New Classification of Pulmonary Vascular DiseaseTreatment Based Classification v1. Pulmonary Arterial Hypertension v2. Pulmonary Venous Hypertension v3. Associated with Disorders of the Respiratory System and/or Hypoxemia v4. Due to Chronic Thrombotic and/or Embolic Disease“Inclusive” New Classification of Pulmonary HypertensionPulmonary Arterial HypertensionDisorders of the Respiratory SystemPulmonary Venous Hypertension“Exclusive” Old Classification of Pulmonary HypertensionPPHSPHPulmonary Vasuclar Disease: Talk OutlinevEstablishing the Etiology vInitial Approach to Treatment vLong-term ManagementRx: First Treat the Underlying Disease (1)Disorders of the Respiratory System Bronchodialators Oxygen Steriods and Immunosupression Pulmonary Venous Hypertension Afterload Reduction Mitral Valve SurgeryTreatment of Pulmonary Vascular DiseaseTreat the Underlying DiseaseSuspected Pulmonary HypertensionLeft Heart Disease ECHO Valvular Heart Disease Congenital Heart DiseaseElectrocardiogramEmphysema Pulmonary Fibrosis Chest X-Ray Cystic Fibrosis Thoracic Cage Abnormalities PFTsSleep Disordered Breathing Sleep StudyChronic Thromboembolic Disease V/Q Scan+/- AngiogramLupus ANA Schleroderma Ph F Rheumatoid ArthritisHIV Infection HIVPulmonary HypertensionLiver FunctionPrimary Pulmonary HypertensionWork-Up of Pulmonary HypertensionPulmonary Arterial HypertensionPulmonary Venous HypertensionDisorders of the Respiratory SystemRight Heart Catheterizationv1. Diagnosis v2. Determine Prognosis v3. Evaluate TherapyPathogenesis of Pulmonary Vascular LesionINSULTINJURYSusceptibility Vascular LesionPulmonary Arterial Hypertensionv1.1 Primary Pulmonary Hypertension (a) Sporadic (b) Familial v1.2 Related to: (a) Collagen Vascular Disease (b) Congenital Shunts (c) Portal Hypertension (d) HIV Infection (e) Drugs/ToxinsRx: First Treat the Underlying Disease (2)Thromboembolic Disease Anticoagulation IVC Filter Thromboendarterectomy SurgeryA Positive Acute Vasodilator ResponsevA reduction in mean pulmonary artery pressure of 10 mmHg associated with either no change or an increase in cardiac output.Executive Summary: World Symposium- Primary Pulmonary Hypertension 1998The Vasodilator Trial No role for calcium channel blockersVasodilator Management: Response to TherapyTimePA PressureReversibleIrreversibleThe Paradox of Epoprostenol Therapy vPatients who do not respond to Epoprostenol in acute vasodilator testing do respond to chronic therapy with Epoprostenol.Choosing a Calcium Channel BlockervAmlodipine vDiltiazem vNifedipineRight Heart Catheterization and Vasodilator TrialAcute ResponderNon-ResponderCalcium Channel BlockerNYHA II-IVEproprostenolEpoprostenol Side EffectsvDrug Delivery vLine Complications vHeadache, Jaw pain, Arthralgia, Neuropathy, Weight loss, Diarrhea, Thrombocytopenia vCostContinuous Intravenous EpoprostenolvIndications vInitiation of Therapy vChronic Management vSide EffectsAdjunct Therapy in Pulmonary Vascular DiseasevSupplemental Oxygen Reduced cardiac output, patent foramen ovale, V/Q mismatching vDiuretics vCardiac Glycosides vAtrial Septostomy vIntravenous InotropesTreatment in Pulmonary Vascular Disease1980 Ca+ Blockers1990 Epoprostenol2000 Vascular Remodeling?Natural History of the Response to EpoprostenolPre-PGIEarly PGILong-Term PGINHYA432A.B.C.Intravenous Vasodilators in Pulmonary HypertensionPulmonary ArterialRespiratory DiseasePulmonary VenousIncreased ShuntPulmonary EdemaVasodilator Management: Response to TherapyTimePA PressureReversibleReversible?(Vasoconstriction)(Proliferation)Monitoring the Effects of Chronic TherapyvNoninvasive Signs and symptoms/NYHA classification Exercise testing/six minute walk Echocardiography vInvasive Hemodynamic measurementsRight Heart Catheterization and Vasodilator TrialImprov edAcute ResponderNon-ResponderCalcium Channel BlockerNYHA II-IVEproprostenolNot Improv ed Recurrent syncope 17(7):679-85.42 Transplant EvaluationRejected 537 Transplant Candidates22 University of MarylandOther Transplant Programs 1512 At BothTransplanted8Off active list8Died Waiting2Waiting4Transplanted5Off active list3Died Waiting1Waiting67 Alive3 AliveMedical Management of Pulmonary Vascular Disease: SummaryvNew treatment based classification vFirst treat the underlying disease vVasodilator trial helps define treatment strategy and prognosis vEpoprostenol may defer transplantation indefinitely vNew treatment will focus on vascular remodeling