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guides & guidelines

Pulmonary hypertension in sickle cell disease

key information

source: Annals of the American Thoracic Society

year: 2014

authors: Hayes MM, Vedamurthy A, George G, Dweik R, Klings ES, Machado RF, Gladwin MT, Wilson KC, Thomson CC


Pulmonary hypertension (PH) is common in sickle cell disease and is known to be a risk factor for death. To improve the care of patients with sickle cell disease, the American Thoracic Society has developed a clinical practice guideline on the treatment of PH in sickle cell disease for pulmonologists, hematologists, cardiologists, pediatricians, and internists. The guideline was created by assembling a panel of experts, reviewing the literature, synthesizing the evidence, and then generating recommendations. This summary for practicing clinicians is prepared from the official guideline document (1).

PH is defined as a resting mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg. It occurs in 6 to 11% of patients with sickle cell disease and is classified in World Health Organization (WHO) PH group 5 because patients have variable hemodynamics (i.e., they may have precapillary, postcapillary, or both pre and postcapillary PH).

Precapillary PH is defined as a mPAP of ≥25 mm Hg with a mean pulmonary artery wedge pressure (PAWP) or left ventricular end diastolic pressure (LVEDP) of ≤15 mm Hg plus increased pulmonary vascular resistance (PVR). Patients with sickle cell disease may have a lower baseline PVR due to anemia; thus, increased PVR is defined as ≥160 dyn⋅s⋅cm−5 or ≥2 Wood units.

Postcapillary PH is defined as a PAWP or LVEDP of ≥15 mm Hg (in the absence of mitral valve disease) without an increase in PVR.

Pre- and postcapillary PH is defined as a mPAP of ≥25 mm Hg, a PAWP of ≥15 mm Hg, and an increased PVR. These patients typically also have an elevated transpulmonary gradient (>12 mm Hg) (1).

organization: Johns Hopkins University School of Medicine, Baltimore

DOI: 10.1513/AnnalsATS.201408-405CME

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