Article

Hemodynamic Predictors of Mortality in Adults with Sickle Cell Disease

Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blool Institute, Bethesda, Maryland, United States.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 11.99). 01/2013; 187(8). DOI: 10.1164/rccm.201207-1222OC
Source: PubMed

ABSTRACT BACKGROUND: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and post-capillary PH. OBJECTIVE: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. METHODS: Nine year follow-up data (median 4.7 years) from the NIH SCD PH screening study are reported. Five hundred twenty-nine adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥25 mmHg. Survival rates were estimated by the Kaplan-Meier method and mortality risk factors were analyzed by the Cox proportional hazards regression. MEASUREMENTS AND MAIN RESULTS: Specific hemodynamic variables were independently related to mortality: mean PAP (HR 1.61, 95% CI 1.05- 2.45, per 10 mmHg increase, P=0.027), diastolic PAP (HR 1.83, 95% CI 1.09-3.08, per 10 mmHg increase, P=0.022), diastolic PAP - pulmonary capillary wedge pressure (HR 2.19, 95% CI 1.23-3.89, per 10 mmHg increase, P=0.008), transpulmonary gradient (HR 1.78, 95% CI 1.14-2.79 per 10 mmHg increase, P=0.011), pulmonary vascular resistance (HR 1.44 , 95% CI 1.09-1.89 per Wood unit increase, P=0.009 ) as risk factors for mortality. CONCLUSION: Mortality in adults with SCD and PH is proportional to the physiological severity of pre-capillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.

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