Pulmonary Hypertension - PHA Online University

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Table of ContentsGuest Editor for this issue:Kristin Highland, MD, MSCRAssistant ProfessorDivision of Pulmonary and CriticalCare MedicineDirector, Pulmonary Hypertension ClinicMedical University of South CarolinaCharleston, South Carolina264 Profiles in PulmonaryHypertension:Virginia D. Steen, MD277 International Corner:Combination Therapy in PAH278 CME Section280 Pulmonary Hypertensionin Systemic LupusErythematosus286 Pulmonary Hypertension andthe AntiphospholipidSyndrome292 Scleroderma AssociatedPulmonary Hypertension299 CME Self-AssessmentExamination301 Pulmonary HypertensionRoundtable: PAH andSclerodermaPublisherPulmonary Hypertension AssociationMichael D. McGoon, MD, Chair of the BoardRino Aldrighetti, PresidentDonica Merhazion, Associate Director of Medical ServicesPHA OfficePulmonary Hypertension Association801 Roeder Rd. Suite 400Silver Spring, MD 20910-4496301-565-3004, 301-565-3994 (fax)www.phassociation.org© 2008 by Pulmonary Hypertension Association. All rightsreserved. None of the contents may be reproduced in anyform whatsoever without the written permission of PHA.ISSN: 1933-088X (print); 1933-0898 (online)Editorial OfficesAdvances in Pulmonary Hypertension, DataMedica,P.O. Box 1688, Westhampton Beach, NY 11978Tel (631) 288-7733 Fax (631) 288-7744E-mail: sbelsonchapman@aol.comPublishing StaffStu Chapman, Executive EditorSusan Chapman, Managing EditorHeidi Green, Associate EditorGloria Catalano, Production DirectorMichael McClain, Design DirectorAdvances in Pulmonary Hypertension is circulated to cardiologists,pulmonologists, rheumatologists, and other selectedphysicians by the Pulmonary Hypertension Association. Thecontents are independently determined by the Editor and theEditorial Advisory Board. All past issues of the journal areavailable at: www.PHAssociation.org/Medical/Advances_in_PH/Cover ImageThis illustration symbolizes the malar ("butterfly") rash that ischaracteristic of patients with systemic lupus erythematosus.The artwork was provided by Martin J. Somsky.Guest Editor’s MemoTargeting the Connection BetweenPAH and Connecive Tissue DiseasePulmonary hypertension (PH) is associated with all of the connectivetissue diseases (CTDs), but it is most commonly seen insystemic sclerosis (scleroderma), followed by systemic lupus erythematosus,and the antiphospholipid antibody syndrome. Although theprognosis for CTD-associated PH has improved with the availabilityof numerous pulmonary arterial hypertension therapies, it stillcarries a substantially poorer prognosis than idiopathic pulmonaryarterial hypertension, and necessitates additional research into itstreatment and prevention.Further complicating treatment, CTD-associated PH is often multifactorial andcan be caused by any of the World Health Organization categories of PH. In thisissue of Advances in Pulmonary Hypertension, the contributors review what is knownregarding the epidemiology, pathobiology, and treatment of PH, with a focus on pulmonaryarterial hypertension in systemic sclerosis, systemic lupus erythematosus,and antiphospholipid syndrome.—Kristin B. Highland, MD, MSCREditor’s MemoHighlighting Current and FuturePerspectives in the JournalThe recent increase in awareness and recognition of pulmonaryhypertension is in part related to the efforts of Rheumatologistswho are now screening many of their patients for this deadly disease.This issue, guest edited by Dr Kristin Highland, focuses onthe spectrum of connective tissue diseases and their relation topulmonary hypertension. While there is still much to learn abouthow and why patients with connective tissue diseases develop pulmonaryhypertension, the benefits of treatment for many of thesepatients are apparent. This issue also spotlights rheumatologist,Dr Virginia Steen, whose efforts in the world of pulmonary hypertension, particularlyon screening and early diagnosis, are close to my heart. For our new InternationalCorner, Dr Nick Morrell provides commentary on combination therapy issues coveredin the previous issue of Advances, with an international perspective.I am pleased to preview important content to be published in Advances in PulmonaryHypertension in the next 2 issues. The journal will be