Primary pulmonary hypertension.

摘要: Primary pulmonary hypertension (PPH) is a rare disease of unknown aetiology which typically results in right heart failure and death within several years the onset symptoms. While there no cure for PPH, pharmacological surgical approaches to treatment have been developed over past decade proved useful significant proportion patients. In particular, vasodilator therapy may produce sustained haemodynamic symptomatic improvement up approximately two-thirds patients; remaining patients, vasodilators either benefit or result deterioration. The calcium channel blocking agents are most widely used oral vasodilators; continuous intravenous infusions epoprostenol (prostacyclin; prostaglandin I2) some patients who refractory therapy, particularly as bridge transplantation. combined heart-lung transplantation has considered procedure choice severe hypertension, single lung performed successfully small number be preferred approach with reasonably preserved function.