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

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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.

Section snippets

Methods

We performed a retrospective cohort study using data prospectively collected from the Pediatric Cardiac Care Consortium (PCCC), a large US-based registry for interventions for pediatric heart diseases that has been previously well described.12, 13 Briefly, the PCCC was established in 1982 to allow collaboration between pediatric cardiovascular centers with the aim to improve outcomes for patients with congenital or acquired heart diseases.14 Estimates report that the PCCC contains 15% to 30% of

Results

There were 2,424 patients from 43 centers who met study inclusion criteria. In-hospital mortality occurred in 57 patients (2.4%). Of the 2,367 patients who were discharged from the hospital alive, 1,913 patients (80.8%) contained adequate identifiers to be submitted to NDI and Organ Procurement and Transplant Network. With a median follow-up of 17.7 years (twenty-fifth to seventy-fifth percentile: 14.4 to 22.0), survival among these patients was 97.5% at 1 year after repair and 95.6% at 20

Discussion

In this retrospective cohort study from the PCCC, the largest study of its kind in this population to our knowledge, we found that the survival to early adulthood was excellent among those who survive to hospital discharge after initial surgical repair of CoA during infancy. Overall, survival in this large cohort of patients followed for nearly 2 decades are similar to previously reported results.4, 5, 6, 7, 8, 9, 10,17 However, the large number of patient-years in this cohort allowed us to

Acknowledgment

We thank the program directors and data collection coordinators from the participating PCCC centers. Susan Anderson and Brian Harvey were especially instrumental in the management of PCCC and initial linkage of this cohort with mortality information.

Disclosures

The data reported here have been supplied by UNOS as the contractor for the Organ Procurement and Transplantation Network (OPTN). The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy of or interpretation by the OPTN or the US Government.

References (33)

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Funding: NHLBI 5R01 HL122392-04.

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