Pathophysiology and treatment of pulmonary arterial hypertension

Pulmonary arterial hypertension is a rare, complex and multifactorial disease in which pulmonary vascular remodelling plays a major role leading to right-heart failure and ultimately death. Pulmonary arterial hypertension therapies mainly target the vasoconstric-tion/vasodilatation balance and are not curative. Lung transplantation should be considered in patients refractory to these therapies. Novel therapies targeting the molecular basis of pul-monary vascular remodelling are in development. Regularly updated international guidelines allow best standard of care of these patients. (c) 2022 l'Academie nationale de medecine. Published by Elsevier Masson SAS. All rights reserved.