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Prepublished online as a Blood First Edition Paper on October 3, 2002; DOI 10.1182/blood-2002-03-0948. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-03-0948v1 101/4/1257    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Castro, O. Articles by Brown, B. D. Search for Related Content PubMed PubMed Citation Articles by Castro, O. Articles by Brown, B. D. Related Collections Red Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 February 2003, Vol. 101, No. 4, pp. 1257-1261 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival Oswaldo Castro, Mohammed Hoque, and Bernice D. Brown From the Center for Sickle Cell Disease and Division of Cardiology, Department of Medicine, Howard University College of Medicine, Washington, DC. Few results on cardiac catheterization have been published for patients with sickle cell disease (SCD) with pulmonary hypertension (PHTN). Their survival once this complication develops is unknown. We analyzed hemodynamic data in 34 adult patients with SCD at right-sided cardiac catheterization and determined the relationship of PHTN to patient survival. In 20 patients with PHTN the average systolic, diastolic, and mean pulmonary artery pressures were 54.3, 25.2, and 36.0 mm Hg, respectively. For 14 patients with SCD without PHTN these values were 30.3, 11.7, and 17.8 mm Hg, respectively. The mean pulmonary capillary wedge pressure in patients with PHTN was higher than that in patients without PHTN (16.0 versus 10.6 mm Hg; P = .0091) even though echocardiography showed normal left ventricular systolic function. Cardiac output was high (8.6 L/min) for both groups of patients. The median postcatheterization follow-up was 23 months for patients with PHTN and 45 months for those without PHTN. Eleven patients (55%) with PHTN died compared to 3 (21%) patients without PHTN (2 = 3.83; P = .0503). The mean pulmonary artery pressure had a significant inverse relationship with survival (Cox proportional hazards modeling). Each increase of 10 mm Hg in mean pulmonary artery pressure was associated with a 1.7-fold increase in the rate (hazards ratio) of death (95% CI = 1.1-2.7; P = .028). The median survival for patients with PHTN was 25.6 months, whereas for patients without PHTN the survival was still over 70% at the end of the 119-month observation period (P = .044, Breslow-Gehan log-rank test). Our findings suggest that PHTN in patients with SCD shortened their survival. © 2003 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. E. Ashley-Koch, L. Elliott, M. E. Kail, L. M. De Castro, J. Jonassaint, T. L. Jackson, J. Price, K. I. Ataga, M. C. Levesque, J. B. Weinberg, et al. Identification of genetic polymorphisms associated with risk for pulmonary hypertension in sickle cell disease Blood, June 15, 2008; 111(12): 5721 - 5726. [Abstract] [Full Text] [PDF] L. De Franceschi, O. S. 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