Development and validation of a risk prediction model in patients with adult congenital heart disease

Abstract Aims To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD). Methods and results A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011−2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004–2013). The primary endpoint was the 4-year risk of death, heart failure, or arrhythmia, which occurred in 135 of 602 patients (22%). Model development was performed using multivariable logistic regression. Model performance was assessed with C-statistics and calibration plots. Of the 14 variables that were selected by an expert panel, the final prediction model included age (OR 1.02, 95%CI 1.00–1.03, p  = 0.031), congenital diagnosis (OR 1.52, 95%CI 1.03–2.23, p  = 0.034), NYHA class (OR 1.74, 95%CI 1.07–2.84, p  = 0.026), cardiac medication (OR 2.27, 95%CI 1.56–3.31, p p  = 0.060), BMI (OR 1.03, 95%CI 0.99–1.07, p  = 0.123), and NT-proBNP (OR 1.63, 95%CI 1.45–1.84, p Conclusion The proposed ACHD risk score combines six readily available clinical characteristics and NT-proBNP. This tool is easy to use and can aid in distinguishing high- and low-risk patients, which could further streamline counselling, location of care, and treatment in ACHD.