The Fontan Adult Brompton clinical score and mortality risk

Area of Science:

• Cardiology
• Congenital Heart Disease
• Adult Congenital Heart Disease

Background:

• Adults with Fontan palliation have an elevated risk of premature death.
• Personalized care is crucial for this growing population with complex congenital heart disease.

Purpose of the Study:

• To develop a risk score for predicting mortality or heart transplantation in adults with Fontan circulation.
• To utilize widely available clinical variables for risk stratification at the first adult visit.

Main Methods:

• Retrospective study of adults with Fontan circulation at their first adult congenital heart disease visit.
• Multivariate prognostic model developed using stepwise backward conditional approach.
• External validation of the derived risk score in a separate cohort.

Main Results:

• A risk score was derived using age, atrial tachyarrhythmia history, heart failure, NYHA class, systolic blood pressure, and oxygen saturation.
• The score effectively discriminated patients into low, intermediate, and high risk groups (C-index: 0.71).
• External validation demonstrated excellent discriminative ability (C-index: 0.92).

Conclusions:

• A novel, externally validated clinical score identifies adults with Fontan palliation at risk of mortality or transplantation.
• This score facilitates personalized care for adults with complex congenital heart disease.
• The risk score is based on readily available clinical variables.