Patients who underwent congenital heart surgery in childhood remain at risk for advanced heart failure (HF) that results in death, transplantation, or placement of a ventricular assist device (VAD) until early in life. adulthood at higher rates than the general population, according to a study published in the American Journal of Cardiology.
The retrospective cohort study included patients from the Pediatric Cardiac Care Consortium (PCCC) from 1982 to 2003. Eligible participants underwent congenital heart surgery when they were under the age of 21 and were linked to the US National Death Index and Organ Procurement and Transplantation Network. data base. The primary outcome was a composite of HF-related mortality, heart transplantation, or VAD placement.
A total of 47,033 patients underwent congenital heart surgery during the study period, of whom 35,610 (52% male) survived their congenital heart surgery without a transplant and were included in the analysis. Patients had a median follow-up of 22.9 years (IQR, 19.3 to 27.0) and a median age at end of follow-up of 25.5 years (IQR, 20.9 to 30.9).
After 804,578 person-years of follow-up, 799 patients had the primary outcome of heart failure-related mortality, heart transplantation, or VAD placement at a median of 2.1 years (IQR, 0.4 to 12.1) after discharge from index hospitalization.
The adjusted sub-risk ratio for moderate defects of the 2 ventricle was 3.21 (95% CI, 2.28-4.52), 9.46 for severe defects of the 2 ventricle (95% CI , 6.71-13.3) and 31.8 for abnormalities of only one ventricle (95% CI , 22.2-45.6), compared with mild malformations of both ventricles.
A significantly higher incidence of heart failure, transplant, or VAD-related mortality at 25 years was observed in patients with a systemic right ventricle compared to those with a systemic left ventricle. CI-specific mortality was higher in all severity groups compared to the age- and sex-matched general population in the entire cohort and among 5-year survivors.
The study authors noted that approximately 18% of PCCC patients had inadequate identifiers for coupling, and sensitivity analysis showed that these patients were younger and had more severe forms of congenital heart disease (CHD) . Therefore, the incidence of HF-related events may be underestimated. Additionally, the use of death certificates may have led to underreporting of CI as a cause of death, and the high number of patients missing racial data did not allow a robust analysis of this effect.
“Further follow-up is needed as these patients age, to determine the changing burden of disease as risk factors for underlying coronary artery disease begin to interact with known adult risk factors for acquired heart disease,” the researchers wrote. “Close monitoring for early development of [HF] is warranted for this population, and studies of [HF] Therapeutic therapies (such as afterload-reducing agents, beta-blockers, and sacubitril/valsartan) are urgently needed for subgroups most at risk of [HF].”
Wright LK, Zmora R, Huang Y, et al. Long-term risk of heart failure-related death and heart transplantation after congenital heart surgery in childhood (from the Pediatric Cardiac Care Consortium). Am J Cardiol. Published online January 3, 2022. doi: 10.1016/j.amjcard.2021.11.052