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New Classification of Pulmonary Vascular Disease,Treatment Based Classification 1. Pulmonary Arterial Hypertension 2. Pulmonary Venous Hypertension 3. Associated with Disorders of the Respiratory System and/or Hypoxemia 4. Due to Chronic Thrombotic and/or Embolic Disease,“Inclusive” New Classification of Pulmonary Hypertension,Pulmonary Arterial Hypertension,Disorders of the Respiratory System,Pulmonary Venous Hypertension,“Exclusive” Old Classification of Pulmonary Hypertension,PPH,SPH,Pulmonary Vasuclar Disease: Talk Outline,Establishing the Etiology Initial Approach to Treatment Long-term Management,Rx: First Treat the Underlying Disease (1),Disorders of the Respiratory System Bronchodialators Oxygen Steriods and Immunosupression Pulmonary Venous Hypertension Afterload Reduction Mitral Valve Surgery,Treatment of Pulmonary Vascular Disease,Treat the Underlying Disease,Suspected Pulmonary Hypertension,Left Heart Disease ECHO Valvular Heart Disease Congenital Heart Disease Electrocardiogram Emphysema Pulmonary Fibrosis Chest X-Ray Cystic Fibrosis Thoracic Cage Abnormalities PFTs Sleep Disordered Breathing Sleep Study Chronic Thromboembolic Disease V/Q Scan +/- Angiogram Lupus ANA Schleroderma Ph F Rheumatoid Arthritis HIV Infection HIV Pulmonary Hypertension Liver Function,Primary Pulmonary Hypertension,Work-Up of Pulmonary Hypertension,Pulmonary Arterial Hypertension Pulmonary Venous Hypertension Disorders of the Respiratory System,Right Heart Catheterization,1. Diagnosis 2. Determine Prognosis 3. Evaluate Therapy,Pathogenesis of Pulmonary Vascular Lesion,INSULT INJURY Susceptibility Vascular Lesion,Pulmonary Arterial Hypertension,1.1 Primary Pulmonary Hypertension (a) Sporadic (b) Familial 1.2 Related to: (a) Collagen Vascular Disease (b) Congenital Shunts (c) Portal Hypertension (d) HIV Infection (e) Drugs/Toxins,Rx: First Treat the Underlying Disease (2),Thromboembolic Disease Anticoagulation IVC Filter Thromboendarterectomy Surgery,A Positive Acute Vasodilator Response,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-Primary Pulmonary Hypertension 1998,The Vasodilator Trial,No role for calcium channel blockers,Vasodilator Management: Response to Therapy,Time,PA Pressure,Reversible,Irreversible,The Paradox of Epoprostenol Therapy,Patients who do not respond to Epoprostenol in acute vasodilator testing do respond to chronic therapy with Epoprostenol.,Choosing a Calcium Channel Blocker,Amlodipine Diltiazem Nifedipine,Right Heart Catheterization and Vasodilator Trial,Acute Responder,Non-Responder,Calcium Channel Blocker,NYHA II-IV,Eproprostenol,Epoprostenol Side Effects,Drug Delivery Line Complications Headache, Jaw pain, Arthralgia, Neuropathy, Weight loss, Diarrhea, Thrombocytopenia Cost,Continuous Intravenous Epoprostenol,Indications Initiation of Therapy Chronic Management Side Effects,Adjunct Therapy in Pulmonary Vascular Disease,Supplemental Oxygen Reduced cardiac output, patent foramen ovale, V/Q mismatching Diuretics Cardiac Glycosides Atrial Septostomy Intravenous Inotropes,Treatment in Pulmonary Vascular Disease,1980 Ca+ Blockers,1990 Epoprostenol,2000 Vascular Remodeling?,Natural History of the Response to Epoprostenol,Pre-PGI Early PGI Long-Term PGI,NHYA,4,3,2,A. B. C.,Intravenous Vasodilators in Pulmonary Hypertension,Pulmonary Arterial,Respiratory Disease,Pulmonary Venous,Increased Shunt,Pulmonary Edema,Vasodilator Management: Response to Therapy,Time,PA Pressure,Reversible,Reversible?,(Vasoconstriction),(Proliferation),Monitoring the Effects of Chronic Therapy,Noninvasive Signs and symptoms/NYHA classification Exercise testing/six minute walk Echocardiography Invasive Hemodynamic measurements,Right Heart Catheterization and Vasodilator Trial,Improved,Acute Responder,Non-Responder,Calcium Channel Blocker,NYHA II-IV,Eproprostenol,Not Improved,Recurrent syncope &/or RHF,Transplant,Atrial Septostomy,Transplantation for Pulmonary Hypertension: Role of Epoprostenol,Bridge to Transplantation Defer the need for Transplantation Alternative to Transplantation long-term ? Bridge to newer Therapies,Portopulmonary Hypertension: Prostacyclin Therapy,N=4 43.7+2.8 yrs Mean PGI2=10 mths Dose (ng/kg/min)=24+5,TPR (mmHg/L/min),Dose (ng/kg/min),Baseline,One Year,15 13 11 9 7 5,Eisenmengers: Prostacyclin Therapy,N=2 40+98 yrs Mean PGI2=16 mths,TPR (mmHg/L/min),Baseline,One Year,16 14 12 10 8 6 4 2,Epoprostenol,Not Improved,Improved,Not Improved,Recurrent syncope &/or RHF,Transplant,Atrial Septostomy,Increase PGI2 Dose,Epoprostenol,Continued Improvement,Improved,Not Improved,List for Transplant,Disimproved,Transplant,Deactivate,The Influence of Epoprostenol on the Timing and Outcome of Transplantation,Conte 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):679-85.,42 Transplant Evaluation,Rejected 5,37 Transplant Candidates,22 University of Maryland,Other Transplant Programs 15,12 At Both,Transplanted 8,Off active list 8,Died Waiting 2,Waiting 4,Transplanted 5,Off active list 3,Died Waiting 1,Waiting 6,7 Alive,3 Alive,Medical Managem

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