Cor Pulmonale - Introduction To Cor Pulmonale, Etiology and Pathophysiology of Cor Pulmonale, Epidemiology of Cor Pulmonale

4/4/2017 CorPulmonale:IntroductiontoCorPulmonale,EtiologyandPathophysiologyofCorPulmonale,EpidemiologyofCorPulmonale
CorPulmonaleOverviewofCorPulmonale
Management
Updated:Mar03,2016
Author:DerekLeong,MDChiefEditor:HenryHOoi,MD,MRCPImore...
OVERVIEWOFCORPULMONALEMANAGEMENT
IntroductiontoCorPulmonale
Corpulmonaleisdefinedasanalterationinthestructureandfunctionoftherightventricle(RV)ofthe
heartcausedbyaprimarydisorderoftherespiratorysystem.Pulmonaryhypertensionisoftenthe
commonlinkbetweenlungdysfunctionandtheheartincorpulmonale.Rightsidedventricular
diseasecausedbyaprimaryabnormalityoftheleftsideoftheheartorcongenitalheartdiseaseisnot
consideredcorpulmonale,butcorpulmonalecandevelopsecondarytoawidevarietyof
cardiopulmonarydiseaseprocesses.Althoughcorpulmonalecommonlyhasachronicandslowly
progressivecourse,acuteonsetorworseningcorpulmonalewithlifethreateningcomplicationscan
occur.[1]
EtiologyandPathophysiologyofCorPulmonale
Thepathophysiologyofcorpulmonaleisaresultofincreasedrightsidedfillingpressuresfrom
pulmonaryhypertensionthatisassociatedwithdiseasesofthelung.Theincreasedafterloadleadsto
structuralalterationsintherightventricle(RV)includingRVhypertrophy(RVH)whichcanbeseenin
chroniccorpulmonale.
Acutecorpulmonale:pulmonaryembolism(morecommon)andacuterespiratorydistresssyndrome
(ARDS).Theunderlyingpathophysiologyinamassivepulmonaryembolismcausingcorpulmonaleis
thesuddenincreaseinpulmonaryresistance.InARDS,RVoverloadcanoccurduetomechanical
ventilationandthepathologicfeaturesofthesyndromeitself.Mechanicalventilation,especiallyhigher
tidalvolumes,requiresahighertranspulmonarypressure.
InthecaseofARDS,corpulmonaleisassociatedwithanincreasedpossibilityofrighttoleftshunting
throughapatentforamenovale,whichcarriesapoorerprognosis.[2]
Severaldifferentpathophysiologicmechanismscanleadtopulmonaryhypertensionand,
subsequently,tocorpulmonale.TheWorldHealthOrganization(WHO)hasfiveclassificationsfor
pulmonaryhypertension,andallexceptoneofthesegroupscanresultincorpulmonale(WHO
Classificationgroup2ispulmonaryarteryhypertensionduetoleftventricular[LV]dysfunction).[3]
NotethefollowingWHOclassifications:
Group1:Pulmonaryarteryhypertension,includingheritablecausesconnectivetissue
disorders,includingsclerodermaandotheridiopathiccauses
Group3:Pulmonaryhypertensionduetolungdiseaseand/orhypoxiathesedisordersinclude
chronicobstructivepulmonarydisease(COPD),whichisthemostcommoncauseoffor
pulmonale.Therehavebeenstudiescorrelatingthedegreeofhypoxiawiththeseverityofcor
http://emedicine.medscape.com/article/154062overview#a3 1/18
pulmonale.Otherdisordersthatcanresultincorpulmonaleinthisgroupincludeinterstitiallung
sisease(ILD)andobstructivesleepapnea(OSA)
Group4:Chronicthromboembolicpulmonaryhypertensionbloodclotsthatforminthelungs
canleadtoincreasedresistance,pulmonaryhypertensionand,subsequently,corpulmonale
Group5:Pulmonaryhypertensioncausedbyotherdiseasesorconditions,includingsarcoidosis,
polycythemiavera(whichcanleadtoincreasedbloodviscosityand,subsequently,pulmonary
hypertension),vasculitis,andotherdisorders.
Theendresultoftheabovemechanismsisincreasedpulmonaryarterialpressureandresistance.
RVandLVoutput
TheRVisathinwalledchamberthatisabettervolumepumpthanapressurepump.Itisbetter
suitedtoadapttochangingpreloadthanafterload.Withanincreaseinafterload,theRVsystolic
pressureisincreasedtomaintainthecirculatorygradient.Atacriticalpoint,afurtherincreasein
pulmonaryarterialpressureandresistanceproducessignificantRVdilatation,anincreaseinRVend
diastolicpressure,andRVcirculatoryfailure.
AdecreaseinRVoutputleadstoadecreaseinLVfilling,whichresultsindecreasedcardiacoutput.
Becausetherightcoronaryarteryoriginatesfromtheaorta,decreasedLVoutputcausesdecreased
rightcoronarybloodflowandischemiatotheRVwall.Whatensuesisaviciouscyclebetween
decreasesinLVandRVoutput.
RVandLVmorphogenesis
Geneticinvestigationshaveconfirmedthatmorphogenesisoftherightandleftventricleoriginated
fromdifferentsetsofprogenitorcells.Theirdifferingembryologicoriginscouldexplainthediffering
ratesofhypertrophyoftherightandleftventricles.[4]
RVoverload
RVpressureandvolumeoverloadisassociatedwithseptaldisplacementtowardtheleftventricle.
Septaldisplacement,whichcanbevisualizedonechocardiography,isanadditionalfactorthat
decreasesLVfillingandoutputinthesettingofcorpulmonaleandRVenlargement.
EpidemiologyofCorPulmonale
AlthoughtheprevalenceofCOPDintheUnitedStatesisreportedtobeabout15million,theexact
prevalenceofcorpulmonaleisdifficulttodetermine,asphysicalexaminationandroutinetestsare
relativelyinsensitiveforthedetectionofpulmonaryhypertensionandRVdysfunction.
