Abstract
Objectives
Despite a remarkable progress in the management of pulmonary arterial hypertension (PAH) over the past decade, PAH still has a poor long-term outlook and remains an incurable condition. Risk assessment continues to be refined to identify patients at risk of early morbidity and mortality. We aimed to evaluate a new model, the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score proposed for the follow-up of patients in our cohort.
Methods
We enrolled a mixed prevalent and incident cohort of patients with idiopathic PAH (20%), heritable (2.5%), congenital heart disease (CHD) (59.2%), and connective tissue diseases (18.3%) subsets (n=121) from 4 PAH centers from March 2007 to January 2019. Individual patient REVEAL 2.0 risk scores were applied at 12 months after the diagnosis (follow-up). Risk scores were calculated with 7 or more variables. Kaplan-Meier survival was estimated for a simplified three-category (low, intermediate, and high risk) model up to 60 months from 1 year after the diagnosis, with all-cause mortality as the end point. Log-rank test was used to compare estimates.
Results
The mean age was 46±16 years (78.5% women). 2/3 of the patients were CHD. The median survival was 60 months. 25 patients had died. The majority of the patients were NYHA FC II (48.8%) and III (42.1%) at follow-up. Figure 1 demonstrates KM 12- and 60- month survival in our cohort according to the simplified three-category (low, intermediate, and high risk) REVEAL 2.0 model. Overall, 58.7%, 15.7%, and 25.6% of the patients were classified as low, intermediate, and high risk, respectively, at follow-up. The REVEAL 2.0 model effectively discriminated risk in our cohort. Both 1 year- and 5 year-survival differed significantly between the 3 risk categories: 12-month mortality estimates were 1.41% for low risk, 5.3% for intermediate risk, and 16.1% for high risk (log-rank, P=0.013); and 60- month mortality estimates were 2.8% for low risk, 10.5% for intermediate risk, and 51.6% for high risk (log-rank, P=0.0001).
Conclusions
The REVEAL 2.0 simplified three-category model can be applied for risk assessment of the PAH subsets, particularly congenital heart disease at follow-up.
Figure 1
Funding Acknowledgement
Type of funding source: None
659 Early Outcomes of the CHD LIFE Program Long-Term Developmental Care Pathway for High-Risk Children With Congenital Heart Disease
