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Updated: Dec 13, 2025

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
A Risk Score for Adults With Congenital Heart Disease Undergoing Heart Transplantation
Laura Seese1, Victor O Morell2, Melita Viegas2
1Division of Cardiac Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
Insights
A new 13-point risk score predicts 1-year mortality in adult congenital heart disease (ACHD) patients after heart transplantation (OHT). This tool can aid in selecting suitable candidates for OHT, improving patient outcomes.
Area of Science:
- Cardiology
- Transplantation Medicine
- Medical Statistics
Background:
- Adult congenital heart disease (ACHD) patients represent a growing population requiring advanced cardiac interventions.
- Orthotopic heart transplantation (OHT) is a critical treatment for end-stage heart failure in ACHD patients.
- Predicting post-transplant mortality is essential for optimizing recipient selection and management.
Purpose of the Study:
- To derive and validate a risk score for predicting 1-year mortality in ACHD patients undergoing OHT.
- To identify key pre-transplant variables associated with 1-year mortality after OHT in ACHD.
- To develop a tool for improving recipient selection in ACHD OHT.
Main Methods:
- Utilized the United Network for Organ Sharing registry data from 1987-2018 for ACHD patients (≥18 years) undergoing OHT.
- Developed a multivariable logistic regression model using derivation cohort (n=950) and validated in a separate cohort (n=438).
- Created a 13-point risk score based on pre-transplant variables: age, dialysis dependence, serum bilirubin, and mechanical ventilation.
Main Results:
- The 13-point risk score incorporated four pre-transplant variables and predicted 1-year mortality ranging from 14.6% to 49.9%.
- The risk score demonstrated strong correlation with observed mortality in the validation cohort (r=0.85, P<.001).
- Risk scores of 4 or higher were associated with significantly worse 1-year post-transplant survival (P<.001).
Conclusions:
- A validated 13-point risk score accurately predicts 1-year mortality in adult congenital heart disease patients post-OHT.
- This risk score can potentially enhance recipient selection for OHT in adult patients with congenital heart disease.
- The developed tool offers a valuable addition to clinical decision-making for ACHD patients awaiting or undergoing OHT.
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-R2, 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. Kaplan-Meier 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.
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