title: Disproportionate pulmonary hypertension in a patient with early-onset pulmonary emphysema treated with specific drugs for pulmonary arterial hypertension.
abstract: Severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is referred to as 'disproportionate' because the elevated pulmonary artery pressure does not match the degree of air flow limitation. We report a 41-year-old man presenting with early-onset pulmonary emphysema and pulmonary hypertension with a mean pressure of 74 mmHg. Continuous intravenous epoprostenol led to marked hemodynamic improvement, and epoprostenol was successfully replaced with bosentan. The patient has been followed for 3 years without exacerbation. This is the first report demonstrating the long-term efficacy of specific drugs for pulmonary arterial hypertension in disproportionate pulmonary hypertension in COPD.
authors: Department of Cardiology, National Hospital Organization Kobe Medical Center, Japan. cheetahms@hera.eonet.ne.jp
authors: Masatoshi
authors: M
authors: Shimizu
authors: Junichi
authors: J
authors: Imanishi
authors: Takatsugu
authors: T
authors: Takano
authors: Yoichi
authors: Y
authors: Miwa
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004359:Drug Therapy, Combination; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D011656:Pulmonary Emphysema / Q000150:complications*; D013449:Sulfonamides / Q000627:therapeutic use*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; C048081:beraprost; D011464:Epoprostenol; D000077300:Bosentan