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NewClassificationofPulmonaryVascularDiseaseTreatmentBasedClassification1.PulmonaryArterialHypertension2.PulmonaryVenousHypertension3.AssociatedwithDisordersoftheRespiratorySystemand/orHypoxemia4.DuetoChronicThromboticand/orEmbolicDisease.NewClassificationofPulmonar1“Inclusive”NewClassificationofPulmonaryHypertensionPulmonaryArterialHypertensionDisordersoftheRespiratorySystemPulmonaryVenousHypertension.“Inclusive”NewClassification2“Exclusive”O(jiān)ldClassificationofPulmonaryHypertensionPPHSPH.“Exclusive”O(jiān)ldClassification3PulmonaryVasuclarDisease:

TalkOutlineEstablishingtheEtiologyInitialApproachtoTreatmentLong-termManagement.PulmonaryVasuclarDisease:

Ta4Rx:FirstTreattheUnderlyingDisease(1)DisordersoftheRespiratorySystem Bronchodialators Oxygen SteriodsandImmunosupressionPulmonaryVenousHypertension AfterloadReduction MitralValveSurgery.Rx:FirstTreattheUnderlying5TreatmentofPulmonaryVascularDiseaseTreattheUnderlyingDisease.TreatmentofPulmonaryVascula6SuspectedPulmonaryHypertensionLeftHeartDisease ECHOValvularHeartDisease

CongenitalHeartDisease ElectrocardiogramEmphysemaPulmonaryFibrosis ChestX-RayCysticFibrosisThoracicCageAbnormalities PFTsSleepDisorderedBreathing SleepStudyChronicThromboembolicDisease V/QScan +/-AngiogramLupus ANASchleroderma PhFRheumatoidArthritis

HIVInfection HIVPulmonaryHypertension LiverFunctionPrimaryPulmonaryHypertension.SuspectedPulmonaryHypertensi7Work-UpofPulmonaryHypertensionPulmonaryArterialHypertensionPulmonaryVenousHypertensionDisordersoftheRespiratorySystem.Work-UpofPulmonaryHypertens8RightHeartCatheterization1.Diagnosis2.DeterminePrognosis3.EvaluateTherapy.RightHeartCatheterization.9PathogenesisofPulmonaryVascularLesionINSULTINJURYSusceptibilityVascularLesion.PathogenesisofPulmonaryVasc10PulmonaryArterialHypertension1.1PrimaryPulmonaryHypertension (a)Sporadic (b)Familial1.2Relatedto: (a)CollagenVascularDisease (b)CongenitalShunts (c)PortalHypertension (d)HIVInfection (e)Drugs/Toxins.PulmonaryArterialHypertensio11Rx:FirstTreattheUnderlyingDisease(2)

ThromboembolicDisease Anticoagulation IVCFilter ThromboendarterectomySurgery.Rx:FirstTreattheUnderlying12APositiveAcuteVasodilatorResponseAreductioninmeanpulmonaryarterypressureof10mmHgassociatedwitheithernochangeoranincreaseincardiacoutput.ExecutiveSummary:WorldSymposium-PrimaryPulmonaryHypertension1998.APositiveAcuteVasodilatorR13TheVasodilatorTrialNoroleforcalciumchannelblockers.TheVasodilatorTrialNorolef14VasodilatorManagement:

ResponsetoTherapyTimePAPressureReversibleIrreversible.VasodilatorManagement:

Respon15TheParadoxofEpoprostenolTherapyPatientswhodonotrespondtoEpoprostenolinacutevasodilatortestingdorespondtochronictherapywithEpoprostenol..TheParadoxofEpoprostenolTh16ChoosingaCalciumChannelBlockerAmlodipineDiltiazemNifedipine.ChoosingaCalciumChannelBlo17RightHeartCatheterizationandVasodilatorTrialAcuteResponderNon-ResponderCalciumChannelBlockerNYHAII-IVEproprostenol.RightHeartCatheterizationan18EpoprostenolSideEffectsDrugDeliveryLineComplicationsHeadache,Jawpain,Arthralgia,Neuropathy,Weightloss,Diarrhea,ThrombocytopeniaCost.EpoprostenolSideEffectsDrug19ContinuousIntravenousEpoprostenolIndicationsInitiationofTherapyChronicManagementSideEffects.ContinuousIntravenousEpopros20AdjunctTherapyinPulmonaryVascularDiseaseSupplementalOxygenReducedcardiacoutput,patentforamenovale,V/QmismatchingDiureticsCardiacGlycosidesAtrialSeptostomyIntravenousInotropes.AdjunctTherapyinPulmonaryV21TreatmentinPulmonaryVascularDisease1980Ca++Blockers1990Epoprostenol2000VascularRemodeling?.TreatmentinPulmonaryVascula22NaturalHistoryoftheResponsetoEpoprostenolPre-PGI EarlyPGI Long-Term PGINHYA432A.B.C..NaturalHistoryoftheRespons23IntravenousVasodilatorsinPulmonaryHypertensionPulmonaryArterialRespiratoryDiseasePulmonaryVenousIncreasedShuntPulmonaryEdema.IntravenousVasodilatorsinPu24VasodilatorManagement:

ResponsetoTherapyTimePAPressureReversibleReversible?(Vasoconstriction)(Proliferation).VasodilatorManagement:

Respon25MonitoringtheEffectsofChronicTherapyNoninvasiveSignsandsymptoms/NYHAclassificationExercisetesting/sixminutewalkEchocardiographyInvasiveHemodynamicmeasurements.MonitoringtheEffectsofChro26RightHeartCatheterizationandVasodilatorTrialImprovedAcuteResponderNon-ResponderCalciumChannelBlockerNYHAII-IVEproprostenolNotImprovedRecurrentsyncope&/orRHFTransplantAtrialSeptostomy.RightHeartCatheterizationan27TransplantationforPulmonaryHypertension:RoleofEpoprostenolBridgetoTransplantationDefertheneedforTransplantationAlternativetoTransplantationlong-term?BridgetonewerTherapies.TransplantationforPulmonary28PortopulmonaryHypertension:ProstacyclinTherapyN=443.7+2.8yrsMeanPGI2=10mthsDose(ng/kg/min)=24+5TPR(mmHg/L/min)Dose(ng/kg/min)BaselineOneYear151311975.PortopulmonaryHypertension:P29Eisenmengers:

ProstacyclinTherapyN=240+98yrsMeanPGI2=16mthsTPR(mmHg/L/min)BaselineOneYear161412108642.Eisenmengers:

ProstacyclinThe30EpoprostenolNotImprovedImprovedNotImprovedRecurrentsyncope&/orRHFTransplantAtrialSeptostomyIncreasePGI2Dose.EpoprostenolNotImprovedImprov31EpoprostenolContinuedImprovementImprovedNotImprovedListforTransplantDisimprovedTransplantDeactivate.EpoprostenolContinuedImprovem32TheInfluenceofEpoprostenolontheTimingandOutcomeofTransplantationConteetal.Theinfluenceofcontinuousintravenousprostacyclintherapyforprimarypulmonaryhypertensiononthetimingandoutcomeoftransplantation..

JHeartLungTransplant1998Jul;17(7):679-85.42TransplantEvaluationRejected537TransplantCandidates22UniversityofMarylandOtherTransplantPrograms1512AtBothTransplanted8Offactivelist8DiedWaiting2Waiting4Transplanted5Offactivelist3DiedWaiting1Waiting67Alive3Alive.TheInfluenceofEpoprostenol33MedicalManagementofPulmonaryVascularDisease:SummaryNewtreatmentbasedclassificationFirsttreattheunderlyingdiseaseVasodilatortrialhelpsdefinetreatmentstrategyandprognosisEpoprostenolmaydefertransplantationindefinitelyNewtreatmentwillfocusonvascularremodeling.MedicalManagementofPulmonar34NewClassificationofPulmonaryVascularDiseaseTreatmentBasedClassification1.PulmonaryArterialHypertension2.PulmonaryVenousHypertension3.AssociatedwithDisordersoftheRespiratorySystemand/orHypoxemia4.DuetoChronicThromboticand/orEmbolicDisease.NewClassificationofPulmonar35“Inclusive”NewClassificationofPulmonaryHypertensionPulmonaryArterialHypertensionDisordersoftheRespiratorySystemPulmonaryVenousHypertension.“Inclusive”NewClassification36“Exclusive”O(jiān)ldClassificationofPulmonaryHypertensionPPHSPH.“Exclusive”O(jiān)ldClassification37PulmonaryVasuclarDisease:

TalkOutlineEstablishingtheEtiologyInitialApproachtoTreatmentLong-termManagement.PulmonaryVasuclarDisease:

Ta38Rx:FirstTreattheUnderlyingDisease(1)DisordersoftheRespiratorySystem Bronchodialators Oxygen SteriodsandImmunosupressionPulmonaryVenousHypertension AfterloadReduction MitralValveSurgery.Rx:FirstTreattheUnderlying39TreatmentofPulmonaryVascularDiseaseTreattheUnderlyingDisease.TreatmentofPulmonaryVascula40SuspectedPulmonaryHypertensionLeftHeartDisease ECHOValvularHeartDisease

CongenitalHeartDisease ElectrocardiogramEmphysemaPulmonaryFibrosis ChestX-RayCysticFibrosisThoracicCageAbnormalities PFTsSleepDisorderedBreathing SleepStudyChronicThromboembolicDisease V/QScan +/-AngiogramLupus ANASchleroderma PhFRheumatoidArthritis

