title: Today's and tomorrow's imaging and circulating biomarkers for pulmonary arterial hypertension.
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' long-term survival, now approaching 60% at 5 years. However, non-invasive 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 follow-up 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 follow-up are discussed.
authors.affiliation: Pulmonary Hypertension Research Group, Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, 2725 Chemin Ste-Foy, Québec, QC, G1V 4G5, Canada.
authors.forename: Marjorie
authors.initials: M
authors.lastname: Barrier
authors.forename: Jolyane
authors.initials: J
authors.lastname: Meloche
authors.forename: Maria Helena
authors.initials: MH
authors.lastname: Jacob
authors.forename: Audrey
authors.initials: A
authors.lastname: Courboulin
authors.forename: Steeve
authors.initials: S
authors.lastname: Provencher
authors.forename: Sébastien
authors.initials: S
authors.lastname: Bonnet
mesh_terms: D015415:Biomarkers / Q000097:blood*; D003952:Diagnostic Imaging; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000175:diagnosis*
chemical_list: D015415:Biomarkers