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Acute vasoreactivity testing (AVT) is seldom performed routinely in CTEPH patients, so there is limited understanding of the frequency and significance of an acute vasodilator response. Systematic vasodilator testing with oxygen (O<jats:sub>2<\/jats:sub>) and oxygen plus inhaled nitric oxide (O<jats:sub>2<\/jats:sub>\u2009+\u2009iNO) was performed as part of the Pulmonary Vascular Disease Omics (PVDOMICS) NHLBI project, providing an opportunity to examine AVT responses in CTEPH. Patients with CTEPH enrolled in PVDOMICS (<jats:italic>n<\/jats:italic>\u2009=\u200949, 40 with prevalent CTEPH\u00a0[82%])\u00a0underwent right heart catheterization including AVT with O<jats:sub>2<\/jats:sub> and O<jats:sub>2<\/jats:sub>\u2009+\u2009iNO. Hemodynamics were obtained at baseline and with each challenge. Fourteen of 49 patients (29%) had &gt;20% drop in pulmonary vascular resistance (PVR) with O<jats:sub>2<\/jats:sub>. With O<jats:sub>2<\/jats:sub>\u2009+\u2009iNO, 30\/49 (61%) had &gt;20% drop in PVR, 20% had &gt;20% drop in mean pulmonary artery pressure\u00a0(mPAP) and PVR, and 8% had &gt;10\u2009mmHg decline in mPAP to mPAP\u2009&lt;\u200940 with normal cardiac output. Patients on riociguat had less response to O<jats:sub>2<\/jats:sub>\u2009+\u2009iNO than patients on phosphodiesterase\u20105\u00a0inhibitors. Our findings shed light on the significant variability in vascular tone that is present in CTEPH, confirming that CTEPH represents a combination of mechanical obstruction and vasoconstriction that appears similar to that observed with Group 1 PAH. Additional study regarding whether results of acute vasodilator testing predict response to therapy and relate to prognosis is warranted.<\/jats:p>","DOI":"10.1002\/pul2.12181","type":"journal-article","created":{"date-parts":[[2022,12,20]],"date-time":"2022-12-20T13:03:06Z","timestamp":1671541386000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Acute vasoreactivity testing during right heart catheterization in chronic thromboembolic pulmonary hypertension: Results from the pulmonary vascular disease phenomics study"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4128-3978","authenticated-orcid":false,"given":"Robert P.","family":"Frantz","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane A.","family":"Leopold","sequence":"additional","affiliation":[{"name":"Harvard Medical School Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Hassoun","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna R.","family":"Hemnes","sequence":"additional","affiliation":[{"name":"Division of Allergy, Pulmonary and Critical Care Medicine Vanderbilt University Medical Center Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn M.","family":"Horn","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen C.","family":"Mathai","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franz P.","family":"Rischard","sequence":"additional","affiliation":[{"name":"Perkin Heart Failure Center, Division of Cardiology Weill Cornell Medicine New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. 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