Primary pulmonary hypertension in adults.
Primary pulmonary hypertension is an enigmatic disease found predominantly in young women, but it also affects a significant number of middle-aged and elderly males and females. Its onset, characterized by progressively worsening dyspnea, fatigue, and chest pain, is insidious. Three distinct histopathologic subtypes have been identified, and the natural history of the disease process has been well-defined. Pharmacologic treatment options have, in general, been disappointing, and it appears that heart-lung transplantation will be applied only to a small minority of young patients with primary pulmonary hypertension in the near future. We review the histopathology, evaluation, treatment, and prognosis of primary pulmonary hypertension.
Hawkins
J W
JW
Section of Cardiovascular Diseases, University of Kansas Medical Center, Kansas City 66103.
Dunn
M I
MI
D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D001706:Biopsy; D006328:Cardiac Catheterization; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D014665:Vasodilator Agents / Q000627:therapeutic use
D000925:Anticoagulants; D014665:Vasodilator Agents