Idiopathic pulmonary arterial hypertension

Idiopathic pulmonary arterial hypertension (IPAH) is a rare, frequently fatal disease.

Presenting symptoms and signs are nonspecific and include dyspnea on exertion, fatigue, and a loud pulmonary component of the second heart sound.

Diagnosis of exclusion. Transthoracic Doppler echocardiography and right heart catheterization can diagnose pulmonary hypertension. Laboratory tests and imaging studies can rule out known causes of pulmonary hypertension.

Treatment options include calcium-channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, the soluble guanylate cyclase stimulator riociguat, the selective prostacyclin IP receptor agonist selexipag, and lung transplantation as appropriate.

Prognosis is not good, as most patients eventually develop right ventricular failure. It is best managed in centers with expertise in pulmonary hypertension.

Idiopathic pulmonary arterial hypertension (IPAH) is a disease of the small pulmonary arteries characterized by vascular proliferation and remodeling. It results in a progressive increase in pulmonary vascular resistance (PVR) and, ultimately, right ventricular failure and death.[1]Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30;351(14):1425-36. http://www.ncbi.nlm.nih.gov/pubmed/15459304?tool=bestpractice.com It is defined by a mean pulmonary arterial pressure ≥20 mmHg at rest with pulmonary arterial wedge pressure ≤15 mmHg and PVR >2 Wood units, without a known cause.[2]Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801913. https://erj.ersjournals.com/content/53/1/1801913 http://www.ncbi.nlm.nih.gov/pubmed/30545968?tool=bestpractice.com ​​