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For the purposes of diagnosing VR\u2010PAH, there is to date no test to replace the acute vasodilator challenge. However, recent studies have identified markers that may aid in the identification of VR\u2010PAH, including peripheral blood lymphocyte RNA expression levels of desmogelin\u20102 and Ras homolog gene family member Q, and plasma levels of provirus integration site for Moloney murine leukemia virus. Genome wide\u2010array studies of peripheral blood DNA have demonstrated differences in disease specific genetic variants between VR\u2010PAH and NR\u2010PAH, with particular convergence on cytoskeletal function pathways and Wnt signaling pathways. These studies offer hope for future non\u2010invasive identification of VR\u2010PAH, and insights into pathogenesis that may lead to novel therapies. Examination of the degree of pulmonary microvascular perfusion in PAH has offered additional insights. During the acute vasodilator challenge, VR\u2010PAH patients demonstrate true vasodilation with recruitment and increased perfusion of the capillary bed, while VN\u2010PAH patients are unable to recruit vasculature. In the very few reports of lung histology, VR\u2010PAH has more medial thickening in the precapillary arterioles, while VN\u2010PAH has the classic histology of PAH, including intimal thickening. VR\u2010PAH is a disorder with a phenotype distinct from VN\u2010PAH and other types of PAH, and should be considered separately in the classification of PAH.<\/jats:p>","DOI":"10.1177\/2045893217714231","type":"journal-article","created":{"date-parts":[[2017,5,23]],"date-time":"2017-05-23T15:28:58Z","timestamp":1495553338000},"page":"588-597","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":12,"title":["Vasodilator responsiveness in idiopathic pulmonary arterial hypertension: identifying a distinct phenotype with distinct physiology and distinct prognosis"],"prefix":"10.1002","volume":"7","author":[{"given":"David","family":"Langleben","sequence":"first","affiliation":[{"name":"Center for Pulmonary Vascular Disease Division of Cardiology Jewish General Hospital McGill University Montreal Quebec 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