Corpulmonaleisestimatedtoaccountfor67%ofalltypesofadultheartdiseaseintheUnited
States,withchronicobstructivepulmonarydisease(COPD)duetochronicbronchitisoremphysema
thecausativefactorinmorethan50%ofcases.MortalityinpatientswithconcurrentCOPDandcor
pulmonaleishigherthanthatinpatientswithCOPDalone.Inaddition,corpulmonaleaccountsfor10
30%ofdecompensatedheartfailurerelatedadmissionsintheUnitedStates.[5]
Incontrast,acutecorpulmonaleisusuallysecondarytomassivepulmonaryembolism.Acutemassive
pulmonarythromboembolismisthemostcommoncauseofacutelifethreateningcorpulmonalein
adults50,000deathsintheUnitedStatesareestimatedtooccurperyearfrompulmonaryemboliand
abouthalfoccurwithinthefirsthourduetoacuterightheartfailure.
Globally,theincidenceofcorpulmonalevarieswidelyamongcountries,dependingontheprevalence
ofcigarettesmoking,airpollution,andotherriskfactorsforvariouslungdiseases.
CorPulmonalePresentation
Theclinicalmanifestationsofcorpulmonalemaybenonspecific.Thesymptomsmaybesubtle,
especiallyinearlystagesofthedisease,andtheymaybemistakenlyattributedtotheunderlying
pulmonarypathology.
Symptoms
Patientsmayreportacombinationoffatigue,tachypnea,exertionaldyspnea,andcough.Anginal
chestpaincanalsooccurandmaybeduetorightventricularischemiaorpulmonaryarterystretching,
whichtypicallydonotrespondtonitrates.Avarietyofneurologicsymptomsmaybeseendueto
decreasedcardiacoutputandhypoxemia.
Hemoptysismayoccurduetoruptureofadilatedoratheroscleroticpulmonaryarteriole.Other
conditions,suchastumors,bronchiectasis,andpulmonaryinfarction,shouldbeexcludedbefore
attributinghemoptysistopulmonaryhypertension.Rarely,thepatientmaycomplainofhoarseness
duetocompressionoftheleftrecurrentlaryngealnervebyadilatedpulmonaryartery.
Inadvancedstages,passivehepaticcongestionsecondarytosevererightventricularfailuremaylead
toanorexia,rightupperquadrantabdominaldiscomfort,andjaundice.Inaddition,syncopewith
exertion,whichmayalsobeseeninseveredisease,reflectsarelativeinabilitytoincreasecardiac
outputduringexercisewithasubsequentdropinthesystemicarterialpressure.
Elevatedpulmonaryarterypressurecanleadtoelevatedrightatrial,peripheralvenous,andcapillary
pressure.Byincreasingthehydrostaticgradient,itleadstotransudationoffluidandaccumulationof
peripheraledema.Althoughthisisthesimplestexplanationforperipheraledemaincorpulmonale,
otherfactorsmaycontribute,especiallyinasubsetofpatientswithchronicobstructivepulmonary
disease(COPD)whodonothaveanincreaseinrightatrialpressure.Adecreaseinglomerular
filtrationrate(GFR)andfiltrationofsodiumaswellasstimulationofargininevasopressin(which
decreasesfreewaterexcretion)byhypoxemiamayplayimportantpathophysiologicrolesinthis
settingandmayevenhavearoleforperipheraledemainpatientswithcorpulmonale.[6]
Signs
Physicalfindingsmayreflecttheunderlyinglungdiseaseorpulmonaryhypertension,rightventricular
hypertrophy(RVH),andRVfailure.Anincreaseinchestdiameter,laboredrespiratoryeffortswith
retractionsofthechestwall,distendedneckveinswithprominentaorvwaves,andcyanosismaybe
seen.
Onauscultationofthelungs,wheezesandcracklesmaybeheardassignsofunderlyinglung
disease.Turbulentflowthroughrecanalizedvesselsinchronicthromboembolicpulmonary
hypertension[7]maybeheardassystolicbruitsinthelungs.Onpercussion,hyperresonanceofthe
lungsmaybeasignofunderlyingCOPD.
Splittingofthesecondheartsoundwithaccentuationofthepulmoniccomponentcanbeheardinthe
earlystages.Asystolicejectionmurmurwithasharpejectionclickovertheregionofthepulmonary
arterymaybeheardinadvanceddisease,alongwithadiastolicpulmonaryregurgitationmurmur.
OtherfindingsuponauscultationofthecardiovascularsystemmaybeRVthirdandfourthsoundsor
thesystolicmurmuroftricuspidregurgitation.
RVHischaracterizedbyaleftparasternalorsubxiphoidheave.Hepatojugularrefluxandpulsatileliver
aresignsofRVfailurewithsystemicvenouscongestion.Inseveredisease,ascitescanalsobe
present.
Examinationofthelowerextremitiesrevealsevidenceofpittingedema.Edemaincorpulmonaleis
stronglyassociatedwithhypercapnia.[8]
DiagnosticConsiderations
Ageneralapproachtodiagnosecorpulmonaleandtoinvestigateitsetiologystartswithroutine
laboratorytests,chestradiography,andelectrocardiography(seetheseparatesectionsbelow).
Echocardiographygivesvaluableinformationaboutthediseaseandrightventricular(RV)function,as
wellasassistingindeterminingtheetiologyofpulmonaryhypertensionandcorpulomale.Rightheart
catheterizationisthemostaccuratebutinvasivetesttoconfirmthediagnosisofcorpulmonaleand
givesimportantinformationregardingunderlyingcauses.[9,10]
Onceadiagnosisofcorpulmonaleismade,itshouldbefollowedbyfurtherinvestigationtodetermine
theunderlyinglungpathology.Sometimesacommonlungdiseasesuchaschronicobstructive
pulmonarydisease(COPD)isnottheonlylungpathologycausingcorpulmonaleotherlungdiseases
maycoexist.Thus,pulmonaryfunctiontestsmayberequiredtoconfirmthepresenceofotherlung
pathologies.Ventilation/perfusion(V/Q)scanningorchestcomputedtomography(CT)scanningmay
beperformedifthepatientshistoryandphysicalexaminationsuggestpulmonarythromboembolism
asthecauseorifotherdiagnostictestsdonotprovideaspecificetiology.
