| Large and medium-sized pulmonary artery obstruction does not play a role of primary importance in the etiology of sickle-cell disease-associated pulmonary hypertension. | |
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MedLine Citation:
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PMID: 18198257 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Pulmonary hypertension (PHT) occurs in approximately 30% of adult patients with sickle-cell disease (SCD) and is a risk factor for early death. The potential role of pulmonary artery obstruction, whether due to emboli or in situ thrombosis, in the etiology of SCD-related PHT is unknown. METHODS: Consecutive SCD patients were screened for PHT (defined as a tricuspid regurgitant jet flow velocity > or = 2.5 m/s) employing echocardiography and were evaluated for pulmonary artery obstruction with ventilation-perfusion (VQ) scintigraphy. RESULTS: Fifty-three HbSS, 6 HbSbeta(0)-thalassemia, 20 HbSC, and 6 HbSbeta(+)-thalassemia patients were included. The overall prevalence of PHT was 41% in HbSS/HbSbeta(0)-thalassemia patients and 13% in HbSC/HbSbeta(+)-thalassemia patients. High-probability VQ defects (Prospective Investigation of Pulmonary Embolism Diagnosis criteria) were detected in two patients, one of whom had PHT. In HbSS/HbSbeta(0)-thalassemia patients with PHT, 19 patients (86%), 2 patients (9%), and 1 patient (5%) had low-, intermediate-, or high-probability scan results as compared to 30 patients (97%), 1 patient (3%), and 0 patients (0%) in HbSS/HbSbeta(0)-thalassemia patients without PHT (p = 0.31). In HbSC/HbSbeta(+)-thalassemia patients with PHT, 3 patients (100%), 0 patients (0%), and 0 patients (0%) had low-, intermediate-, and a high-probability scan as compared to 19 patients (90%), 1 patient (5%), and 1 patient (5%) in HbSC/HbSbeta(+)-thalassemia patients without PHT (p = 0.86). There were no statistical differences in irregular distribution of the radiopharmaceutical or nonspecific signs associated with PHT between patients with and without PHT. CONCLUSIONS: Although small pulmonary artery obstruction cannot be excluded, large to medium-sized pulmonary artery obstruction is an unlikely primary causative factor in SCD-related PHT. |
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Authors:
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Eduard J van Beers; Berthe L F van Eck-Smit; Melvin R Mac Gillavry; Charlotte F J van Tuijn; Joost W J van Esser; Dees P M Brandjes; Mies C Kappers-Klunne; Ashley J Duits; Bart J Biemond; John-John B Schnog; |
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Publication Detail:
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Type: Comparative Study; Journal Article; Multicenter Study Date: 2008-01-15 |
Journal Detail:
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Title: Chest Volume: 133 ISSN: 0012-3692 ISO Abbreviation: Chest Publication Date: 2008 Mar |
Date Detail:
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Created Date: 2008-03-06 Completed Date: 2008-04-22 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0231335 Medline TA: Chest Country: United States |
Other Details:
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Languages: eng Pagination: 646-52 Citation Subset: AIM; IM |
Affiliation:
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Department of Hematology, Academic Medical Center, Amsterdam, the Netherlands. |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Anemia, Sickle Cell / complications*, diagnosis Arterial Occlusive Diseases / complications*, diagnosis, epidemiology Echocardiography Female Humans Hypertension, Pulmonary / diagnosis, epidemiology, etiology* Male Middle Aged Netherlands / epidemiology Prevalence Prognosis Pulmonary Artery* Pulmonary Wedge Pressure / physiology* Severity of Illness Index Survival Rate |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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