HIVInfection HIVPulmonaryHypertension LiverFunctionPrimaryPulmonaryHypertension.SuspectedPulmonaryHypertensi41Work-UpofPulmonaryHypertensionPulmonaryArterialHypertensionPulmonaryVenousHypertensionDisordersoftheRespiratorySystem.Work-UpofPulmonaryHypertens42RightHeartCatheterization1.Diagnosis2.DeterminePrognosis3.EvaluateTherapy.RightHeartCatheterization.43PathogenesisofPulmonaryVascularLesionINSULTINJURYSusceptibilityVascularLesion.PathogenesisofPulmonaryVasc44PulmonaryArterialHypertension1.1PrimaryPulmonaryHypertension (a)Sporadic (b)Familial1.2Relatedto: (a)CollagenVascularDisease (b)CongenitalShunts (c)PortalHypertension (d)HIVInfection (e)Drugs/Toxins.PulmonaryArterialHypertensio45Rx:FirstTreattheUnderlyingDisease(2)

ThromboembolicDisease Anticoagulation IVCFilter ThromboendarterectomySurgery.Rx:FirstTreattheUnderlying46APositiveAcuteVasodilatorResponseAreductioninmeanpulmonaryarterypressureof10mmHgassociatedwitheithernochangeoranincreaseincardiacoutput.ExecutiveSummary:WorldSymposium-PrimaryPulmonaryHypertension1998.APositiveAcuteVasodilatorR47TheVasodilatorTrialNoroleforcalciumchannelblockers.TheVasodilatorTrialNorolef48VasodilatorManagement:

ResponsetoTherapyTimePAPressureReversibleIrreversible.VasodilatorManagement:

Respon49TheParadoxofEpoprostenolTherapyPatientswhodonotrespondtoEpoprostenolinacutevasodilatortestingdorespondtochronictherapywithEpoprostenol..TheParadoxofEpoprostenolTh50ChoosingaCalciumChannelBlockerAmlodipineDiltiazemNifedipine.ChoosingaCalciumChannelBlo51RightHeartCatheterizationandVasodilatorTrialAcuteResponderNon-ResponderCalciumChannelBlockerNYHAII-IVEproprostenol.RightHeartCatheterizationan52EpoprostenolSideEffectsDrugDeliveryLineComplicationsHeadache,Jawpain,Arthralgia,Neuropathy,Weightloss,Diarrhea,ThrombocytopeniaCost.EpoprostenolSideEffectsDrug53ContinuousIntravenousEpoprostenolIndicationsInitiationofTherapyChronicManagementSideEffects.ContinuousIntravenousEpopros54AdjunctTherapyinPulmonaryVascularDiseaseSupplementalOxygenReducedcardiacoutput,patentforamenovale,V/QmismatchingDiureticsCardiacGlycosidesAtrialSeptostomyIntravenousInotropes.AdjunctTherapyinPulmonaryV55TreatmentinPulmonaryVascularDisease1980Ca++Blockers1990Epoprostenol2000VascularRemodeling?.TreatmentinPulmonaryVascula56NaturalHistoryoftheResponsetoEpoprostenolPre-PGI EarlyPGI Long-Term PGINHYA432A.B.C..NaturalHistoryoftheRespons57IntravenousVasodilatorsinPulmonaryHypertensionPulmonaryArterialRespiratoryDiseasePulmonaryVenousIncreasedShuntPulmonaryEdema.IntravenousVasodilatorsinPu58VasodilatorManagement:

ResponsetoTherapyTimePAPressureReversibleReversible?(Vasoconstriction)(Proliferation).VasodilatorManagement:

Respon59MonitoringtheEffectsofChronicTherapyNoninvasiveSignsandsymptoms/NYHAclassificationExercisetesting/sixminutewalkEchocardiographyInvasiveHemodynamicmeasurements.MonitoringtheEffectsofChro60RightHeartCatheterizationandVasodilatorTrialImprovedAcuteResponderNon-ResponderCalciumChannelBlockerNYHAII-IVEproprostenolNotImprovedRecurrentsyncope&/orRHFTransplantAtrialSeptostomy.RightHeartCatheterizationan61TransplantationforPulmonaryHypertension:RoleofEpoprostenolBridgetoTransplantationDefertheneedforTransplantationAlternativetoTransplantationlong-term?BridgetonewerTherapies.TransplantationforPulmonary62PortopulmonaryHypertension:ProstacyclinTherapyN=443.7+2.8yrsMeanPGI2=10mthsDose(ng/kg/min)=24+5TPR(mmHg/L/min)Dose(ng/kg/min)BaselineOneYear151311975.PortopulmonaryHypertension:

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