Imagingstudiesmayshowevidenceofunderlyingcardiopulmonarydiseases,pulmonary
hypertension,orRVenlargement.Cardiacmagneticresonance(CMR)imagingisanotherformof
noninvasiveimagingthatdoesnotuseionizingradiation.CMRcanbeusedtoevaluatecor
pulmonale,anditisusefulindeterminingRVstructure,remodeling,andfunctionthismodalityis
especiallyusefulinassessingpulmonaryarterydimensionswhencomparedtotraditional
echocardiography.
Differentials
Whendiagnosingcorpulmonale,itisimportanttoconsiderthepossibilityofthromboembolicdisease
andprimarypulmonaryhypertensionaspossibleetiologies.Inaddition,alsoassessforthefollowing
conditions:
Atrialmyxoma
Blooddisordersthatareassociatedwithincreasedbloodviscosity
Congestive(biventricular)heartfailure
Constrictivepericarditis
Highoutputheartfailure
Infiltrativecardiomyopathies
Primarypulmonicstenosis
Rightheartfailureduetorightventricularinfarction
Rightheartfailureduetocongenitalheartdiseases
Ventricularseptaldefect
DiagnosticTests
Laboratoryinvestigationsaredirectedtowarddefiningthepotentialunderlyingetiologiesaswellas
evaluatingthecomplicationsofcorpulmonale.Inspecificinstances,appropriatelaboratorystudies
mayincludethefollowing:
Hematocritforpolycythemia,whichcanbeaconsequenceofunderlyinglungdiseasebutcan
alsoincreasepulmonaryarterialpressurebyincreasingviscosity
Serumalpha1antitrypsin,ifdeficiencyissuspected
Antinuclearantibody(ANA)levelforcollagenvasculardisease,andantiSCL70antibodiesin
scleroderma
Coagulationsstudiestoevaluatehypercoagulabilitystates(eg,serumlevelsofproteinsSand
C,antithrombinIII,factorVLeyden,anticardiolipinantibodies,homocysteine)
ArterialBloodGasAnalysis
Arterialbloodgasmeasurementsmayprovideimportantinformationaboutthelevelofoxygenation
andtypeofacidbasedisorder.
BrainNatriureticPeptide
Brainnatriureticpeptide(BNP)isapeptidehormonethatisreleasedinresponsetovolumeexpansion
andtheincreasedwallstressofcardiacmyocytes.BNPhelpstopromotediuresis,natriuresis,
vasodilationofthesystemicandpulmonaryvasculature,andreductionofcirculatinglevelsof
endothelinandaldosterone.Asaresult,bothcongestiveheartfailureduetoleftventricular(LV)failure
andcorpulmonaleduetononcardiacpulmonaryhypertensioncanleadtoelevationsinplasmaBNP.
Althoughnotspecific,severeacutedecompensatedLVheartfailurecanresultinhigherlevelsofBNP.
ChestRadiography
Inpatientswithchroniccorpulmonale,thechestradiographmayshowenlargementofthecentral
pulmonaryarterieswitholigemicperipherallungfields.Pulmonaryhypertensionshouldbesuspected
whentherightdescendingpulmonaryarteryislargerthan16mmindiameterandtheleftpulmonary
arteryislargerthan18mmindiameter.Rightventricularenlargementleadstoanincreaseofthe
transversediameteroftheheartshadowtotherightontheposteroanteriorviewandfillingofthe
retrosternalairspaceonthelateralview.Thesefindingshavereducedsensitivityinthepresenceof
kyphoscoliosisorhyperinflatedlungs.
Electrocardiography
Electrocardiographic(ECG)abnormalitiesincorpulmonalereflectthepresenceofrightventricular
hypertrophy(RVH),RVstrain,orunderlyingpulmonarydisease(seetheimagebelow).SuchECG
changesmayincludethefollowing:
Rightaxisdeviation
R/SamplituderatioinV1greaterthan1(anincreaseinanteriorlydirectedforcesmaybeasign
ofposteriorinfarction)
R/SamplituderatioinV6lessthan1
Ppulmonalepattern(anincreaseinPwaveamplitudeinleads2,3,andaVF)
S1Q3T3patternandincomplete(orcomplete)rightbundlebranchblock,especiallyif
pulmonaryembolismistheunderlyingetiology
LowvoltageQRSbecauseofunderlyingCOPDwithhyperinflation
SevereRVHmayreflectasQwavesintheprecordialleadsthatmaybemistakenlyinterpretedasan
anteriormyocardialinfarction(however,aselectricalactivityoftheRVissignificantlylessthantheleft
ventricle[LV],smallchangesinRVforcesmaybelostintheECG).Seetheimagebelow.
ThisECGshowssometypicalabnormalitiesthatmaybeseenincorpulmonaleandotherchronicpulmonary
diseases:(1)R/Sratio>1inV1and<1inV6suggestiveofrightventricularhypertrophy/enlargement,(2)right
superioraxisdeviation,(3)leftatrialtypeofpwavewithincreasedwidthofthepwaveandbiphasicpwaveinV1,
and(4)rightbundlebranchblockpatternwithwideQRSandRsR1patterninV1andslurredswaveinV6.This
ECGalsopresentsasinusbradycardiarhythmwithfirstdegreeAVblockandleftanteriorfascicularblock.
Additionally,manyrhythmdisturbancesmaybepresentinchroniccorpulmonaletheserangefrom
isolatedprematureatrialdepolarizationstovarioussupraventriculartachycardias,including
paroxysmalatrialtachycardia,multifocalatrialtachycardia,atrialfibrillation,atrialflutter,andjunctional
tachycardia.Thesedysrhythmiasmaybetriggeredbyprocessessecondarytotheunderlyingdisease,
(eg,anxiety,hypoxemia,acidbaseimbalance,electrolytedisturbances,excessiveuseof
bronchodilators,heightenedsympatheticactivity).Lifethreateningventriculartachyarrhythmiasare
lesscommon.
Inselectedcases,pulmonaryfunctiontestingmaybeindicatedtodetermineunderlyingobstructiveor
interstitiallungdisease.
