We aimed to describe the longitudinal risk of advanced heart failure (HF) leading
to death, heart transplantation, or ventricular assist device (VAD) placement after
congenital heart surgery (CHS) and how it varies across the spectrum of congenital
heart disease. We linked the records of patients who underwent first CHS in the Pediatric
Cardiac Care Consortium between 1982 and 2003 with the United States National Death
Index and Organ Procurement and Transplantation Network databases. Primary outcome
was time from CHS discharge to HF-related death, heart transplant, or VAD placement,
analyzed with proportional hazards models accounting for competing mortality. In 35,610
patients who survived a first CHS, there were 799 HF deaths, transplants, or VADs
over a median of 23 years (interquartile range, 19 to 27). Cumulative incidence at
25 years was 2.3% (95% confidence interval [CI] 2.1% to 2.4%). Compared to mild 2-ventricle
defects, the adjusted subhazard ratio for moderate and severe 2-ventricle defects
was 3.21 (95% CI 2.28 to 4.52) and 9.46 (95% CI 6.71 to 13.3), respectively, and for
single-ventricle defects 31.8 (95% CI 22.2 to 45.6). Systemic right ventricle carried
the highest risk 2 years after CHS (subhazard ratio 2.76 [95% CI 2.08 to 3.68]). All
groups had higher rates of HF-related death compared with the general population (cause-specific
standardized mortality ratio 56.1 [95% CI 51.0 to 61.2]). In conclusion, the risk
of advanced HF leading to death, transplantation, or VAD was high across the spectrum
of congenital heart disease. While severe defects carry the highest risk, those with
mild disease are still at greater risk than the general population.
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Article Info
Publication History
Published online: January 03, 2022
Received in revised form:
November 16,
2021
Received:
August 25,
2021
Footnotes
Dr. Wright and Dr. Kochilas was supported by the National Institutes of Health. Dr. Oster and Dr. Kochilas was supported by the United States Department of Defense.
Identification
Copyright
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