Pulmonary Hypertension in SS, SC and Sβ Thalassemia: Prevalence, Associated Clinical Syndromes, and Mortality

• Published in: Blood 2004-11, Vol.104 (11), p.1663-1663
• Main Authors: De Castro, Laura M., Jonassaint, Jude C., Graham, Felicia L., Ashley-koch, Allison, Telen, Marilyn J.
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V104.11.1663.1663

The natural history and mechanisms associated with pulmonary hypertension (pHTN) in sickle cell disease (SCD) are incompletely characterized. We investigated the prevalence of pHTN, diagnosed by echocardiography and/or cardiac catheterization, in adults with all types of SCD, to determine whether the frequency of pHTN varied by Hgb diagnosis. We also analyzed which clinical conditions and laboratory findings were associated with pHTN. We screened 125 outpatients with Hgb SS, SC, Sβ0 or Sβ+ thalassemia who presented with symptoms including either shortness of breath, fatigue, or low or decreasing O2 saturation. PHTN was defined by tricuspid regurgitation jet velocity (TRjet) of ≥ 2.5 m/s by echo and was present in 36% (28/77) of SS & Sβ0 and in 25% (12/48) of SC & Sβ+ patients studied. Of patients with pHTN, 16 (57%) of the SS & Sβ0 patients had a peak TR jet >3.0 m/sec and 12 (43%) ≥2.5-