Original Research
Right Heart Score for Predicting Outcome in Idiopathic, Familial, or Drug- and Toxin-Associated Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.jcmg.2014.12.029Get rights and content
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Abstract

Objectives

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).

Background

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 mm Hg, and pulmonary vascular resistance index was 25 ± 12 Wood units × m2. 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 (hazard ratio [HR]: 3.4 per grade; 95% confidence interval [CI]: 2.0 to 7.8; p < 0.001), severe RA enlargement (HR: 3.0; 95% CI: 1.3 to 8.1; p = 0.009), and systemic blood pressure <110 mm Hg (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 (0.88; 95% CI: 0.79 to 0.94 vs. 0.60; 95% CI: 0.49 to 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 to 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.

Key Words

atrial function
heart failure
outcome
pulmonary hypertension
right heart

Abbreviations and Acronyms

NT-proBNP
N-terminal pro–B-type natriuretic peptide
PAH
pulmonary arterial hypertension
PVRI
pulmonary vascular resistance index
RAEF
right atrial emptying fractions
RAP
right atrial pressure
RVEDA
right ventricular end-diastolic area
RVEF
right ventricular ejection fraction
RVESA
right ventricular end-systolic area
RVFAC
right ventricular fractional area change
RVGLS
right ventricular global longitudinal strain
RVMPI
right ventricular myocardial performance index
SBP
systolic blood pressure
TAPSE
tricuspid annular systolic excursion

Cited by (0)

This work was supported by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford University, Stanford Cardiovascular Institute, and the Pai Chan Lee Research Fund. Dr. Denault has received speaker fees from Covidien and CAE Healthcare. Dr. Brunner has been a consultant for Actelion and Bayer. Dr. Zamanian has received research grants from United Therapeutics and Actelion; and has received consulting fees from United Therapeutics and Bayer. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.