Document Detail

Hemodynamic predictors of mortality in adults with sickle cell disease.
MedLine Citation:
PMID:  23348978     Owner:  NLM     Status:  MEDLINE    
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 postcapillary PH.
OBJECTIVES: 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 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 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 mm Hg. 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 (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14-2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality.
CONCLUSIONS: Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.
Alem Mehari; Shoaib Alam; Xin Tian; Michael J Cuttica; Christopher F Barnett; George Miles; Dihua Xu; Catherine Seamon; Patricia Adams-Graves; Oswaldo L Castro; Caterina P Minniti; Vandana Sachdev; James G Taylor; Gregory J Kato; Roberto F Machado
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural    
Journal Detail:
Title:  American journal of respiratory and critical care medicine     Volume:  187     ISSN:  1535-4970     ISO Abbreviation:  Am. J. Respir. Crit. Care Med.     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-04-16     Completed Date:  2013-06-11     Revised Date:  2014-04-15    
Medline Journal Info:
Nlm Unique ID:  9421642     Medline TA:  Am J Respir Crit Care Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  840-7     Citation Subset:  AIM; IM    
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MeSH Terms
Anemia, Sickle Cell / complications*,  mortality
Cardiac Catheterization
Clinical Trials as Topic
Echocardiography, Doppler
Follow-Up Studies
Hypertension, Pulmonary / diagnosis,  etiology*,  mortality
Kaplan-Meier Estimate
Logistic Models
Mortality, Premature
Proportional Hazards Models
Pulmonary Wedge Pressure / physiology
Risk Assessment
Survival Rate
Grant Support
Comment In:
Am J Respir Crit Care Med. 2013 Apr 15;187(8):790-2   [PMID:  23586378 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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