Pulmonary function in primary pulmonary hypertension. Journal of the American College of Cardiology

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doi:10.1016/S0735-1097(02)02964-9 2003;41;1028-1035 J. Am. Coll. Cardiol.Xing-Guo Sun, James E. Hansen, Ronald J. Oudiz, and Karlman Wasserman Pulmonary function in primary pulmonary hypertensionThis information is current as of November 19, 2009 http://content.onlinejacc.org/cgi/content/full/41/6/1028located on the World Wide Web at: The online version of this article, along with updated information and services, is

by on November 19, 2009 content.onlinejacc.orgDownloaded from PulmonaryFunctioninPrimaryPulmonaryHypertension

Xing-GuoSun,MD,JamesE.Hansen,MD,RonaldJ.Oudiz,MD,FACC,KarlmanWasserman,PHD,MD

Torrance,California

OBJECTIVESThestudywasdonetoascertainthedegreetowhichabnormalitiesinrestinglungfunctioncorrelatewiththediseaseseverityofpatientswithprimarypulmonaryhypertension(PPH).BACKGROUNDPatientswithPPHareoftendifficulttodiagnoseuntilseveralyearsaftertheonsetofsymptoms.Despitetheseriousnessofthedisorder,thediagnosisofPPHisoftendelayedbecauseitisunsuspectedandrequiresinvasivemeasurements.AlthoughPPHoftencausesabnormalitiesinrestinglungfunction,theseabnormalitieshavenotbeenshowntobestatisticallysignificantwhencorrelatedwithothermeasuresofPPHseverity.METHODSRestinglungmechanicsanddiffusingcapacityforcarbonmonoxideDLCOwereassessedin79

patientswhosefindingsconformedtotheclassicaldiagnosticcriteriaofPPHandwhohadnoevidenceofsecondarycausesofpulmonaryhypertension.Thesefindingswerecorrelatedwithseverityofdiseaseasassessedbycardiaccatheterization,NewYorkHeartAssociation(NYHA)class,andcardiopulmonaryexercisetesting.RESULTSWhenPPHpatientswerefirstevaluatedatourreferralclinic,theDLCOandlungvolumeswere

decreasedinapproximatelythree-quartersandone-half,respectively.ThedecreasesinDLCO,and

toalesserextentlungvolumes,correlatedsignificantlywithdecreasesinpeakoxygenuptake(reflectingmaximumcardiacoutput),peakoxygenpulse(reflectingmaximumstrokevolume),andanaerobicthreshold(reflectingsustainableexercisecapacity)andhigherNYHAclass.CONCLUSIONSPatientswithPPHcommonlyhaveabnormalitiesinlungmechanicsandDLCOlevelsthat

correlatesignificantlywithdiseaseseverity.Thesemeasurementscanbeusefulinevaluatingpatientswithunexplaineddyspneaandfatigue.(JAmCollCardiol2003;41:1028–35)©2003bytheAmericanCollegeofCardiologyFoundation

Primarypulmonaryhypertension(PPH)isarare,life-threateningillnessthatistypicallydiagnosedayearormoreafterpatientsbecomesymptomatic(1–4).Itbeginswithalterationstothepulmonaryarteriolesandcapillariesthatleadtoincreasedpulmonaryvascularresistance,rightventricularhypertrophyand/ordilation,decreasedsystemicandpulmo-naryperfusion,andanincreaseindead-spaceventilation.Boththeincreasedventilatoryrequirementandthedecreasedcardiacoutputresponsetoexercisecontributetothepredominantsymptomsofexercisedyspneaandfatigue(5,6),symptomscommontomanydisorders,eitherorganicorfunctional.Unfortunately,mostpatientswithPPHarediagnosedinadvancedstagesoftheirdisease,whenthemeansurvivalrateislessthanthreeyearswithouttreatment(5,6).Becauseofthelackofdistinctivephysical,radiographic,andelectrocardio-graphicfindingsinPPH,cardiaccatheterizationisrequiredtoestablishandconfirmthediagnosis(5,7).Severalstudies(6–12)havefoundthatsimple,noninvasivelungfunctionmeasurements,especiallythegastransferindexordiffusingcapacityforcarbonmonoxide(DLCO),canalsobeabnormalinPPHpatients.ThisisnotsurprisingconsideringthatthepathologyofPPHprimarilyinvolvesthesmallpulmonaryarteriesandcapillaries,andthattheDLCOisdependentontheaccessandtransferofinhaledcarbonmonoxidetothehemoglobininthepulmonarycapillaries.However,noneoftheabovestudieshaveshownsignificantcorrelationsofDLCOwiththeseverityofthediseaseasmeasuredbyNewYorkHeartAssociation(NYHA)class,restinghemodynamicmeasurements,orcardiopulmonaryex-ercisetest(CPET)parameters.TheCPETcanbesafelyperformedinPPHpatientsto:1)detectpatternsofgasexchangeabnormalitiesthataretypicalofPPH,2)quantifydiseaseseverity,and3)identifythepresenceofright-to-leftshunting(2,3,13,14).Specifically,theseverityofPPHhasbeenshowntobecorrelatedwithseveralCPETparameters,includ-ingpeakO2uptake(maximalaerobiccapacity),peakO2pulse,andanaerobicthreshold(maximalsustainableexerciselevel)(2).WehypothesizedthattheDLCO,andperhapsotherlungfunctionmeasurements,wouldbesignificantlycorrelatedwiththeseverityofPPHassessedinotherways.Thus,in79patientswithwell-documenteddiagnosesofPPHand20controlsubjects,restinglungfunctionmeasurements(includ-ingspirometric,lungvolume,andDLCOvalues)werecorre-

latedwithCPETparameters,restinghemodynamicvariables(measuredduringcardiaccatheterization),andNYHAsymp-tomclass.