The management of primary pulmonary hypertension
H. I. Palevsky and A. P. Fishman
Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia.
Primary pulmonary hypertension is a clinical syndrome characterized by
pulmonary hypertension in the absence of sufficient underlying cardiac,
parenchymal pulmonary, or systemic disease to account for it. The
population of patients with primary pulmonary hypertension is a
heterogeneous one, both clinically and histologically. As the etiologic
mechanisms are unknown, therapy is directed toward the consequences of the
pulmonary vascular process. Oxygen supplementation, the use of digoxin and
diuretics for symptomatic heart failure, and anticoagulation all may have a
role in treating primary pulmonary hypertension, although vasodilator
therapy has been the main area of investigation. Screening for vasodilator
responsiveness, defining a favorable vasodilator effect, predicting
long-term effectiveness, and deciding who to treat have all been
controversial. New approaches, such as use of high-dose calcium
channel-blocking agents and continuous intravenous infusion of prostacyclin
(an investigational agent), have recently been proposed. When medical
therapies are exhausted, heart-lung or lung transplantation has
increasingly become an option for selected patients.
