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Diagnostic Evaluation of Pulmonary Arterial Hypertension: ACCP Guidelines

Diagnostic Evaluation of Pulmonary Arterial Hypertension: ACCP Guidelines. Lewis J. Rubin, MD, FCCP, FRCP University of California, San Diego. Lewis Rubin, MD, FCCP, Chair University of California, San Diego Steven H. Abman, MD University of Colorado Gregory S. Ahearn, MD

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Diagnostic Evaluation of Pulmonary Arterial Hypertension: ACCP Guidelines

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  1. Diagnostic Evaluation of Pulmonary Arterial Hypertension: ACCP Guidelines Lewis J. Rubin, MD, FCCP, FRCP University of California, San Diego

  2. Lewis Rubin, MD, FCCP, Chair University of California, San Diego Steven H. Abman, MD University of Colorado Gregory S. Ahearn, MD Duke University Rino Aldrighetti, PHA Rep Pulmonary Hypertension Association Charles Atwood, MD, FCCP University of Pittsburgh David B. Badesch, MD, FCCP University of Colorado Robyn J. Barst, MD, FCCP Columbia University Richard N. Channick, MD, FCCP University of California, San Diego John Conte, MD, FCCP Johns Hopkins University Ramona L. Doyle, MD, FCCP Stanford University Terry A. Fortin, MD Duke University Joe G. N. Garcia, MD, FCCP Johns Hopkins University Joseph A. Govert, MD, FCCP Duke University David Gutterman, MD, FCCP Medical College of Wisconsin Sandra Zelman Lewis, PhD American College of Chest Physicians James E. Loyd, MD, FCCP Vanderbilt University Douglas C. McCrory, MD, MHS Duke University Michael D. McGoon, MD, FCCP Mayo Clinic Vallerie V. McLaughlin, MD, FCCP University of Michigan Kenneth Presberg, MD, FCCP Medical College of Wisconsin Stuart Rich, MD, FCCP, ACC Rep Rush Presbyterian St. Luke’s Hospital Gerald Simonneau, MD Hospital Antoine Beclere Virginia Steen, MD Georgetown University John Sundy, MD Duke University Fredrick M. Wigley, MD, ACR Rep Johns Hopkins University Members of the ACCP Guidelines Committee

  3. Organizational Endorsements • American College of Chest Physicians • American College of Cardiology • American College of Rheumatology • American Heart Association • Pulmonary Hypertension Association

  4. Cardiac Catheterization • RA mean 10 mm Hg • RV 100/14 mm Hg • PA 100/40 (mean 60) mm Hg • PCW mean 12 mm Hg • LV 110/0-10 mm Hg • Ao 110/70 (mean 83) mm Hg • Cardiac Output 3.0 L/min • Room Air SaO2 82%; SvO2 50% • PVR 20 Units • No acute response to iNO

  5. Echocardiogram also demonstrated no intracardiac shunt

  6. Baseline HRCT Baseline PFTs (% Predicted) FVC 80% FEV1 80% TLC 76% DLCO 22% Arterial Blood Gases Room Air: pH 7.46 PCO2 32 PO2 51 100% O2: PO2 67 After 1 month of continuous intravenous epoprostenol therapy

  7. What Would YOU Do? • Begin oral therapy (either sildenafil or bosentan), and warfarin • Begin therapy with epoprostenol • Begin therapy with inhaled iloprost • Do something else

  8. What was Done • She was started on continuous intravenous epoprostenol and initially improved, with less dyspnea. • The dose of epoprostenol was gradually increased from 2-12 ng/kg/min • She returned 1 month later with the complaint of increased dyspnea. • SaO2 was 74%

  9. Before continuous intravenous epoprostenol therapy After 1 month of continuous intravenous epoprostenol therapy

  10. What Would YOU Do? • Add oral therapy (either sildenafil or bosentan) • Increase epoprostenol dose after diuresis • Discontinue epoprostenol and switch to another therapy • Refer for a thoracoscopic lung biopsy • Do something else

  11. What was Done • She was switched to inhaled prostanoid therapy • She stabilized but remained hypoxemic and her activity tolerance was severely impaired • She underwent lung transplantation 6 months later • What’s the Diagnosis?

  12. 1.Pulmonary arterial hypertension • Idiopathic PAH • Familial PAH • Related to: – Connective tissue diseases – HIV – Portal hypertension – Anorexigens – Congenital heart diseases • PPHN • PAH with venule/cap inv (PVOD) 3. PH with Lung Diseases/Hypoxemia • COPD • Interstitial lung diseases • Sleep-disordered breathing • Developmental abnormalities 4. PH due to chronic thrombotic and/or embolic disease • TE obstruction of proximal PA • TE obstruction of distal PA • Non thrombotic Pulm embolism 5. Miscellaneous 2.PH with left heart disease • Atrial or ventricular • Valvular Pulmonary Arterial HypertensionDiagnostic classificationJACC 2004 and Chest 2004

  13. Diagnosis of Pulmonary Hypertension Suspected Pulmonary Hypertension • Emphysema • Fibrosis • Thoracic abnl Sleep disorder • Left heart disease • Valvular/Congenital • Heart Disease Sleep Study Echocardiogram Chest X-Ray PFT’s • Scleroderma • SLE • RA • Vasculitis Ventilation- Perfusion scan, angiography Chronic Thrombo- embolism Autoantibody tests LFTs and clinical evidence of cirrhosis and portal htn HIV test HIV infection Portopulmonary Hypertension When Clinically Indicated Required

