures - methods; Cardiac Surgical Procedures - mortality; Cardiovascular diseases; Child; Child, Preschool; Confidence intervals; Congenital diseases; Consortia; Coronary artery disease; Databases, Factual; Defects; Etiology; Female; Heart diseases; Hospitals; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Male; Mortality; Multivariate Analysis; Pediatrics; Procurement; Prognosis; Retrospective Studies; Risk Assessment; Statistical analysis; Studies; Survival; Survivors; Transplants & implants; Treatment Outcome; United States > US
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2019.05.047

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.