Pulmonary arterial hypertension (PAH) is a progressive, debilitating, and potentially fatal disease. Symptoms of PAH are non-specific and may include dyspnea, fatigue, chest discomfort, pre-syncope/syncope, palpitations, lower extremity swelling, non-specific gastro-intestinal symptoms, weight loss, and cyanosis. Pulmonologists are increasingly involved in the care of these patients, whether at the initial presentation and diagnosis, medication titration, or for symptom relief and management of complications. The pulmonologist and intensivist also frequently care for patients that are at an increased risk of developing PAH, such as patients with cirrhosis, hypoxic lung disease, scleroderma, sickle cell disease, and HIV to name a few. An echocardiogram, although often inaccurate in estimating the right-sided heart pressures, is the best screening tool for PAH. A right heart catheterization is the gold standard for diagnosing PAH. Supplemental therapies may include oxygen, diuretics, and anticoagulation. In addition to the limited role of calciumchannel blockers, PAH-specific therapies fall into one of 3 families for now: Phospho-diesterase-5 inhibitors, endothelin receptor antagonists, and prostacyclins. The current estimated 2 year survival of PAH patients now exceeds 90%, compared to a historical median survival of 2-3 years.
Keywords: Pulmonary hypertension, diagnosis, management, Dana Point, ERA, PDE5 inhibitor, prostanoid, Clinical Presentation, Symposium, fatal disease, pulmonary artery, anti-inflammatory molecules, hepatitis C