2DandDopplerEchocardiography
Twodimensional(2D)echocardiographyusuallydemonstratessignsofchronicrightventricular(RV)
pressureoverload.Asthisoverloadprogresses,increasedthicknessoftheRVwallwithparadoxical
motionoftheinterventricularseptumduringsystoleoccurs.Atanadvancedstage,RVdilatation
occurs,andtheseptumshowsabnormaldiastolicflattening.Inextremecases,theseptummay
actuallybulgeintotheleftventricular(LV)cavityduringdiastole,resultingindecreasedLVdiastolic
volumeandreductionofLVoutput.
Dopplerechocardiographyisusedtoestimatepulmonaryarterialpressure,takingadvantageofthe
functionaltricuspidinsufficiencythatisusuallypresentinpulmonaryhypertension.Thisimaging
modalityisconsideredthemostreliablenoninvasivetechniquetoestimatepulmonaryarterypressure.
However,theefficacyofDopplerechocardiographymaybelimitedbytheabilitytoidentifyan
adequatetricuspidregurgitantjet,whichmaybefurtherenhancedbyusingsalinecontrast.[11]
SeveralmethodsexisttoassessRVfunction.Onemethodincludestricuspidannularplanesystolic
excursion(TAPSE),whichismeasuredbyviewingtheheartintheapicalfourchamberviewandusing
theMmodefunctionalongthelateraltricuspidannulus.Bymeasuringthedistancetraveledofthis
referencepointduringsystole,thelongitudinalshorteningoftheRVcanbeusedasasurrogatefor
globalRVfunction.LimitationsincludeinadequateMmodeplacementandtheassumptionthatone
segmentofRVmotionisrepresentativeoftheentireRV.
Strain,whichisdistinctfrommeasuringwallmotionabnormalitiesintraditionalechocardiography,
involvesmeasuringmyocardialdeformationtoquantitativelyassessmyocardialfunction.Two
methodscurrentlyexistformeasuringstrain,includingtissueDopplerimaging(TDI)and2Dspeckle
tracking.TDIusespostprocessingtoconvertvelocitytostrainandstrainrates,butitissignificantly
limitedbytheDopplerangleofincidence.2Dspeckletrackingusesgreyscaletodetectspeckle
patternsbytrackingnaturalacousticmarkerstocalculatevelocityvectorswith2Dultrasonography.
However,2Dspeckletrackingreliesonhighimagequality.[12,13]
Additionally,myocardialperformanceindex(MPI)canalsobeusedtomeasureRVfunctionby
calculatingtheisovolumetricrelaxationtimeandcontractiontimedividedbytheejectiontime.Higher
MPIindicatesgreaterRVdysfunction,anditisindependentofRVchambersizeandgeometry.
PulmonaryThromboembolismImagingStudies
Pulmonarythromboembolismhasawiderangeofclinicalpresentationsfrommassiveembolismwith
acuteandseverehemodynamicinstabilitytomultiplechronicperipheralembolismsthatmaypresent
withcorpulmonale.[14]
Pulmonaryangiographywashistoricallythegoldstandardfordiagnosingacutepulmonaryembolism.
Theinjectionofaradiocontrastdyeunderfluoroscopyallowsfordirectimagingofthepulmonary
vasculature.Thishasbeenlargelyreplacedbycomputedtomographypulmonaryangiography
(CTPA),whichinvolvestheinjectionofaniodinatedcontrastwhileobtainingCTscanningofthechest.
CTPAisbothsensitiveandspecificandonlyrequiresintravenous(IV)accessasaresult,itisthe
firstlinediagnosticimagingmodalitytodiagnoseasuspectedpulmonaryembolism.
Ventilation/perfusion(V/Q)scanningisoftenperformedincasesinwhichtheiodinatedcontrastagent
usedinCTPAiscontraindicated(eg,pregnancy,renalinsufficiency,contrastallergy).Bycomparing
bothventilationandperfusionusingaradionucleotide,perfusiondeficitswithinareasofnormal
ventilationarehighlysuspiciousofapulmonaryembolism.V/Qscanningisthetestofchoicein
diagnosingchronicthromboembolicpulmonaryhypertension(CTEPH),asitismoresensitivethan
CTPA.[15]
Ultrafast,ECGgatedCTscanning
Ultrafast,electrocardiographically(ECG)gatedcomputedtomography(CT)scanninghasbeen
evaluatedtostudyrightventricular(RV)function.InadditiontoestimatingRVejectionfraction
(RVEF),thisimagingmodalitycanestimateRVwallmass.Althoughtheuseofultrafast,ECGgated
CTscanningisstillexperimental,withfurtherimprovement,itmaybeusedtoevaluatethe
progressionofcorpulmonaleinthenearfuture.
MagneticResonanceImaging
Cardiacmagneticresonance(CMR)imaginghasbeenusedasamethodofprovidinghighquality
imagesanddiagnosticcapabilitiesthatarecurrentlybeingexplored.Electrocardiographic(ECG)
gatedtechniquesandrespiratorymotionsuppressionhaveenabledprotocolsthatcanprovide
valuableinformationaboutrightventricular(RV)mass,septalflattening,andventricularfunction.By
incorporatinggadolinium,myocardialscarandfibrosiscanalsobeevaluatedviaCMR.Sucha
techniquecanbeusefulindeterminingthesizeandlocationofaninfarction.Spinecho,whichcauses
bloodtoappearblack,canbeusedforanatomicimagingandidentifyingabnormalmyocardium,and
cineimaging,inwhichbloodappearsbrightandthemyocardiumappearsdark,canhelpinthe
assessmentofwallmotionabnormalities,valvefunction,andpatternsofbloodflow.Asaresult,CMR
isbeingexploredtobettercharacterizeandquantifypulmonaryhypertension.[16,17]
NuclearImaging
Radionuclideventriculographycannoninvasivelydeterminerightventricularejectionfraction.
