1、New Classification of Pulmonary Vascular DiseaseTreatment Based Classificationv1.Pulmonary Arterial Hypertensionv2.Pulmonary Venous Hypertensionv3.Associated with Disorders of the Respiratory System and/or Hypoxemiav4.Due to Chronic Thrombotic and/or Embolic Disease.“Inclusive”New Classification of
2、Pulmonary HypertensionPulmonary Arterial HypertensionDisorders of the Respiratory SystemPulmonary Venous Hypertension.“Exclusive”Old Classification ofPulmonary HypertensionPPHSPH.Pulmonary Vasuclar Disease:Talk OutlinevEstablishing the EtiologyvInitial Approach to TreatmentvLong-term Management.Rx:F
3、irst Treat the Underlying Disease(1)Disorders of the Respiratory SystemBronchodialatorsOxygenSteriods and ImmunosupressionPulmonary Venous HypertensionAfterload ReductionMitral Valve Surgery.Treatment of Pulmonary Vascular DiseaseTreat theUnderlying Disease.Suspected Pulmonary HypertensionLeft Heart
4、 Disease ECHOValvular Heart DiseaseCongenital Heart DiseaseElectrocardiogramEmphysemaPulmonary Fibrosis Chest X-RayCystic FibrosisThoracic Cage Abnormalities PFTsSleep Disordered Breathing Sleep StudyChronic Thromboembolic Disease V/Q Scan +/-AngiogramLupus ANASchleroderma Ph FRheumatoid ArthritisHI
5、V Infection HIVPulmonary HypertensionLiver FunctionPrimary Pulmonary Hypertension.Work-Up of Pulmonary HypertensionPulmonary Arterial HypertensionPulmonary Venous HypertensionDisorders of the Respiratory System.Right Heart Catheterizationv1.Diagnosisv2.Determine Prognosisv3.Evaluate Therapy.Pathogen
6、esis of Pulmonary Vascular LesionINSULTINJURYSusceptibilityVascular Lesion.Pulmonary Arterial Hypertensionv1.1 Primary Pulmonary Hypertension(a)Sporadic(b)Familialv1.2 Related to:(a)Collagen Vascular Disease(b)Congenital Shunts(c)Portal Hypertension(d)HIV Infection(e)Drugs/Toxins.Rx:First Treat the
7、Underlying Disease(2)Thromboembolic DiseaseAnticoagulationIVC FilterThromboendarterectomy Surgery.A Positive Acute Vasodilator Responsev A reduction in mean pulmonary artery pressure of 10 mmHg associated with either no change or an increase in cardiac output.Executive Summary:World Symposium-Primar
8、y Pulmonary Hypertension 1998.The Vasodilator Trial No role for calcium channel blockers.Vasodilator Management:Response to TherapyTimePA PressureReversibleIrreversible.The Paradox of Epoprostenol Therapy vPatients who do not respond to Epoprostenol in acute vasodilator testing do respond to chronic
9、 therapy with Epoprostenol.Choosing a Calcium Channel BlockervAmlodipinevDiltiazemvNifedipine.Right Heart Catheterization and Vasodilator TrialAcute ResponderNon-ResponderCalcium Channel BlockerNYHA II-IVEproprostenol.Epoprostenol Side EffectsvDrug DeliveryvLine ComplicationsvHeadache,Jaw pain,Arthr
10、algia,Neuropathy,Weight loss,Diarrhea,ThrombocytopeniavCost.Continuous Intravenous EpoprostenolvIndicationsvInitiation of TherapyvChronic ManagementvSide Effects.Adjunct Therapy in Pulmonary Vascular DiseasevSupplemental OxygenReduced cardiac output,patent foramen ovale,V/Q mismatchingvDiureticsvCar
11、diac GlycosidesvAtrial SeptostomyvIntravenous Inotropes.Treatment in Pulmonary Vascular Disease1980Ca+Blockers1990Epoprostenol2000VascularRemodeling?.Natural History of the Response to EpoprostenolPre-PGIEarly PGILong-TermPGINHYA432A.B.C.Intravenous Vasodilators in Pulmonary HypertensionPulmonary Ar
12、terialRespiratory DiseasePulmonary VenousIncreased ShuntPulmonary Edema.Vasodilator Management:Response to TherapyTimePA PressureReversibleReversible?(Vasoconstriction)(Proliferation).Monitoring the Effects of Chronic Therapyv NoninvasiveSigns and symptoms/NYHA classificationExercise testing/six min
13、ute walkEchocardiographyv InvasiveHemodynamic measurements.Right Heart Catheterization and Vasodilator TrialImprovedAcute ResponderNon-ResponderCalcium Channel BlockerNYHA II-IVEproprostenolNot ImprovedRecurrent syncope&/or RHFTransplantAtrial Septostomy.Transplantation for Pulmonary Hypertension:Ro
14、le of EpoprostenolvBridge to TransplantationvDefer the need for TransplantationvAlternative to Transplantation long-termv?Bridge to newer Therapies.Portopulmonary Hypertension:Prostacyclin TherapyN=443.7+2.8 yrsMean PGI2=10 mthsDose(ng/kg/min)=24+5TPR(mmHg/L/min)Dose(ng/kg/min)BaselineOne Year151311
15、975.Eisenmengers:Prostacyclin TherapyN=240+98 yrsMean PGI2=16 mthsTPR(mmHg/L/min)BaselineOne Year161412108642.EpoprostenolNot ImprovedImprovedNot ImprovedRecurrent syncope&/or RHFTransplantAtrial SeptostomyIncrease PGI2 Dose.EpoprostenolContinued ImprovementImprovedNot ImprovedList for TransplantDis
16、improvedTransplantDeactivate.The Influence of Epoprostenol on the Timing and Outcome of TransplantationConte et al.The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation.J Heart Lung Transplant 1998 Jul;17(7):679-8
17、5.42 Transplant EvaluationRejected 537 Transplant Candidates22 University of MarylandOther Transplant Programs 1512 At BothTransplanted8Off active list8Died Waiting2Waiting4Transplanted5Off active list3Died Waiting1Waiting67 Alive3 Alive.Medical Management of Pulmonary Vascular Disease:SummaryvNew treatment based classificationvFirst treat the underlying diseasevVasodilator trial helps define treatment strategy and prognosisvEpoprostenol may defer transplantation indefinitelyvNew treatment will focus on vascular remodeling.