  14. Echocardiographic Appearance of PAH

  15. Tricuspid Regurgitant Jet Velocity

  16. Arcasoy et al: AJRCCM 2003; 167: 735-740

  17. Mukerjee et al: Rheumatology 2004; 43: 461-466

  18. The Chest X-ray Normal Right Heart Enlargement

  19. HRCT: Ground Glass or Septal Lines?

  20. CT AngiogramMosaic Pattern

  21. Perfusion Lung Scan

  22. CT AngiogramCentral Thrombus

  23. Pulmonary Angiography Web Filling defect Abrupt cut-off Hypoperfusion Hypoperfusion

  24. PTE: The Procedure

  25. PTE Specimen

  26. 50 40 30 Frequency Normal Range Normal Range Normal Range Normal Range 20 10 0 20 40 60 80 100 120 Mean Pulmonary Artery Pressure(mm Hg) 50 40 Frequency 30 20 10 0 1 2 3 4 5 6 7 8 Cardiac Index (L/Min/M2) Hemodynamics – NIH Registry 30 25 20 15 Frequency 10 5 0 10 20 30 40 50 60 70 80 90 Pulmonary Vascular Resistance Index(L/Min/M2) 30 20 Frequency 10 0 4 8 12 16 20 24 28 Right Atrial Pressure (mm Hg)

  27. Hemodynamic Abnormalitiesand Prognosis < 55 mmHg 85 mmHg < 10 mmHg 20 mmHg  4 L/min/m2< 2 L/min/m2 50 40 30 Median survival (months) 20 10 0 Mean PAP Mean RAP Mean CI D'Alonzo et al. Annals Int Med 1991;115:343-349

  28. Acute Testing of Vasoreactivity • Goal is to determine whether oral vasodilator therapy may be worthwhile as first-line approach • Testing should be performed in experienced setting with short-acting agent (ie, NO, PGI2, Adenosine) • Few patients who do not have PPH will respond

  29. Definition of “Responder” • Reduction of Pam to < 40 mm Hg: • With a >10% fall in Pam • And a normal CO • Without change in SAPm • PVR should be < 6 units JACC 2004: Proceedings of the Third World Symposium Chest 2004: ACCP Evidence-Based Guidelines

  30. CCB Response Rates in PAH Lee SH and Rubin LJ: J Intern Med 2005; 258: 199-215. Sitbon O et al: Circulation 2005; 111: 3105-3111.

  31. ACCP PAH GuidelinesPredictors of Poor Survival • Advanced Functional Class (A) • Poor Exercise Endurance (6MWT) (A) • Presence of a Pericardial Effusion (A) • Elevated Mean Right Atrial Pressure (A) • Reduced Cardiac Index (A) McLaughlin VV et al. CHEST, 2004

  32. ACCP PAH GuidelinesPredictors of Poor Survival • Elevated Mean Pulmonary Artery Pressure (B) • Elevated Tei index (C) • Low VO2 max, peak SBP, DBP on CPET (C) • ECG findings of RAE, RVE (C) • Elevated BNP (C) McLaughlin VV et al. CHEST, 2004

  33. Historical Mortality inClass III / IV PPH 100 Class II & IIIClass IV 80 60 Percent survival 40 20 0 0 300 600 900 1200 1500 Days

  34. Echo/Doppler endpoints • Effusion score • RA area • RVET (Tei Index) • RV EF (surrogate) • Mitral valve early filling • LV diastolic dimensions

  35. Survival correlates with BNP values in PPH Baseline BNP Follow-up BNP 100 100 BNP < 180 pg/ml 80 80 BNP < 150 pg/ml 60 60 Survival rate (%) BNP  150 pg/ml 40 40 20 20 BNP  180 pg/ml 0 0 0 12 24 36 48 0 12 24 36 48 Time (months) Time (months) Nagaya N et al. Circulation 2000;102:865-70.

  36. NYHA Functional Class Remains an Important Determinant of Survival in PPH McLaughlin, Circulation 2002

  37. Influence of 6 Minute Walk on Survival in PPH Sitbon et al, JACC 2002: 40: 780-788

  38. Maximal oxygen consumption as a determinant of survival in PPH 100 80 Peak VO2> 10.4 ml/kg/min 60 Cumulative survival (%) 40 Peak VO2 10.4 ml/kg/min 20 0 0 1 2 3 Time (years) Peak VO2> 10.4 ml/kg/min At risk 36 29 14 8 - Deaths 0 3 9 11 Peak VO2 10.4 ml/kg/min At risk 34 14 2 0 Deaths 0 16 23 23 Wensel et al. Circulation 2002; 106:319-24

  39. Peak SBP as a predictor of survivalin PPH 100 80 Peak SBP > 120 mmHg 60 Cumulative survival (%) 40 Peak SBP  120 mmHg 20 0 0 1 2 3 Time (years) Peak SBP > 120 mmHg At risk 45 35 16 8 - Deaths 0 3 10 12 Peak SBP  120 mmHg At risk 25 8 2 Deaths 0 16 22 Wensel et al. Circulation 2002; 106:319-24.

  40. Summary • Comprehensiveinitial evaluation should focus on: • Establishing etiology • Assessing severity • Determining preferred approach to treatment

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