focusing on 2 meetingsheld earlier this year that provided relevant and timely updates on pulmonaryhypertension. First, physicians who were unable to attend the World Health Organization4 th World Symposium on Pulmonary Hypertension will be pleased to seethe next issue and its comprehensive discussion of key findings from this meeting.Among the topics to be covered are: current perspectives on pathophysiology,diagnosis and assessment of pulmonary arterial hypertension (PAH), interventionaland surgical modalities, endpoints and clinical trial design in PAH, as well asinflammation, growth factors, and pulmonary vascular remodeling.In the following issue, the journal will feature content from another importantmeeting, this year’s 8 th International Conference and Scientific Sessions of thePulmonary Hypertension Association. The information from this meeting will begleaned from physicians who led the sessions and who are among the leadinginvestigators in the field. We look forward to providing articles on these importantissues and welcome your feedback. Please send your comments and suggestionsto our executive editor at Stulink@aol.com.Ronald J. Oudiz, MDEditor-in-Chief
Editorial Advisory BoardEditor-in-ChiefRonald J. Oudiz, MDAssociate Professor of MedicineUCLA School of MedicineDirector, Liu Center for PulmonaryHypertensionDivision of CardiologyLos Angeles Biomedical ResearchInstitute at Harbor-UCLAMedical CenterTorrance, CaliforniaImmediate Past EditorVallerie V. McLaughlin, MDAssociate Professor of MedicineDirector, Pulmonary HypertensionProgramUniversity of Michigan Health SystemAnn Arbor, MichiganEditor-in-Chief ElectRichard Channick, MDProfessor of Clinical MedicinePulmonary and Critical Care DivisionUniversity of California, San DiegoMedical CenterSan Diego, CaliforniaAssociate EditorsErika Berman Rosenzweig, MDAssistant Professor of PediatricsDepartment of PediatricsColumbia College of Physiciansand SurgeonsNew York, New YorkTodd Bull, MDAssociate Professor of MedicineMedical Director, ICU AnshutzInpatient PavilionDivision of Pulmonary Sciences andCritical Care MedicineUniversity of Colorado Health SciencesCenterDenver, ColoradoRobert Schilz, DO, PhDMedical Director of Lung Transplantationand Pulmonary Vascular DiseaseUniversity Hospital of ClevelandCase Western Reserve UniversityCleveland, OhioEditorial BoardTeresa De Marco, MDDirector, Heart Failure andPulmonary Hypertension ProgramUniversity of California, San FranciscoSan Francisco, CaliforniaEli Gabbay, MDAssociate ProfessorUniversity of Western AustraliaSchool of Medicine and PharmacologyMedical Director, Advanced Lung Diseaseand Pulmonary Vascular UnitRoyal Perth HospitalPerth, AustraliaKristin Highland, MDAssistant ProfessorDivision of Pulmonary and CriticalCare MedicineDirector, Pulmonary Hypertension ClinicMedical University of South CarolinaCharleston, South CarolinaOmar Minai, MDStaff PhysicianCleveland ClinicCleveland, OhioMyung H. Park, MDDirector, Pulmonary VascularDiseases ProgramUniversity of Maryland School ofMedicineBaltimore, MarylandIoana Preston, MDAssistant Professor of MedicineTufts-New England Medical CenterBoston, MassachusettsZeenat Safdar, MDAssistant Professor of MedicineDepartment of Medicine,Pulmonary & Critical Care SectionPulmonary Hypertension CenterBaylor College of MedicineHouston, TexasRajan Saggar, MDAssistant Professor of MedicineDivision of Pulmonary and Critical CareMedicine and HospitalistsDavid Geffen School ofMedicine at UCLALos Angeles, CaliforniaFrancisco Soto, MDAssistant ProfessorDirector, Pulmonary HypertensionProgramMedical College of WisconsinMilwaukee, WisconsinFernando Torres, MDDirector, Pulmonary HypertensionProgramUT Southwestern Medical CenterDallas, TexasProgram DescriptionThe mission of Advances in PulmonaryHypertension is to serve as the premiereforum for state of the art information regardingdiagnosis, pathophysiology, and treatment ofpulmonary hypertension. The 2003 Venicerevision of the World Health OrganizationClassification serves as a guide to categoriesof pulmonary hypertension addressed by theJournal. While focusing on WHO Group I PAH,the other categories (Group II, Left heartdisease; Group III, Associated with lung diseaseand/or hypoxemia; Group IV, Thromboticand/or Embolic Disease; Group V, Miscellaneous)are also addressed. This mission isachieved by a combination of invited reviewarticles, Roundtable discussions with panelsconsisting of international experts in PH, andoriginal contributions. In addition, a specialsection entitled “Profiles in PulmonaryHypertension”recognizes major contributors tothe field and serves as an inspiring reminderof the rich and collegial history of dedicationto advancing the field.Objectives• Provide up-to-date information regardingdiagnosis, pathophysiology, and treatmentof pulmonary hypertension.