Page 42 - Assessing right ventricular function and the pulmonary circulation in pulmonary hypertension Onno Anthonius Spruijt

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Abstract
Introduction: This study sought to determine whether a simple score combining indexes of right ventricular (RV) function and right atrial (RA) size would offer good discrimination of outcome in patients with pulmonary arterial hypertension (PAH). Identifying a simple score of outcome could simplify risk stratification of patients with PAH and potentially lead to improved tailored monitoring or therapy.
Methods: We recruited patients from both Stanford University (derivation cohort) and VU University Medical Center (validation cohort). The composite endpoint for the study was death or lung transplantation. A Cox proportional hazard with bootstrap CI adjustment model was used to determine independent correlates of death or transplantation. A predictive score was developed using the beta coefficients of the multivariable models.
Results: For the derivation cohort (n=95), the majority of patients were female (79%), average age was 43±11 years, mean pulmonary arterial pressure was 54±14 mmHg, and pulmonary vascular resistance index was 25±12 WUxm2. Over an average follow-up of 5 years, the composite endpoint occurred in 34 patients, including 26 deaths and 8 patients requiring lung transplant. On multivariable analysis, RV systolic dysfunction grade (HR: 3.4 per grade; 95%CI: 2.0-7.8 p<0.001), severe RA enlargement (HR: 3.0 95%CI: 1.3-8.1 p=0.009), and systemic blood pressure <110 mmHg (HR: 3.3; 95% CI: 1.5 to 9.4; p < 0.001) were independently associated with outcome. A right heart (RH) score constructed on the basis of these 3 parameters compared favorably with the National Institutes of Health survival equation (AUC=0.88; 95%CI: 0.79-0.94 vs. AUC=0.60; 95%CI: 0.49-0.71 p<0.001) but was not statistically different than the REVEAL (Registry to Evaluate Early and Long- Term PAH Disease Management) score c-statistic of 0.80 (95%CI: 0.69-0.88) with p=0.097. In the validation cohort (n=87), the RH score remained the strongest independent correlate of outcome. Conclusions: In patients with prevalent PAH, a simple RH score may offer good discrimination of long-term outcome.