Right Heart Score for Predicting Outcome in Pulmonary Arterial Hypertension | Journal Scan

Study Questions:

Can a simple score combining indexes of right ventricular (RV) function and right atrial (RA) size offer good discrimination of outcome in patients with pulmonary arterial hypertension (PAH)?


Subjects were recruited from both Stanford University (derivation cohort) and VU University Medical Center (Amsterdam, Netherlands) (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.


For the derivation cohort (n = 95), 79% were female, average age was 43 ± 11 years, mean PA was 54 ± 14 mm Hg, and pulmonary vascular resistance index was 25 ± 12 Wood units per m2. Over an average follow-up of 5 years, the composite endpoint occurred in 34 patients, including 26 deaths and eight lung transplants. On multivariable analysis, RV systolic dysfunction grade (hazard ratio [HR], 3.4 per grade; p < 0.001), severe RA enlargement (HR, 3.0; p = 0.009), and systemic blood pressure <110 mm Hg (HR, 3.3; p < 0.001) were independently associated with outcome. A right heart (RH) score constructed on the basis of these three parameters compared favorably with the National Institutes of Health survival equation (0.88 vs. 0.60, 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 with p = 0.097. In the validation cohort (n = 87), the RH score remained the strongest independent correlate of outcome.


In patients with prevalent PAH, a simple RH score may offer good discrimination of long-term outcome.


Indices for predicting death or lung transplant in PAH suffer from many confounders including the need for reasonably long duration of follow-up during which time treatment options change, the initial change from baseline, and a relatively inconsistent time point at which to make the observations. The definition of first outpatient visit after stabilization on disease-modifying medications was used for the RH score. When studying patients on treatment, I would prefer the response at 6 months after beginning a disease-modifying treatment so as to eliminate many of the biases inherent in clinical follow-up. However, the simplicity of the RH score and the finding of no additional value of many of the classic other variables (New York Heart Association class, N-terminal pro–B-type natriuretic peptide, 6-minute walk distance, and invasive hemodynamic data) will make the score very attractive if it can be validated in much larger cohorts. The utility in connective tissue diseases and other Group 1 PAH cohorts remains to be determined.

Clinical Topics: Anticoagulation Management, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Pulmonary Hypertension, Echocardiography/Ultrasound, Hypertension

Keywords: Arterial Pressure, Blood Pressure, Connective Tissue Diseases, Disease Management, Echocardiography, Hypertension, Hypertension, Pulmonary, Incidence, Lung Transplantation, Outpatients, Peptide Fragments, Universities, Vascular Resistance, Ventricular Function, Right, Toxins, Biological

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