Myocardialperfusionmayalsoshowapermanentincreaseinbrightnessoftherightventricle.[18]
Ventilation/perfusion(V/Q)scanningcanbeparticularlyusefulinevaluatingpatientswithcor
pulmonale,especiallyifpulmonaryhypertensionisduetochronicthromboembolicpulmonary
hypertension(CTEPH).V/Qscansareperformedbyhavingthepatientinhalearadionucleotide
(typicallyxenonortechnetium)toassessventilation,whereasperfusionisevaluatedbythe
intravenousinjectionofanotherradionucleotide.Thetwoimagesarethenanalyzedtodetermineif
thereareanymismatchedperfusiondefects,whichissuggestiveofapulmonaryembolism.
V/Qscansaretypicallyinterpretedasbeingnormal,orhavingahigh,intermediate,orlowprobability
forpulmonaryembolism.InCTEPH,theV/Qscantypicallydemonstrateshavingahighprobabilityfor
pulmonaryembolismaswellashavingmultiplemismatchedperfusiondefectswhichcanbe
visualized.
CardiacCatheterization
Althoughhighresolutionechocardiographyandmagneticresonanceimagingareaccuratemethodsto
measurepulmonarypressure,[19]rightheartcatheterizationisconsideredthemostprecisemethod
fordiagnosisandquantificationofpulmonaryhypertension.Thisprocedureisindicatedwhen
echocardiographycannotassesstheseverityofatricuspidregurgitantjet,thusexcludingan
assessmentofpulmonaryhypertension.
Inpatientswithcorpulmonale,rightheartcatheterizationrevealsevidenceofrightventricular(RV)
dysfunctionwithoutleftventricular(LV)dysfunction.Hemodynamically,thistypicallypresentsasa
meanpulmonaryarterypressure(PAP)above25mmHg,whichleadstoelevatedRVsystolic
pressuresandcentralvenouspressures(CVP).However,thesefindingsarealsoseeninLV
dysfunction.Onemethodofdifferentiatingleftsidedfromrightsideddiseaseincludesmeasuringthe
pulmonarycapillarywedgepressure(PCWP),whichisanestimationofleftatrialpressure.Thus,RV
dysfunctionisalsodefinedashavingaPCWPbelow15mmHg,becausefailureoftheLVwouldresult
inelevatedLVenddiastolicpressuresand,subsequently,leftatrialpressures.[5]
Rightheartcatheterizationisoccasionallyimportantfordifferentiatingcorpulmonalefromoccultleft
ventriculardysfunction,especiallywhenthepresentationisconfusing.Anotherindicationisfor
evaluationofthepotentialreversibilityofpulmonaryarterialhypertensionwithvasodilatortherapyor
whenaleftsidedheartcatheterizationisindicated.
LungBiopsy
Lungbiopsymayoccasionallybeindicatedtodeterminetheetiologyofunderlyinglungdisease.This
isespeciallytrueifinterstitiallungdisease(ILD)isthesuspectedetiologyforpulmonaryhypertension
resultingincorpulmonale.
ILDencompassesabroadrangeofdiagnoses,includingbutnotlimitedtoexposurerelatedcauses
(eg,asbestosis,silicosis),complicationsofconnectivetissuedisorders(eg,rheumatoidarthritis,
systemiclupuserythematosus,scleroderma),andidiopathicpneumonia(eg,usualinterstitial
pneumonia,acuteinterstitialpneumonia,nonspecificinterstitialpneumonia,cryptogenicorganizing
pneumonia).
Typically,laboratorytests,pulmonaryfunctiontests,andimagingstudies,includinghighresolution
computedtomography(HRCT)scanning,areperformedbeforeproceedingtoinvasivelungbiopsy.
Lungbiopsycansometimesbeimportantindeterminingprognosisandmanagement,dependingon
thediagnosisobtainedviapathology.Biopsiescanbeobtainedwiththeuseoftransbronchialbiopsy,
thoracotomy,orvideoassistedthoracoscopicsurgery(VATS).
OverviewofCorPulmonaleManagement
Medicaltherapyforchroniccorpulmonaleisgenerallyfocusedontreatmentoftheunderlying
pulmonarydiseaseandimprovingoxygenationandrightventricular(RV)functionbyincreasingRV
contractilityanddecreasingpulmonaryvasoconstriction.[20]However,theapproachmightbedifferent
tosomedegreeinanacutesetting,withprioritygiventostabilizingthepatient.
CardiopulmonarysupportforpatientsexperiencingacutecorpulmonalewithresultantacuteRV
failureincludesfluidloadingandvasoconstrictor(eg,epinephrine)administrationtomaintain
adequatebloodpressure.Ofcourse,theprimaryproblemshouldbecorrected,ifpossible.For
example,formassivepulmonaryembolism,consideradministrationofanticoagulation,thrombolytic
agentsorsurgicalembolectomy,especiallyifcirculatorycollapseisimpendingconsider
bronchodilationandinfectiontreatmentinpatientswithchronicobstructivepulmonarydisease
(COPD)andconsidersteroidandimmunosuppressiveagentsininfiltrativeandfibroticlungdiseases.
Oxygentherapy,diuretics,vasodilators,digitalis,theophylline,andanticoagulationtherapyareall
differentmodalitiesusedinthelongtermmanagementofchroniccorpulmonale.
Patienteducation
Patienteducationregardingtheimportanceofadherencetomedicaltherapyisvitalbecause
appropriatetreatmentofbothhypoxiaandunderlyingmedicalillnesscanimprovemortalityand
morbidity.
Complications
Complicationsofcorpulmonaleincludesyncope,hypoxia,pedaledema,passivehepaticcongestion,
anddeath.
OxygenTherapy
Oxygentherapyisofgreatimportanceinpatientswithunderlyingchronicobstructivepulmonary
disease(COPD),[21]particularlywhenadministeredonacontinuousbasis.Withcorpulmonale,the
partialpressureofoxygen(PaO2)islikelytobebelow55mmHganddecreasesfurtherwithexercise
andduringsleep.
Oxygentherapyrelieveshypoxemicpulmonaryvasoconstriction,whichthenimprovescardiacoutput,
lessenssympatheticvasoconstriction,alleviatestissuehypoxemia,andimprovesrenalperfusion.The
multicenter,randomizedNocturnalOxygenTherapyTrial(NOTT)showedthatcontinuouslowflow
oxygentherapyforpatientswithsevereCOPDresultedinsignificantreductioninthemortalityrate.
