Document Detail

Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.
MedLine Citation:
PMID:  14985486     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown. METHODS: We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [+/-SD] age, 36+/-12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up. RESULTS: Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P<0.001) and remained so after adjustment for other possible risk factors in a proportional-hazards regression model. CONCLUSIONS: Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Therapeutic trials targeting this population of patients are indicated.
Mark T Gladwin; Vandana Sachdev; Maria L Jison; Yukitaka Shizukuda; Jonathan F Plehn; Karin Minter; Bernice Brown; Wynona A Coles; James S Nichols; Inez Ernst; Lori A Hunter; William C Blackwelder; Alan N Schechter; Griffin P Rodgers; Oswaldo Castro; Frederick P Ognibene
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The New England journal of medicine     Volume:  350     ISSN:  1533-4406     ISO Abbreviation:  N. Engl. J. Med.     Publication Date:  2004 Feb 
Date Detail:
Created Date:  2004-02-26     Completed Date:  2004-03-03     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0255562     Medline TA:  N Engl J Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  886-95     Citation Subset:  AIM; IM    
Copyright Information:
Copyright 2004 Massachusetts Medical Society
Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, Md 20892-1662, USA.
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MeSH Terms
Anemia, Sickle Cell / complications*,  drug therapy,  mortality
Antisickling Agents / therapeutic use
Blood Pressure
Cause of Death*
Drug Resistance
Echocardiography, Doppler
Hydroxyurea / therapeutic use
Hypertension, Pulmonary / classification,  etiology*,  physiopathology,  ultrasonography
Logistic Models
Pulmonary Artery / physiopathology,  ultrasonography
Risk Factors
Survival Rate
Tricuspid Valve Insufficiency / physiopathology,  ultrasonography
Ventricular Function
Reg. No./Substance:
0/Antisickling Agents; 127-07-1/Hydroxyurea
Comment In:
N Engl J Med. 2004 Jun 10;350(24):2521-2; author reply 2521-2   [PMID:  15195345 ]
N Engl J Med. 2004 Feb 26;350(9):857-9   [PMID:  14985481 ]
N Engl J Med. 2004 Jun 10;350(24):2521-2; author reply 2521-2   [PMID:  15190149 ]

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