Pathophysiology and treatment of pulmonary hypertension in sickle cell disease.

  title={Pathophysiology and treatment of pulmonary hypertension in sickle cell disease.},
  author={Victor R Gordeuk and Oswaldo L. Castro and Roberto F. Machado},
  volume={127 7},
Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric… CONTINUE READING
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Plexiform lesions in pulmonary arterial hypertension composition, architecture, and 826 GORDEUK et al BLOOD, 18 FEBRUARY 2016 x VOLUME 127, NUMBER 7 For personal use

  • D Jonigk, H Golpon, CL Bockmeyer
  • only. on September
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