Vasodilator-Responsive Idiopathic Pulmonary Arterial Hypertension: Evidence for a New Disease?
Authors: Evan L. Brittain, Anna R. Hemnes
Journal: Annals of Internal Medicine (2015)

Editorials20 January 2015Vasodilator-Responsive Idiopathic Pulmonary Arterial Hypertension: Evidence for a New Disease?Evan L. Brittain, MD, MSCI and Anna R. Hemnes, MDEvan L. Brittain, MD, MSCIFrom Vanderbilt University Medical Center, Nashville, Tennessee. and Anna R. Hemnes, MDFrom Vanderbilt University Medical Center, Nashville, Tennessee.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/M14-2700 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Idiopathic pulmonary arterial hypertension (IPAH) is a highly morbid disease characterized by progressive obliteration of precapillary arterioles and a contemporary survival time after diagnosis of slightly more than 5 years (1). A small subset (5% to 10%) of patients with IPAH displays dramatic hemodynamic improvement in response to acute exposure to pulmonary vasodilators, and approximately one half of these "vasodilator-responsive" patients improve clinically with calcium-channel blocker therapy (2). The disparity in outcomes has fueled speculation for decades that vasodilator-responsive and nonresponsive IPAH are distinct diseases.Idiopathic pulmonary arterial hypertension is believed to arise from pulmonary vascular remodeling, with vasoconstriction providing ...References1. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448-56. [PMID: 22281797] CrossrefMedlineGoogle Scholar2. Sitbon O, Humbert M, Jaäs X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111:3105-11. [PMID: 15939821] CrossrefMedlineGoogle Scholar3. Palevsky HI, Schloo BL, Pietra GG, Weber KT, Janicki JS, Rubin E, et al. Primary pulmonary hypertension. Vascular structure, morphometry, and responsiveness to vasodilator agents. Circulation. 1989;80:1207-21. [PMID: 2805259] CrossrefMedlineGoogle Scholar4. Langleben D, Orfanos SE, Giovinazzo M, Schlesinger RD, Hirsch AM, Blenkhorn F, et al. Acute vasodilator responsiveness and microvascular recruitment in idiopathic pulmonary arterial hypertension. Ann Intern Med. 2015;162:154-6. doi:10.7326/M14-1402 LinkGoogle Scholar5. Orfanos SE, Armaganidis A, Glynos C, Psevdi E, Kaltsas P, Sarafidou P, et al. Pulmonary capillary endothelium-bound angiotensin-converting enzyme activity in acute lung injury. Circulation. 2000;102:2011-8. [PMID: 11034953] CrossrefMedlineGoogle Scholar6. Orfanos SE, Langleben D, Khoury J, Schlesinger RD, Dragatakis L, Roussos C, et al. Pulmonary capillary endothelium-bound angiotensin-converting enzyme activity in humans. Circulation. 1999;99:1593-9. [PMID: 10096936] CrossrefMedlineGoogle Scholar7. Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation. 1987;76:135-41. [PMID: 2954725] CrossrefMedlineGoogle Scholar8. Hemnes AR, Trammell AW, Archer SL, Rich S, Yu C, Nian H, et al. A peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation. 2014. [PMID: 25361553] MedlineGoogle Scholar9. Brittain EL, Pugh ME, Wheeler LA, Robbins IM, Loyd JE, Newman JH, et al. Prostanoids but not oral therapies improve right ventricular function in pulmonary arterial hypertension. JACC Heart Fail. 2013;1:300-7. [PMID: 24015376] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Evan L. Brittain, MD, MSCI; Anna R. Hemnes, MDAffiliations: From Vanderbilt University Medical Center, Nashville, Tennessee.Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M14-2700.Corresponding Author: Anna R. Hemnes, MD, Division of Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, 2213 Garland Avenue, Medical Research Building IV, Room P475, Nashville, TN 37232; e-mail,anna.r.[email protected]edu.Current Author Addresses:Dr. Brittain: Division of Cardiovascular Medicine, Vanderbilt University Medical Center, 2525 West End Avenue, Suite 300, Nashville, TN 37203.Dr. Hemnes: Division of Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, 2213 Garland Avenue, Medical Research Building IV, Room P475, Nashville, TN 37232. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoAcute Vasodilator Responsiveness and Microvascular Recruitment in Idiopathic Pulmonary Arterial Hypertension David Langleben , Stylianos E. Orfanos , Michele Giovinazzo , Robert D. Schlesinger , Andrew M. Hirsch , Fay Blenkhorn , Lyda Lesenko , Apostolos Armaganidis , and John D. Catravas Acute Vasodilator Responsiveness and Microvascular Recruitment in Idiopathic Pulmonary Arterial Hypertension David Langleben , Stylianos E. Orfanos , Michele Giovinazzo , Robert D. Schlesinger , Andrew M. Hirsch , Fay Blenkhorn , Lyda Lesenko , Apostolos Armaganidis , and John D. Catravas Metrics Cited byThe Pathobiology of Pulmonary Arterial HypertensionPeakP ET CO 2 combined with FEV1/FVC predicts vasodilator‐responsive patients with idiopathic pulmonary arterial hypertensionMacrophage Immunomodulation: The Gatekeeper for Mesenchymal Stem Cell Derived-Exosomes in Pulmonary Arterial Hypertension?What's in a side effect? The association between pulmonary vasodilator adverse drug events and clinical outcomes in patients with pulmonary arterial hypertensionVasodilator responsiveness in idiopathic pulmonary arterial hypertension: identifying a distinct phenotype with distinct physiology and distinct prognosisEffect of calcium channel blockers evaluated by cardiopulmonary exercise testing in idiopathic pulmonary arterial hypertension responding to acute pulmonary vasoreactivity testing 20 January 2015Volume 162, Issue 2Page: 148-149KeywordsCapillariesCardiac outputHealth services researchHemodynamicsPhenotypesPulmonary diseasesPulmonary hypertensionPulmonary vascular resistanceVasoconstrictionVasodilators ePublished: 20 January 2015 Issue Published: 20 January 2015 Copyright & PermissionsCopyright © 2015 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...