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Diagnostic approaches in pulmonary hypertension: functional evaluation

Diagnostic approaches in pulmonary hypertension: functional evaluation. Prof L.P.Nicod Pulmonary service University hospital of Canton Vaud: CHUV-CH. Mrs BN 30 years old , non smoker , with BMI 29

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Diagnostic approaches in pulmonary hypertension: functional evaluation

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  1. Diagnostic approaches in pulmonary hypertension: functionalevaluation Prof L.P.Nicod Pulmonary service Universityhospital of Canton Vaud: CHUV-CH

  2. Mrs BN 30 yearsold, non smoker, with BMI 29 History: Stoppedplaying tennis 2 yearsago ( Dyspnea stage I) Can not climbtwoflights of stairssince a year (Dyspnea stage II) Do have to slow down while shopping since 2 months (Dyspnea stage III) Home doctor:-normal physicalexamination -normal hemogram -normal smallspirometry -normal chestXray advice: pleaseexercise and looseweight

  3. Mrs BN 30 yearsold, non smoker, with BMI 29 Presenthistory: can not copewithher kids and house keepingdespite weightloss of 2 kg and exercisedaily. Look for second opinion: Examination: -lungsclear -no legedema and heartsoundwithlouder B2 (?) Investigations: CXR: increased PA and heart size ECHO: thick and dilated right heart ventricule PAPs:80mmHg V/Q: »normal »

  4. Mrs BN 30 yearsold, non smoker, with BMI 29 RX: large Pulmartery, right ventricule and atrium NB:normal in 10% ECG: right axial deviation, tall R in V1

  5. Mrs BN 30 yearsold, non smoker, with BMI 29

  6. Mrs BN 30 yearsold, non smoker, with BMI 29 Catheter: PAPm 60mmHg CO 4.0 l/min wedge pressure :10 mmHg reversibility test :with 20ppm NO decrease of PAPm to 50mmHg

  7. Pulmonary Hypertension mainetiologicgroups Frequence + ++++ +++ ++ + 1. Pulmonaryarterialhypertension(PAH) 1.1.Idiopathic pulmonaryhypertension(IPAH) 1.2.Heritable 1.3.Associated with...(APAH) 2. Pulmonaryhypertensionwithleftheartdesease 3.Pulmonaryhypertensiondue tolungdeseasesand and/orhypoxemia 4.Chronicthromboembolicpulmonaryhypertension 5.Pulmonary hypertensionwithunclearmultifactorial deseases

  8. Idiopathic pulmonary-arterial hypertension

  9. Pulmonary Hypertension in Switzerland 1999-2004 pat N 80 1999-2004 PAH 1991-1999 60 40 20

  10. Clinical progression of IPAH NB:Preclinical stage is noticed as progressive dyspnea stage 1-2

  11. Pulmonaryfunction in PAH .Normal spirometry or slight restriction .Normal lung volumes or slight restriction .Low DLCO/KCO .Low PaO2 but normal PaCO2

  12. Pulmonary Hypertension diagnostic pathway Clinical Breathless Clearlung Signs of PHT Signs of associateddisease CXR: large Pulmartery, right atrium ECG: right axial deviationtall R in V1 ABG: slightfall in PaO2 PFTs : normal spirometry and volumes but low DLCO Basic Investigations Advanced non Invasive investigations V/Q scan normal High resolution CT ECHO:-signs of PHT -signs of RV/LV dysfunction -signs of L-R shunt Right heartcatheterisation -pressures -flow -saturation -(angiography) Invasive Pulmonaryvascular Center

  13. Aetiology of pulmonary hypertension in Switzerland 1999-2004 29% CTEPH 4% HIV 5% congenital shunts 28% idiopathic 6.5% not specified 7% hypoxemia related 16% collagen vascular disease < 3%: drugs, portal hypertension, PVOD, others (sarcoidosis, histiocytosis) N = 250

  14. PAH associated with connective tissue disease Leading causes for CTD-PAH: systemic sclerosis 75% lupus erythematosus 7-15% mixed connective tissue disease 9% Coghlan JG. Lupus 2006;15 Prevalence of PAH in patients with systemic sclerosis 12-16% Mukerjee D. Ann Rheum Dis 2003;62 89/722 patients (echocardiography,right heart catheter)

  15. Portopulmonary Hypertension Prevalence with cirrhosis: Autopsie-Study :0,73%McDonnel et al, Am Rev Respir Dis 1983;127:437 Studies based on catheterism : 10/507 = 2%Hadengue et al, Gastroenterology 1991;100:520 2/43 = 4,7% Sen et al, Indian J Gastroenterol 1999;18:158 10/322 = 3,1%Yang et al, J Gastroenterol 2001;36:181 liver transplant-candidates 8/257 = 3,5%Taura et al, AnsthAnal 1996;83:675 15/362 = 4%Castro et al, Mayo CliProc 1996;71:543

