Acute Vasodilator Responsiveness and Microvascular Recruitment in Idiopathic Pulmonary Arterial Hypertension
Authors: David Langleben, Stylianos E. Orfanos, Michele Giovinazzo, Robert D. Schlesinger, Andrew M. Hirsch, Fay Blenkhorn, Lyda Lesenko, Apostolos Armaganidis, John D. Catravas
Journal: Annals of Internal Medicine (2015)

Letters20 January 2015Acute Vasodilator Responsiveness and Microvascular Recruitment in Idiopathic Pulmonary Arterial HypertensionDavid Langleben, MD, Stylianos E. Orfanos, MD, Michele Giovinazzo, BSc, Robert D. Schlesinger, MD, Andrew M. Hirsch, MD, Fay Blenkhorn, RN, Lyda Lesenko, RN, Apostolos Armaganidis, MD, and John D. Catravas, PhDDavid Langleben, MDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Stylianos E. Orfanos, MDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Michele Giovinazzo, BScFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Robert D. Schlesinger, MDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Andrew M. Hirsch, MDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Fay Blenkhorn, RNFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Lyda Lesenko, RNFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., Apostolos Armaganidis, MDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia., and John D. Catravas, PhDFrom McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/M14-1402 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Idiopathic pulmonary arterial hypertension (IPAH) may be characterized according to the hemodynamic response to an acute vasodilator challenge (1). Criteria for response are an acute reduction in the mean pulmonary arterial pressure (mPAP) greater than 10 mm Hg resulting in an mPAP less than 40 mm Hg without a decrease in pulmonary arterial blood flow (that is, cardiac output) (2). Approximately 90% to 95% of patients do not respond and are treated with approved therapies for IPAH; their long-term prognoses vary. The few who do respond generally improve clinically with long-term, high-dose calcium-channel blocker therapy and have an excellent ...References1. Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42-50. [PMID: 24355641] doi:10.1016/j.jacc.2013.10.032 CrossrefMedlineGoogle Scholar2. Sitbon O, Humbert M, Jaïs 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. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992;327:76-81. [PMID: 1603139] CrossrefMedlineGoogle Scholar4. West JB. Pulmonary embolism. In: West JB, eds. Pulmonary Physiology and Pathophysiology: An Integrated, Case-Based Approach. Philadelphia: Lippincott Williams & Wilkins; 2001:84-99. Google Scholar5. Langleben D, Orfanos SE, Giovinazzo M, Hirsch A, Baron M, Senécal JL, et al. Pulmonary capillary endothelial metabolic dysfunction: severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2008;58:1156-64. [PMID: 18383374] doi:10.1002/art.23405 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. 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 Scholar8. Orfanos SE, Psevdi E, Stratigis N, Langleben D, Catravas JD, Kyriakidis M, et al. Pulmonary capillary endothelial dysfunction in early systemic sclerosis. Arthritis Rheum. 2001;44:902-11. [PMID: 11315930] CrossrefMedlineGoogle Scholar9. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al; American College of Cardiology Foundation Task Force on Expert Consensus Documents. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573-619. [PMID: 19389575] doi:10.1016/j.jacc.2009.01.004 CrossrefMedlineGoogle Scholar10. Orfanos SE, Ehrhart IC, Barman S, Hofman WF, Catravas JD. Endothelial ectoenzyme assays estimate perfused capillary surface area in the dog lung. Microvasc Res. 1997;54:145-55. [PMID: 9327385] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From McGill University, Montréal, Quebec, Canada; Attikon Hospital, Athens, Greece; and Old Dominion University, Norfolk, Virginia.Note: Drs. Langleben and Orfanos contributed equally to this work.Acknowledgment: The authors thank the staff of the Coronary Care Unit and Cardiac Catheterization Laboratory of the Jewish General Hospital for their assistance with the study and their tender care of the patients.Financial Support: By the William and Ida Pencer Family Foundation, the Dimitrios Banousis Foundation, the Bank of Montréal Center for the Study of Heart Disease in Women, and the Jewish General Hospital Annual Walk for Pulmonary Hypertension, all at the Jewish General Hospital.Disclosures: Authors have disclosed no conflicts of interest. Forms can be viewed at www.acponline.org/authors/icmje/ConflictOfInterest Forms.do?msNum=M14-1402. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoVasodilator-Responsive Idiopathic Pulmonary Arterial Hypertension: Evidence for a New Disease? Evan L. Brittain and Anna R. Hemnes Vasodilator-Responsive Idiopathic Pulmonary Arterial Hypertension: Evidence for a New Disease? Evan L. Brittain and Anna R. Hemnes Metrics Cited byEndothelial Damage in Acute Respiratory Distress SyndromePulmonary hypertension in end-stage renal diseaseNovel Biomarkers of Pulmonary HypertensionPathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an updatePulmonary capillary recruitment in exercise and pulmonary hypertensionVasodilator responsiveness in idiopathic pulmonary arterial hypertension: identifying a distinct phenotype with distinct physiology and distinct prognosisWhat's in a side effect? 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Hemnes, MDResearch Reviews: Vasoreactivity in PAH 20 January 2015Volume 162, Issue 2Page: 154-156KeywordsBlood flowCapillariesCardiac outputCatheterizationHemodynamicsLungsPhenotypesPulmonary hypertensionVasodilators ePublished: 20 January 2015 Issue Published: 20 January 2015 Copyright & PermissionsCopyright © 2015 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...