• Serve as a forum for presentation and discussionof important issues in the field,including new paradigms of disease understandingand investigational trial design.• Recognize and preserve the rich history ofindividuals who have made major contributionsto the field via dedication to patientcare, innovative research, and furtheringthe mission of the PH community to curepulmonary hypertension.The Scientific LeadershipCouncil of the PulmonaryHypertension AssociationThe scientific program of the PulmonaryHypertension Association is guided bythe association’s Scientific LeadershipCouncil. The Council includes thefollowing health care professionals:Vallerie V. McLaughlin, MDSLC ChairUniversity of Michigan Health SystemAnn Arbor, MichiganDavid B. Badesch, MDSLC Immediate Past ChairUniversity of Colorado HealthSciences CenterDenver, ColoradoJohn H. Newman, MDSLC Chair ElectVanderbilt Medical SchoolNashville, TennesseeRobyn J. Barst, MDNew York, New YorkRaymond L. Benza, MDUniversity of Alabama Health SystemBirmingham, AlabamaTodd Bull, MDUniversity of Colorado HealthSciences CenterDenver, ColoradoRichard N. Channick, MDUCSD Medical CenterSan Diego, CaliforniaC. Gregory Elliott, MDLDS HospitalUniversity of Utah School of MedicineSalt Lake City, UtahKaren A. Fagan, MDUniversity of South AlabamaCollege of MedicineMobile, AlabamaAdaani Frost, MDBaylor College of MedicineHouston, TexasJohn Granton, MDToronto General HospitalToronto, CanadaNazzareno Galiè, MDInstitute of CardiologyUniversity of BolognaBologna, ItalyNicholas S. Hill, MDDivision of Pulmonary, Critical Careand Sleep MedicineTufts-New England Medical CenterBoston, MassachusettsMarius Hoeper, MDHannover Medical schoolHannover, GermanyDunbar Ivy, MDUniversity of Colorado HealthSciences CenterDenver, ColoradoZhi-Cheng Jing, MDFu wai Heart HospitalBeijing, ChinaAnne M. Keogh, MDSt. Vincent’s Public HospitalSydney, AustraliaMichael J. Krowka, MDMayo ClinicRochester, MinnesotaJames E. Loyd, MDVanderbilt University Medical CenterNashville, TennesseeMichael D. McGoon, MDChair, PHA Board of TrusteesPulmonary Hypertension ClinicMayo ClinicRochester, MinnesotaSrinivas Murali, MDAllegheny General HospitalPittsburgh, PennsylvaniaRonald J. Oudiz, MDLiu Center for Pulmonary HypertensionLos Angeles Biomedical ResearchInstituteHarbor-UCLA Medical CenterTorrance, CaliforniaMarlene Rabinovitch, MDStanford University School of MedicineStanford, CaliforniaErica Berman-Rosenzweig, MDColumbia-Presbyterian Medical CenterNew York, New YorkIvan M. Robbins, MDSLC Scientific Sessions CommitteeVanderbilt UniversityNashville, TennesseeJulio Sandoval, MDCardiopulmonary DepartmentNational Institute of Cardiologyof MexicoTlalpan, MexicoRichard Silver, MDMedical University of South CarolinaCharleston, South CarolinaVictor F. Tapson, MDDivision of Pulmonary and CriticalCare MedicineDuke University Medical CenterDurham, North CarolinaLiaisonsArlene Schiro, RN, MA, ACNP-BCChair, PH Resource NetworkMassachusetts General HospitalBoston, MassachusettsJoanne Sperando SchmidtPatient LiaisonEmeritus MembersBruce H. Brundage, MDSt. Charles Medical Center-BendBend, OregonAlfred P. Fishman, MDUniversity of Pennsylvania HealthSystemPhiladelphia, PennsylvaniaThe Mission of the Scientific LeadershipCouncil is to provide medical and scientificguidance and support to the PHA by:• Developing and disseminating knowledgefor diagnosing and treating pulmonaryhypertension• Advocating for patients with pulmonaryhypertension• Increasing involvement of basic and clinicalresearchers and practitionersMore information on PHA’s ScientificLeadership Council and associatedcommittees can be found at:www.PHAssociation.org/SLC/Advances in Pulmonary Hypertension 263
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