Authors: Marjorie Barrier Jolyane Meloche Maria Helena Jacob Audrey Courboulin Steeve Provencher Sébastien Bonnet
Publish Date: 2012/03/25
Volume: 69, Issue: 17, Pages: 2805-2831
Abstract
The pathobiology of pulmonary arterial hypertension PAH involves a remodeling process in distal pulmonary arteries as well as vasoconstriction and in situ thrombosis leading to an increase in pulmonary vascular resistance right heart failure and death Its etiology may be idiopathic but PAH is also frequently associated with underlying conditions such as connective tissue diseases During the past decade more than welcome novel therapies have been developed and are in development including those increasingly targeting the remodeling process These therapeutic options modestly increase the patients’ longterm survival now approaching 60 at 5 years However noninvasive tools for confirming PAH diagnosis and assessing disease severity and response to therapy are tragically lacking and would help to select the best treatment After exclusion of other causes of pulmonary hypertension a final diagnosis still relies on right heart catheterization an invasive technique which cannot be repeated as often as an optimal followup might require Similarly other techniques and biomarkers used for assessing disease severity and response to treatment generally lack specificity and have significant limitations In this review imaging as well as current and future circulating biomarkers for diagnosis prognosis and followup are discussed
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