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

Chest
Eduard J van BeersCURAMA Study Group

Abstract

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. 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. 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 patient...Continue Reading

References

Jan 1, 1979·American Journal of Hematology·B K WalkerE R Burka
Mar 1, 1987·Journal of the American College of Cardiology·K L ChanA J Tajik
Jun 9, 1994·The New England Journal of Medicine·O S PlattP P Klug
May 29, 1998·American Journal of Respiratory and Critical Care Medicine·G L YungK M Moser
Nov 8, 2001·Archives of Pathology & Laboratory Medicine·M O AdedejiM D Hughson
Jan 5, 2002·Blood·Amiram Eldor, Eliezer A Rachmilewitz
Oct 9, 2003·British Journal of Haematology·Elizabeth A ManciUNKNOWN Investigators of the Cooperative Study of Sickle Cell Disease
Feb 27, 2004·The New England Journal of Medicine·Elliott P Vichinsky
Feb 27, 2004·The New England Journal of Medicine·Mark T GladwinFrederick P Ognibene
Jun 11, 2004·The New England Journal of Medicine·Elizabeth S Klings, Harrison W Farber
Oct 27, 2004·The American Journal of Medicine·Kenneth I AtagaEugene P Orringer
Oct 11, 2005·Hematology/oncology Clinics of North America·Oswaldo Castro, Mark T Gladwin
Jun 29, 2006·British Journal of Haematology·Kenneth I AtagaEugene P Orringer
Sep 27, 2006·The American Journal of Medicine·Paul D SteinRonald E Olson
Mar 24, 2007·American Journal of Respiratory and Critical Care Medicine·Anastasia AnthiMark T Gladwin
May 4, 2007·Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine·Nina TunariuAdil Al-Nahhas
Sep 15, 2007·British Journal of Haematology·Kenneth I AtagaEliezer A Rachmilewitz

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Citations

Dec 6, 2011·Revue de pneumologie clinique·G QuéréL De Saint-Martin
May 7, 2014·American Journal of Hematology·Eduard J van BeersPaul Bresser
Nov 21, 2013·Respirology : Official Journal of the Asian Pacific Society of Respirology·Mart N van der PlasPaul Bresser
Apr 16, 2013·The American Journal of Medicine·Rakhi P NaikSophie Lanzkron
Jun 9, 2018·Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine·Alem MehariNargues A Weir
Mar 21, 2009·Current Opinion in Endocrinology, Diabetes, and Obesity

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