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This Article Full Text (PDF) All Versions of this Article: chest.07-1694v1 133/3/646    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by van Beers, E.J. Articles by Schnog, J.B. Search for Related Content PubMed PubMed Citation Articles by van Beers, E.J. Articles by Schnog, J.B.

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

¹Department of Hematology, Academic Medical Center, Amsterdam, The Netherlands ²Department of Nuclear Medicine, Academic Medical Center, Amsterdam, The Netherlands ³Department of Cardiology, Slotervaart Hospital, Amsterdam, The Netherlands ⁴Department of Hematology, Erasmus Medical Center, Rotterdam, The Netherlands ⁵Department of Internal Medicine, Slotervaart Hospital, Amsterdam, The Netherlands ⁶The Red Cross Blood Bank Foundation, Curaçao, Netherlands Antilles

Abstract

Background: Pulmonary hypertension (PHT) occurs in approximately 30% of adult sickle cell patients 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 sickle cell disease (SCD) related PHT is unknown.

Methods: Consecutive sickle cell patients were screened for PHT (defined as a tricuspid regurgitant jet flow velocity 2.5m/s) employing echocardiography and were evaluated for pulmonary artery obstruction with ventilation-perfusion (VQ) scintigraphy.

Results: Fifty-three HbSS, 6 HbSβ⁰-thalassemia, 20 HbSC and 6 HbSβ⁺-thalassemia patients were included. The overall prevalence of PHT was 41% in HbSS/HbSβ⁰-thal patients and 13% in HbSC/HbSβ⁺-thal patients. High probability VQ-defects (PIOPED criteria) were detected in 2 patients, one of whom had PHT. In HbSS/HbSβ⁰-thal patients with PHT, 19 (86%), 2 (9%) and 1 (5%) had low, intermediate or high-probability scans as compared to 30 (97%), 1 (3%) and 0(0%) in HbSS/HbSβ⁰-thal patients without PHT (p=0.31). In HbSC/HbSβ⁺-thal patients with PHT, 3(100%), 0(0%) and 0(0%) had low, intermediate and a high-probability scans as compared to 19(90%), 1(5%), and 1 (5%) in HbSC/HbSβ⁺-thal patients without PHT (p=0.86). There were no statistical differences in irregular distribution of the radiopharmaceutical or non-specific 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.