Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

BACKGROUND: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. METHODS: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. RESULTS: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P = .0001). Patients achieving 2 or 3 noninvasive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P = .02; hazard ratio: 4.258, CI: 1.143-15.860, P = .031; hazard ratio: 0.095, CI: 0.013-0.672, P = .018, respectively). CONCLUSIONS: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.