An overview of pulmonary arterial hypertension: risks, pathogenesis, clinical manifestations, and management

This article focuses on pulmonary arterial hypertension, including both primary pulmonary hypertension (PPH) and those forms of pulmonary arterial hypertension that are related to other factors, including collagen vascular diseases, congenital shunts, portal hypertension, human immunodeficiency viral infection, and exposure to specific drugs and toxins. Risks for different types of pulmonary arterial hypertension are identified. The common pathogenesis for pulmonary arterial hypertension is discussed, and includes an overview of the role of key vasoactive substances such as nitric oxide, prostacyclin, endothelin, and thromboxane. Typical presenting clinical manifestations, recommendations for screening of patients at risk, and key diagnostic findings are discussed. The mainstay of treatment is identified as pharmacologic, and may include diuretics, digoxin, warfarin, calcium channel antagonists, and prostacyclin analogues such as epoprostenol. Surgical interventions are considered as a last resort, and may include unilateral or bilateral lung transplant or atrial septostomy. Treatment options for patients with pulmonary arterial hypertension hold more hope today than they did a decade ago and are identified so as to guide the advanced practice nurse in recognizing and then facilitating the appropriate management of patients with this rare but disabling disease.