[22]
Ingeneral,inpatientswithCOPD,longtermoxygentherapyisrecommendedwhenthePaO2isless
than55mmHgortheO2saturationislessthan88%.However,inthepresenceofcorpulmonaleor
impairedmentalorcognitivefunction,longtermoxygentherapycanbeconsideredevenifthePaO2
isgreaterthan55mmHgortheO2saturationisgreaterthan88%.
Althoughtheimpactofoxygentherapyonsurvivalinpatientswithcorpulmonaleduetopulmonary
disordersotherthanCOPDisunclear,itmayprovidesomedegreeofsymptomaticreliefand
improvementinfunctionalstatus.Therefore,oxygentherapyplaysanimportantroleinboththe
immediatesettingandlongtermmanagement,especiallyinpatientswhoarehypoxicandhave
COPD.
Pharmacotherapy
Diureticsareusedtodecreasetheelevatedrightventricular(RV)fillingvolumeinpatientswithchronic
corpulmonale.Calciumchannelblockersarepulmonaryarteryvasodilatorsthathavesomeefficacyin
thelongtermmanagementofchroniccorpulmonalesecondarytoprimarypulmonaryarterial
hypertension(PAH).[23]
USFoodandDrugAdministration(FDA)approvedprostacyclinanaloguesandendothelinreceptor
antagonistsareavailablefortreatmentofpulmonaryarterialhypertension(PAH).Thebeneficialroleof
cardiacglycosides,namelydigitalis,onthefailingrightventriclearecontroversialtheseagentsmay
improveRVfunctionbutmustbeusedwithcautionandshouldbeavoidedduringacuteepisodesof
hypoxia.
Themainindicationfororalanticoagulantsinthemanagementofcorpulmonaleisinthesettingofan
underlyingthromboemboliceventorPAH.
Methylxanthines,liketheophylline,canbeusedasanadjunctivetreatmentforchroniccorpulmonale
secondarytochronicobstructivepulmonarydisease(COPD).Besidesthemoderatebronchodilatory
effectofmethylxanthine,thisagentimprovesmyocardialcontractility,causesamildpulmonary
vasodilatoryeffect,andenhancesdiaphragmaticcontractility.
Diureticagents
Diureticsareusedinthemanagementofchroniccorpulmonale,particularlywhentheRVfilling
volumeismarkedlyelevatedandinthemanagementofassociatedperipheraledema.Theseagents
mayresultinimprovementofthefunctionofboththerightandleftventricleshowever,diureticsmay
producehemodynamicadverseeffectsiftheyarenotusedcautiously.Excessivevolumedepletion
canleadtoadeclineincardiacoutput.
Anotherpotentialcomplicationofdiuresisistheproductionofahypokalemicmetabolicalkalosis,
whichdiminishestheeffectivenessofcarbondioxidestimulationontherespiratorycentersand
lessensventilatorydrive.Theadverseelectrolyteandacidbaseeffectofdiureticusecanalsoleadto
cardiacarrhythmia,whichcandiminishcardiacoutput.Therefore,diuresis,whilerecommendedinthe
managementofchroniccorpulmonale,needstobeusedwithgreatcaution.
Vasodilatordrugs
Vasodilatorshavebeenadvocatedinthelongtermmanagementofchroniccorpulmonalewith
modestresults.Calciumchannelblockers,particularlyoralsustainedreleasenifedipine[24]and
diltiazem,canlowerpulmonarypressures,althoughtheseagentsappearmoreeffectiveinprimary
ratherthansecondarypulmonaryhypertension.[25]
Otherclassesofvasodilators,suchasbetaagonists,nitrates,andangiotensinconvertingenzyme
(ACE)inhibitorshavebeentriedbut,ingeneral,vasodilatorshavefailedtoshowsustainedbenefitin
patientswithCOPD,andtheyarenotroutinelyused.Atrialofvasodilatortherapymaybeconsidered
onlyinpatientswithCOPDwithdisproportionatelyhighpulmonaryhypertension.
Betaselectiveagonistdrugs
Betaselectiveagonistshaveanadditionaladvantageofbronchodilatorandmucociliaryclearance
effect.Rightheartcatheterizationhasbeenrecommendedduringinitialadministrationofvasodilators
toobjectivelyassesstheefficacyanddetectthepossibleadversehemodynamicconsequencesof
vasodilators.
Prostacyclinanaloguesandrecepteragonists
Epoprostenol,treprostinil,andbosentanareprostacyclin(PGI2)analoguesandhavepotent
vasodilatoryproperties.[26]Epoprostenolisadministeredintravenously(IV).Treprostinilcanbe
administeredIVandsubcutaneously(SC)theFDAhasapprovedoralandinhaled
formulations.Iloprostiscommonlyinhaledbutrequiresfrequentdosing.
Oftheseprostacyclinanalogues,epoprostenolhasbeenthemoststudiedithasbeenshownto
improvesurvivalinidiopathicpulmonaryarterialhypertensionaswellassomebenefitinothertypesof
WorldHealthOrganization(WHO)classificationgroup1pulmonaryhypertension,particularlyin
patientswithmoreseverefunctionalstatus.[27]
Selexipagisaprostacyclinreceptoragonist,whichactstovasodilatethepulmonaryvasculature.Itis
administeredorallyandhasbeenshowntoreducediseaseprogressioninPAH.[28]
Endothelinreceptorantagonists
BosentanandmacitentanaremixedendothelinAandendothelinBreceptorantagonists,whereas
ambrisentanisaselectiveendothelinAreceptorantagonist.Endothelinsarepeptidesthatactvia
vasoconstrictionthus,endotelinreceptorantagonistsindicatedresultinsubsequentvasodilation.In
clinicaltrials,bosentanimprovedexercisecapacity,decreasedrateofclinicaldeterioration,and
improvedhemodynamics.[26]
Theendothelinreceptorantagonistsareindicatedinidiopathicpulmonaryarteryhypertensionaswell
aspulmonaryhypertensionsecondarytoconnectivetissuedisorders(groupIpulmonary
hypertension).Commonsideeffectsincludeelevatedliverfunctiontestfindings.
