What is the significance of pulmonary hypertension in patients with sickle cell disease (SCD), and what factors contribute to pulmonary hypertension?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Pulmonary hypertension, defined as a tricuspid regurgitant jet velocity (TRJV) greater than 2.5 m/s on echocardiography, is an emergent complication seen in 32% of adult patients with SCD and is associated with a high mortality rate. Pulmonary hypertension is a complication of chronic intravascular hemolysis. Additional factors contributing to pulmonary hypertension include older age, renal insufficiency, cardiovascular disease, cholestatic hepatopathy, systolic hypertension, high hemolytic markers, iron overload, and a history of priapism.


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