|HIV||0.5% estimate (74,75)|
|Sickle cell disease|
|Thyroid diseases||PH can be detected in up to 41% of patients with hyperthyroidism (82) and 49% of those with hypothyroidism (83), and usually responds to treatment of the thyroid disease (84,85).|
|Myeloproliferative disorders||Literature does not provide meaningful data (89,90).|
In CTD patients who are newly screened with echocardiography, the degree of PAH, if present, detected at RHC is usually mild, and the future course is unknown. Based on estimated prevalence of scleroderma 210 to 276/million (68) and U.S. population of 302 million, the number of scleroderma cases is 250 × 302 = 75,500, so approximately 7,550 (10%) people would be expected to have PAH by right heart catheterization criteria. In African Americans, 0.15% have sickle cell disease (homozygotes) and 8% have sickle cell trait (heterozygotes).
APAH = associated pulmonary hypertension; CTD = connective tissue disease; CTEPH = chronic thromboembolic pulmonary hypertension; FPAH = familial pulmonary arterial hypertension; HIV = human immunodeficiency virus; ILD = interstitial lung disease; IPAH = idiopathic pulmonary arterial hypertension; MCTD = mixed connective tissue disease; mPAP = mean pulmonary arterial pressure; OLT = orthotopic liver transplantation; PAH = pulmonary arterial hypertension; PAWP = pulmonary capillary wedge pressure; PE = pulmonary embolism; PH = pulmonary hypertension; RHC = right heart catheterization; RV = right ventricular; RVSP = right ventricular systolic pressure; TRV = tricuspid regurgitation velocity; VTR = peak velocity of tricuspid regurgitation.