Original clinical science
Risk assessment in pulmonary hypertension associated with heart failure and preserved ejection fraction

https://doi.org/10.1016/j.healun.2011.11.017Get rights and content

Background

Pulmonary hypertension (PH) is common in patients with left heart failure (HF), especially those with HF and preserved ejection fraction (HFpEF). However, there is limited data on risk stratification in these patients.

Methods

Baseline clinical and hemodynamic variables of 339 patients with World Health Organization (WHO) Group 2 PH, 90% of whom had HFpEF, were studied to derive a multivariate Cox proportional hazards model. A simplified prognostic risk score was created based on the outcome of all-cause mortality. Nine predictors, significant after stepwise multivariable regression (p < 0.05), were used to create the risk score. Components of the risk score were functional class, diastolic blood pressure, pulmonary artery saturation, interstitial lung disease, hypotension on initial presentation, right ventricular hypertrophy, diffusion capacity of the lung for carbon monoxide, and 2 serum creatinine variables (≤ 0.9 mg/dl and ≥ 1.4 mg/dl).

Results

Overall 2-year survival was 73.8% ± 2.4% in the derivation cohort, and 87.5% ± 2.3%, 66.4% ± 4.9%, and 24.4% ± 6.7% for risk scores of 0 to 2, 3 to 4, and 5+, respectively (p < 0.0001 for the trend), with a C-index of 0.76 (95% confidence interval [CI], 0.71–0.81). The risk score was validated in 2 independent PH-HFpEF cohorts: 179 patients with a C-index of 0.68 (95% CI, 0.55–0.80) and 117 patients with a C-index of 0.68 (95% CI, 0.53–0.83). For the 3 cohorts combined (N = 635), the overall C-index was 0.72 (95% CI 0.68–0.76). In all 3 cohorts individually and in the 3 cohorts combined, the risk score predicted death (hazard ratio, 1.4–1.6; p < 0.01).

Conclusions

Several clinical factors independently predict death in PH-HFpEF confirmed by validation. A novel risk score composed of these factors can be used to determine prognosis and may be useful in making therapeutic decisions.

Section snippets

Data collection

The model derivation sample, the Pulmonary Hypertension Connection (PHC) registry, was initiated in March 2004 and consists of patients evaluated at a single practice at 3 university hospitals—University of Illinois, Rush University Medical Center, and University of Chicago, all Chicago, Illinois—between 1982 and 2009, as described in detail previously.10 Patients were entered retrospectively from 1982 to February 2004 and prospectively from March 2004 onward. The PHC registry is based on

Demographics

Mean age on entry into the study was 63 ± 14 years, and 79% of patients were female. Comorbid conditions were highly prevalent, including hypertension (67%), obesity (39%), and diabetes (33%). Ethnicity data was available in only 98 patients (29%), of whom 63% were white, 33% were black, 3% were Hispanic, and 0.3% were Asian. Owing to the lack of complete data, the prognostic utility of ethnicity could not be determined.

The PHC registry largely consisted of HFpEF patients, which comprised 90.0%

Discussion

In this study we created a prognostic risk score for PH-HFpEF. The validation of our risk score in 2 independent PH-HFpEF cohorts (including 1 prospective, systematic cohort of patients with HFpEF) with varying disease severity illustrates the importance and consistency of the components of our risk model. Our current risk model compares favorably with other well-known prognostic models in systolic heart failure and cardiac transplantation, with similar discriminatory power, as illustrated by

Disclosure statement

The authors thank those individuals who have seen patients at the PH center and those who helped with data entry, including Jill Russo, RN, APN, Thenappan Thenappan, MD, and Tobias Perrino. We also thank Dave P. Miller for his excellent guidance and statistical support.

Sanjiv J. Shah reports receiving research grants from the American Heart Association, the National Institutes of Health, Gilead Sciences, Actelion Pharmaceuticals, the American Society of Echocardiography, the Heart Failure

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