  16. Portopulmonary Hypertension Clinical presentation: Symptoms essentiallyifPAPm (> ca. 40 mm Hg): Dyspnea on exertion 92% Deasiness on exertion 24% Chestpain 13% Hemoptysis 8% Rightheartinsufficiency 22% Serie with 76 patientsHadengue et al, Gastroenterology 1991 Water retension : leg edema, Anasarque, neck veins distension ,Ascitis

  17. Changes in pulmonaryarteryhypertensionduring COPD exacerbations E Weitzenblum et al B.Decker 2002

  18. Emphysemafibrosis and severepulmonary hypertension V. Cottin et al. E R J 2010; 35: 105

  19. Typical perfusion defects during V/Q scyntigraphy in chronic thromboembolic desease Ventilation Perfusion Perfusion

  20. Chronic thrombotic/embolic PH • 5.1% survivors of acute PE develop CTEPH after 1 year • Ribeiro A. Circulation 1999;99 • 3.8% survivors of first episode of acute PE develop CTEPH after 2 years • Pengo V. NEJM 2004;350 NB: 63% of CTEPH patients without history of symptomatic PE Lang IM. NEJM 2004;350

  21. Treatment in PAH isexpected to : .Preventprematuredeath .Slow / stop progressive functional limitation .Reduce -symptoms -hospitalisations -consequences of associated complications .ImproveQuality of life

  22. Therapeutic goals in PAH

  23. Improvement of WHO functionalclassimpacts on prognosis FC after 3-months epoprostenol therapy >1-year epoprostenol* 100 100 80 80 WHO I WHO I or II(n=91) 60 60 Survival (%) WHO II Survival (%) 40 40 p<0.0001 20 WHO III WHO III or IV(n=75) 20. WHO IV 0 0 0 12 24 36 48 60 72 84 0 12 24 36 48 60 72 84 96 108 Time (months) Time (months) *Mean prior treatment duration: 17 months Sitbon O, et al. J Am Coll Cardiol 2002; 40:780-8. McLaughlin VV, et al.Circulation 2002; 106:1477-82.

  24. Improved survival when 6-MWD reaches 380 m with therapy Survival after 3 months on epoprostenol 1.0 0.8 6-MWD 380 m 0.6 Cumulative survival p=0.0005 0.4 0.2 6-MWD <380 m 0 0 12 24 36 48 60 72 84 96 108 Time (months) 78 71 56 41 28 15 4 6-MWD 380 m Subjectsat risk (n) 78 54 28 16 8 6 3 3 1 6-MWD <380 m Sitbon O, et al.J Am Coll Cardiol 2002; 40:780-8.

  25. Cardiopulmonaryexercise test and survivalprediction in PAH : VO2 and Systolicblood pressure Wenzel R et al Circulation 2002;106:319

  26. NB: 1)Correlationwith RV function 2) Kaplan and Meier estimates of survival (primary endpoint, all-cause mortality) in patients stratified by TAPSE values tricuspidannular plane systolic excursion [TAPSE] P.Forfia et al.Am J RespirCrit Care Med 2006 : 174 ; 1034

  27. Goal-oriented use of combination therapy for PAH – Hanover algorithm in 2005 Diagnosis of PAHVasoreactivity test negative Baseline examination and 3 to 6 monthly re-evaluation to assess treatment goals (6-min walk distance >380 m, peak VO2 >10.4 ml/min/kg, peak SBP >120 mmHg) Treatment goals not met Treatment goals met Start bosentan Continue treatment Add sildenafil Continue treatment Sequentialcombination Addition of inhaled iloprost Continue treatment Transition from inhaled to intravenous iloprost Continue treatment Urgent lung transplantation ModifiedfromHoeper MM, et al. EurResp J 2005; 26:858-63.

  28. Survival of the goal orientedtreatment of PAH vs historical control group in Hannover M.M. Hoeper et al. EurRespir J 2005; 26: 858

  29. ESC/ERS Guidelines on Pulmonary Hypertension 2009 Therapeuticalgorithm BAS = balloon atrial septostomy

  30. Thanks for your attention ! Castle and cathedral of Lausanne -Switzerland

  31. ESC/ERS Guidelines on Pulmonary Hypertension 2009 Therapeuticalgorithm Acute vasoreactivity test

  32. Pulmonary Hypertension in Switzerland (1999-2004) NYHA I 2 (1%) NYHA II 29 (12%) NYHA III 151 (62%) NYHA IV 60 (25%)

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