A Risk Score for Adults With Congenital Heart Disease Undergoing Heart Transplantation

Background. This study derived and validated a risk score for 1-year mortality in patients with adult congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). Methods. The United Network for Organ Sharing registry identified patients with ACHD (>= 18 years of age) who underwent OHT between 1987 and 2018. The primary outcome was 1-year mortality. Associated covariates (univariate P < .2) were entered into a multivariable logistic regression model. Variable inclusion in the model was assessed by improvement in the McFadden pseudo-R-2, likelihood ratio test, and C-index. A risk score was created using the absolute magnitude of the odds ratios from the derivation cohort, and its ability to predict 1-year mortality was tested in the validation cohort. Results. A total of 1388 recipients were randomly divided into derivation (66.7%, n = 950) and validation (33.3%, n = 438) cohorts. A 13-point risk score incorporating 4 pretransplant variables (age, dialysis dependence, serum bilirubin level, and mechanical ventilation) was created. The predicted 1-year mortality ranged from 14.6% (0 points) to 49.9% (13 points) (P < .001). In weighted regression analysis, there was a strong correlation between predicted 1-year mortality and observed 1-year mortality in the validation cohort (r = 0.85, P < .001). Logistic regression also demonstrated a significant association (odds ratio, 1.18; 95% confidence interval, 1.1-1.3; P = .004). The Brier score of the composite score in the validation cohort was 0.14. KaplanMeier analysis demonstrated that risk scores of 4 points or higher portended worse survival at 1-year posttransplant (P < .001). Conclusions. This 13-point risk score for ACHD is predictive of mortality within 1 year after OHT and has potential utilization in improving recipient selection for OHT in adult patients with CHD. (C) 2021 by The Society of Thoracic Surgeons

Automated summary

This study developed and validated a 13-point risk score for predicting 1-year mortality in adult congenital heart disease patients undergoing orthotopic heart transplantation. The risk score showed a strong correlation with observed mortality rates and could help in improving recipient selection for heart transplantation in this population.