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

October 7, 2021

A team of UPMC and UPMC Children’s Hospital of Pittsburgh cardiologists and heart surgeons published a study that aimed to determine a risk score for 1-year mortality in patients with adult congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).

Advances in treatment approaches and surgical repair for congenital heart disease (CHD) during infancy has allowed for more patients with CHD to reach adulthood. Conversely, older patients with ACHD are a complex group that face a higher risk for advanced, medically refractory heart failure. Patients with ACHD who require advanced heart failure treatment often undergo OHT. 

UPMC’s Laura Seese, MD, MS, Victor O. Morell, MD, Melita Viegas, MD, Mary Keebler, MD, Gavin Hickey, MD, and Yisi Wang, MPH, conducted a study that aimed to refine the process for optimum recipient selection for OHT in the ACHD population.

The study population consisted of 1,338 adult patients identified in the United Network for Organ Sharing (UNOS) registry who had a diagnosis of CHD and underwent OHT between 1987 and 2018. The study population was randomly divided into two cohorts, a derivation cohort consisting of approximately 66% of the study participants and a validation cohort composed of the residual 34%.

Pretransplant variables within the derivation cohort like the individual’s age, dialysis dependence, serum bilirubin level, and mechanical ventilation status were factors that influenced the development of the 13-point risk score. The risk score’s ability to predict 1-year mortality was tested in the validation cohort. 

The composite score was generated by summing the individual scores for each patient in the derivation and validation cohorts. Weighted regression analysis was used to correlate between predicted 1-year mortality in patients with ACHD with the observed rates of 1-year mortality. Logistic regression, calibration plots, and Brier score were also used to assess the association of the ACHD risk score with 1-year mortality.

Results from the study showed that the predicted 1-year mortality ranged from 14.6% (0 points) to 49.9% (13 points) (P < .001).1 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).1 

After the analysis of 1,338 OHT recipients from the UNOS data registry, the study concluded that the 13-point risk score for ACHD is a strong predictor of mortality within 1 year after OHT. It is also believed that the 13-point risk score optimizes the recipient selection process for OHT in adult patients with CHD.

Read more about the results of the study here.

1. Seese L, Morell VO, Viegas M, et al. A Risk Score for Adults With Congenital Heart Disease Undergoing Heart Transplantation. Ann Thorac Surg. 2021; 111(6): 2033-2040. doi:10.1016/j.athoracsur.2020.05.154