Phosphodiesterasetype5(PDE5)inhibitors
ThePDE5inhibitorsfunctionbypreventingthedegradationofcyclicGMPandsubsequently
prolongingthevasodilatoryeffectofnitricoxide.Ofthese,sildenafilhasbeenintensivelystudied[29,
30,31] andwasapprovedbytheFDAfortreatmentofpulmonaryhypertension.Sildenafilpromotes
selectivesmoothmusclerelaxationinlungvasculature.[32]TadalafilandvardenafilareotherPDE5
inhibitorsalsoapprovedbytheFDAforthetreatmentofPAHtoimproveexerciseability.[33]
Therearenotenoughdataavailableyetregardingtheefficacyofthesedrugsinpatientswith
secondarypulmonaryhypertension,suchasinpatientswithCOPD.
Guanylatecyclasestimulants
Riociguatisasolubleguanylatecyclasestimulantthatmimicsthefunctionofnitricoxideaswellas
actssynergisticallywithittopromotevasodilation.Unlikeotheradvancedtherapies,riociguathas
beenFDAapprovedforthetreatmentofgroupIpulmonaryhyprtensionaswellasgroup4pulmonary
hypertension(chronicthromboembolicpulmonaryhypertension).Itwasshowntoimproveexercise
toleranceaswellasreducesymptoms.[34]
Cardiacglycosideagents
Theuseofcardiacglycosides,suchasdigitalis,inpatientswithcorpulmonalehasbeencontroversial,
andthebeneficialeffectofthesedrugsisnotasobviousasinthesettingofleftheartfailure.
Nevertheless,studieshaveconfirmedamodesteffectofdigitalisonthefailingrightventriclein
patientswithchroniccorpulmonale.[35]Thisdrugmustbeusedcautiously,however,andshouldnot
beusedduringtheacutephasesofrespiratoryinsufficiencywhenlargefluctuationsinlevelsof
hypoxiaandacidosismayoccur.Patientswithhypoxemiaoracidosisareatincreasedriskof
developingarrhythmiasduetodigitalisthroughdifferentmechanisms,includingsympathoadrenal
stimulation.
Theophylline
Inadditiontobronchodilatoryeffects,theophyllinehasbeenreportedtoreducepulmonaryvascular
resistanceandpulmonaryarterialpressuresacutelyinpatientswithchroniccorpulmonalesecondary
toCOPD.[36]Theophyllinehasaweakinotropiceffectandthusmayimproverightandleftventricular
ejection.Lowdosesoftheophyllinehavealsobeensuggestedtohaveantiinflammatoryeffectsthat
helptocontrolunderlyinglungdiseasessuchasCOPD.[37]Asaresult,consideringtheuseof
theophyllineasadjunctivetherapyinthemanagementofchronicordecompensatedcorpulmonaleis
reasonableinpatientswithunderlyingCOPD.Theophyllinehasanarrowtherapeuticindex,and
adverseeffectsincludeseizures,tachycardia,andothercardiacarrhythmias.
Warfarin
Anticoagulationwithwarfarinisrecommendedinpatientsathighriskforthromboembolism.The
beneficialroleofanticoagulationinimprovingthesymptomsandmortalityinpatientswithprimary
PAHhasbeendemonstratedinseveralstudies.[38,39,40]Theevidenceofbenefit,however,hasnot
beenestablishedinpatientswithsecondaryPAH.Therefore,anticoagulationtherapymaybeusedin
patientswithcorpulmonalesecondarytothromboembolicphenomenaandwithunderlyingprimary
PAH.
Thrombolytictherapy
Thrombolytictherapyisindicatedinpatientswithacutecorpulmonaleduetoapulmonaryembolism
resultinginhemodynamicinstability.Insomecases,thrombolytictherapymaybeindicatedinpatients
withsevereRVdysfunctionwithoutresultanthypotensiontopreventfurtherdecompensation.[41]
Thrombolyticagents,includingtissueplasminogenactivator(tPA),resultinacceleratedlysisofclots
andcanbeadministeredsystemicallyorviaacatheter.Asalways,theriskofbleedingmustbea
strongconsiderationwhenusingthrombolytictherapy.
SurgicalManagementofCorPulmonale
Phlebotomyisindicatedinpatientswithchroniccorpulmonaleandchronichypoxiacausingsevere
polycythemia,definedashematocritof65%ormore.Phlebotomyresultsinadecreaseinmean
pulmonaryarterypressure,adecreaseinmeanpulmonaryvascularresistance,[42]andan
improvementinexerciseperformanceinsuchpatients.However,noevidencesuggestsimprovement
insurvival.
Generally,phlebotomyshouldbereservedasanadjunctivetherapyforpatientswithacute
decompensationofcorpulmonaleandpatientswhoremainsignificantlypolycythemicdespite
appropriatelongtermoxygentherapy.Replacementoftheacutevolumelosswithasalineinfusion
maybenecessarytoavoidimportantdecreasesinsystemicbloodpressure.
Uvulopalatopharyngoplasty(UPPP)inselectedpatientswithsleepapneaandhypoventilationmay
relievecorpulmonale.[43]
Pulmonaryembolectomyisindicatedinpatientswithacutepulmonaryembolismandhemodynamic
instabilitywhenthrombolytictherapyiscontraindicated.Catheterdirectedembolectomycanbe
accomplishedwithavarietyofmodalities,includingsuctionembolectomy,rotationalembolectomy,
andrheolyticembolectomy,whichinvolvestheinjectionofpressuredsalineandconcurrentaspiration
ofthemaceratedthrombus.
Surgicalembolectomymaybealsobeindicatedinsimilarpatientsorinpatientswhoseprevious
thrombolytictherapyfailed,particularlyifthelocationofthethrombusisinamoreproximallocation.
