Cor Vasa 2002, 43(12):519-523
New concepts regarding the treatment of primary pulmonary hypertension
- Klinika kardiologie, Institut klinické a experimentální medicíny, Praha, Česká republika
The aim of this communication is to inform readers about the aspects of primary pulmonary hypertension (PPH). It addresses the incidence, vascular pathology, and current as well as state-of-the-art therapy of this serious progressive disease. Primary pulmonary hypertension is defined as an increase in pulmonary artery pressure in the absence of other known causes. Its incidence in the European and U. S. adult populations is estimated at 1-2 cases per 1 mil. pop. Standard drug therapy is based on calcium-channel blockers. However, no more than 24% of patients will benefit from this therapy. Anticoagulation therapy is recommended because of an increased risk for thrombosis in the vascular bed whilst diuretic therapy is indicated in the presence of signs on systemic circulation congestion. Surgical methods include lung transplantation or, alternatively, simultaneous heart and lung transplantation. Balloon atrial septostomy has but a short-term effect and is regarded as a bridge to lung transplantation. However, the procedure is associated with relatively high mortality rates. There is no doubt the potential of the current therapeutic options is limited.
Recently, there have been reports of new therapeutic procedures based on concepts regarding the pathogenesis of primary pulmonary hypertension. Beneficial effects are exerted by prostacyclin, with experience related to i. v. prostacyclin dating back to 1984. Considering the potential of continuous administration, associated with a variety of side effects, preference is given to stable prostacyclin analogs such as treprostinol (subcutaneous), beraprost (oral), or iloprost (inhaled). Particularly promising is inhaled therapy with the drug acting selectively on the pulmonary vascular bed.
Mention should also be made of endothelin receptor blockers, with bosentan being the best known one and showing a very good therapeutic effect. Also promising are treatments with sildenafil and L-arginine; however, large clinical trials with these agents have not been reported to date.
The development of PPH therapy seems to be heading for combination therapy, which is to be strictly individualized by the patient's status and outcome of acute hemodynamic assessment.
Keywords: Primary pulmonary hypertension (PPH); Pathophysiology of PPH; Current and new therapeutic procedures in the treatment of PPH
Published: November 1, 2002 Show citation