Stephen J Halliday1, Anna R Hemnes2, Ivan M Robbins2, Meredith E Pugh2, David X Zhao3, Robert N Piana4, Pete P Fong4, Evan L Brittain5. 1. Department of Internal Medicine. 2. Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee. 3. Section of Cardiology, Wake Forest Baptist Medical Center, Winston-Salem, North Carolina. 4. Division of Cardiovascular Medicine Vanderbilt University Medical Center, Nashville, Tennessee. 5. Division of Cardiovascular Medicine Vanderbilt University Medical Center, Nashville, Tennessee. Electronic address: evan.brittain@vanderbilt.edu.
Abstract
BACKGROUND: A "classic" response to acute vasodilator testing (drop of >10 mm Hg in mean pulmonary artery pressure [mPAP] to <40 mm Hg) confers an excellent prognosis in patients with idiopathic pulmonary arterial hypertension (IPAH) and identifies candidates for treatment with calcium channel blockers (CCB). Little is known about vasodilator responsiveness (VR) in other types of PAH, or about outcomes in patients with a significant but "non-classic" decrease in mPAP. We hypothesized that VR occurs in non-idiopathic PAH and non-classic VR portends a better prognosis than no VR in PAH. METHODS: Acute VR testing with nitric oxide was performed on 155 consecutive patients referred for PH evaluation. Non-classic response was defined as decrease in mPAP >10 mm Hg to >40 mm Hg with preserved cardiac output. Demographics and functional status were assessed at baseline and the first clinic visit after VR testing, and survival was followed over time. RESULTS: Twenty patients (13%) displayed classic VR. Among classic responders, 12 (60%) had IPAH and 8 (40%) had connective tissue disease-associated PAH (CTD-PAH); however, only responders with IPAH had improved survival compared with non-responders (p = 0.02). Thirteen patients (8%) had a non-classic VR. Non-classic response was not associated with improved survival compared with non-responders (p = 0.86). Acute change in mPAP or pulmonary vascular resistance in the entire cohort did not predict survival. CONCLUSIONS: Classic acute VR occurs in CTD-PAH as well as IPAH; however, only IPAH patients have improved outcomes. A significant but non-classic VR is not associated with improved survival.
BACKGROUND: A "classic" response to acute vasodilator testing (drop of >10 mm Hg in mean pulmonary artery pressure [mPAP] to <40 mm Hg) confers an excellent prognosis in patients with idiopathic pulmonary arterial hypertension (IPAH) and identifies candidates for treatment with calcium channel blockers (CCB). Little is known about vasodilator responsiveness (VR) in other types of PAH, or about outcomes in patients with a significant but "non-classic" decrease in mPAP. We hypothesized that VR occurs in non-idiopathic PAH and non-classic VR portends a better prognosis than no VR in PAH. METHODS: Acute VR testing with nitric oxide was performed on 155 consecutive patients referred for PH evaluation. Non-classic response was defined as decrease in mPAP >10 mm Hg to >40 mm Hg with preserved cardiac output. Demographics and functional status were assessed at baseline and the first clinic visit after VR testing, and survival was followed over time. RESULTS: Twenty patients (13%) displayed classic VR. Among classic responders, 12 (60%) had IPAH and 8 (40%) had connective tissue disease-associated PAH (CTD-PAH); however, only responders with IPAH had improved survival compared with non-responders (p = 0.02). Thirteen patients (8%) had a non-classic VR. Non-classic response was not associated with improved survival compared with non-responders (p = 0.86). Acute change in mPAP or pulmonary vascular resistance in the entire cohort did not predict survival. CONCLUSIONS: Classic acute VR occurs in CTD-PAH as well as IPAH; however, only IPAH patients have improved outcomes. A significant but non-classic VR is not associated with improved survival.
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