Singlelung,doublelung,andheartlungtransplantationareallusedtosalvagetheterminalphasesof
severaldiseases(eg,PPH,emphysema,idiopathicpulmonaryfibrosis,cysticfibrosis)complicatedby
corpulmonale.Lungtransplantationmayleadtoareversalofrightventriculardysfunctionfromthe
chronicstressofpulmonaryhypertension.However,strictselectioncriteriaforlungtransplant
recipientsmustbemetbecauseofthelimitedavailabilityoforgandonors.
OutpatientMonitoring
Patientswithcorpulmonalegenerallyrequirecloseattentionintheoutpatientsetting.Itisappropriate
toregularlyassessthepatientsoxygenneedsandpulmonaryfunction.Consideraformalprogramof
pulmonaryrehabilitation,asmanypatientsbenefitfromthistherapy.
PrognosisofCorPulmonale
Theprognosisofcorpulmonaleisvariabledependingupontheunderlyingpathology.Developmentof
corpulmonaleasaresultofaprimarypulmonarydiseaseusuallyheraldsapoorerprognosis.For
example,patientswithchronicobstructivepulmonarydisease(COPD)whodevelopcorpulmonale
havea30%chanceofsurviving5years.However,whethercorpulmonalecarriesanindependent
prognosticvalueorissimplyreflectingtheseverityofunderlyingCOPDorotherpulmonarydiseaseis
notclear.
Prognosisintheacutesettingduetomassivepulmonaryembolismoracuterespiratorydistress
syndrome(ARDS)hasnotpreviouslybeenshowntobedependentonthepresenceorabsenceofcor
pulmonale.However,aprospective,multicentercohortstudybyVolschanetalindicatedthatincases
ofpulmonaryembolism,corpulmonalemaybeapredictorofinhospitalmortality.[44]Theauthors
collecteddemographic,comorbidity,andclinicalmanifestationdataon582patientsadmittedto
emergencyorintensivecareunitsanddiagnosedwithpulmonaryembolism.Assessingthe
informationusinglogisticregressionanalysis,theinvestigatorsbuiltapredictionmodel.Theirresults
indicatedthatinhemodynamicallystablepatientswithpulmonaryembolism,thefollowingfactorsmay
beindependentpredictorsofinhospitalmortality[44]:
Ageolderthan65years
Bedrestforlongerthan72hours
Chroniccorpulmonale
Sinustachycardia
Tachypnea
AChinesestudyindicatedthatchroniccorpulmonaleisoneofthemajorriskfactorsforearlyhospital
readmissioninpatientsfollowinghospitalizationforacuteexacerbationofCOPD.Thestudy,byLinet
al,of692patients,included63patientswhowerereadmittedtothehospitalwithin31daysafter
discharge.Throughmultivariateanalysis,theinvestigatorsfoundthatriskfactorsforearlyreadmission
included,inorderofsignificance,chroniccorpulmonale(oddsratio[OR],2.14),hypoproteinemia(OR,
2.02),andanelevatedpartialpressureofCO2(PaCO2[OR,1.03]).[45]
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MediaGallery
ThisECGshowssometypicalabnormalitiesthatmaybeseenincorpulmonaleandother
chronicpulmonarydiseases:(1)R/Sratio>1inV1and<1inV6suggestiveofrightventricular
hypertrophy/enlargement,(2)rightsuperioraxisdeviation,(3)leftatrialtypeofpwavewith
increasedwidthofthepwaveandbiphasicpwaveinV1,and(4)rightbundlebranchblock
patternwithwideQRSandRsR1patterninV1andslurredswaveinV6.ThisECGalsopresents
asinusbradycardiarhythmwithfirstdegreeAVblockandleftanteriorfascicularblock.
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ContributorInformationandDisclosures
Author
DerekLeong,MDResidentPhysician,DepartmentofInternalMedicine,CedarsSinaiMedicalCenter
Disclosure:Nothingtodisclose.
Coauthor(s)
RaviHDave,MDAssociateProfessorofMedicine,UniversityofCaliforniaatLosAngelesDavid
GeffenSchoolofMedicine
AbrahamGKocheril,MD,FACC,FACP,FHRSProfessorofMedicine,UniversityofIllinoisCollege
ofMedicine
AbrahamGKocheril,MD,FACC,FACP,FHRSisamemberofthefollowingmedicalsocieties:
AmericanCollegeofCardiology,CentralSocietyforClinicalandTranslationalResearch,HeartFailure
SocietyofAmerica,CardiacElectrophysiologySociety,AmericanCollegeofPhysicians,American
HeartAssociation,AmericanMedicalAssociation,IllinoisStateMedicalSociety
AliASovari,MD,FACP,FACCAttendingPhysician,CardiacElectrophysiologist,CedarsSinai
MedicalCenterandStJohn'sRegionalMedicalCenter
AliASovari,MD,FACP,FACCisamemberofthefollowingmedicalsocieties:AmericanCollegeof
Cardiology,AmericanCollegeofPhysicians,AmericanPhysicianScientistsAssociation,American
PhysiologicalSociety,BiophysicalSociety,HeartRhythmSociety,SocietyforCardiovascular
MagneticResonance

SpecialtyEditorBoard
FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedical
CenterCollegeofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:ReceivedsalaryfromMedscapeforemployment.for:Medscape.
StevenJCompton,MD,FACC,FACP,FHRSDirectorofCardiacElectrophysiology,AlaskaHeart
Institute,ProvidenceandAlaskaRegionalHospitals
StevenJCompton,MD,FACC,FACP,FHRSisamemberofthefollowingmedicalsocieties:
AmericanCollegeofPhysicians,AmericanHeartAssociation,AmericanMedicalAssociation,Heart
RhythmSociety,AlaskaStateMedicalAssociation,AmericanCollegeofCardiology
ChiefEditor
HenryHOoi,MD,MRCPIDirector,AdvancedHeartFailureandCardiacTransplantProgram,
NashvilleVeteransAffairsMedicalCenterAssistantProfessorofMedicine,VanderbiltUniversity
SchoolofMedicine

Cor Pulmonale - Introduction To Cor Pulmonale, Etiology and Pathophysiology of Cor Pulmonale, Epidemiology of Cor Pulmonale

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