Real life management of CTEPH: patient case

Transcription

1 2 nd International Congress on cardiovascular imaging in clinical practice k Real life management of CTEPH: patient case Anastasia Anthi Pulmonary Hypertension Clinic, Attikon University Hospital, Athens

2 Disclosures Sponsored to attend scientific meetings, or honoraria by: Actelion Bayer MSD Galenica GSK Pfizer

3 Patient case 37 years old male referral to the PH Clinic of Attikon Hospital (January 2015) due to an abnormal trans-thoracic echocardiogram (epasp: 80mmHg)

4 Patient case 37 years old male referral to the PH Clinic of Attikon Hospital (January 2015) due to an abnormal trans-thoracic echocardiogram (epasp: 80mmHg) past medical history 13 years old: malignant bradycardia pacemaker implantation 21 years old: pacemaker removal due to infective endocarditis recurrent episodes of hemoptysis hospitalizatin (June 2012): hemoptysis attributed to bronchiectasis no anticoagulation NYHA II late

5 Patient case The patient presented a syncopal episode (during his first 6MWT) and he was transferred immediately to our Cardiology Unit

6 Patient case The patient presented a syncopal episode (during his first 6MWT) and he was transferred immediately to our Cardiology Unit Trans-thoracic echocardiogram RV: dilatation with reduced contractility LV: normal size & function

7 Patient case The patient presented a syncopal episode (during his first 6MWT) and he was transferred immediately to our Cardiology Unit CT pulmonary angiography significant occlusions of multiple segmental pulmonary arteries (both lungs, especially in the lower lobes) enlarged collateral bronchial & intercostal arteries dilatation of the pulmonary artery dilatation and hypertrophy of the right ventricle

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11 Patient case Jan Right Heart Catheterization RAP 5 mmhg PAP 70/30/44 mmhg (S/D/M) PAWP 10 mmhg CO l/min CI l/min/m 2 PVR Wood Units SvO2 % Left Heart Catheterization : normal findings

12 Patient case What is the Diagnosis? acute pulmonary embolism acute pulmonary embolism on chronic CTEPH

13 Patient case What is the Diagnosis? acute pulmonary embolism acute pulmonary embolism on chronic CTEPH The patient was treated with LMWH overlapping with VKA targeting an INR of 2.5-3

14 Patient case Jan May 2015 Right Heart Catheterization RAP 5 9 mmhg PAP 70/30/44 81/27/45 mmhg (S/D/M) PAWP mmhg CO 5,4 l/min CI 2,4 l/min/m 2 PVR 6,1 Wood Units SvO2 71 %

15 Patient case Perfusion lung scanning multiple bilateral segmental perfusion defects

16 Patient case Perfusion lung scanning multiple bilateral segmental perfusion defects Pulmonary function tests FEV1: 4.01 L (85% predicted) FVC : 5.16 L (90%) FEV1/ FVC : 77 TLC: 7,15 L (87%) SaO2: 97% (on room air) Laboratory screening for CTD: negative Thrombophilia molecular tests: heterozygosity for MTHFR gene

17 Patient case The patient was evaluated for potential pulmonary endarterectomy

18 Patient case The patient was evaluated for potential pulmonary endarterectomy He underwent Bilateral Pulmonary Endarterectomy (PEA) in Germany (October 2015)

19 Patient case The patient was evaluated for potential pulmonary endarterectomy He underwent Bilateral Pulmonary Endarterectomy (PEA) in Germany (October 2015) difficulties related to previous surgery for removal of infected pacemaker

20 Patient case 10 months later

21 Patient case 10 months later TTE epasp: 50 mmhg

22 post PEA

23 post PEA

24 pre post 1 year

25 post PEA

26 Patient case PEA Jan May 2015 Oct Right Heart Catheterization RAP mmhg PAP 70/30/44 81/27/45 61/27/38 mmhg (S/D/M) PAWP mmhg CO 5,4 6,8 l/min CI 2,4 3 l/min/m 2 PVR 6,1 3,4 Wood Units SvO %

27 Patient case Jan May 2015 Oct Right Heart Catheterization RAP mmhg PAP 70/30/44 81/27/45 61/27/38 mmhg (S/D/M) PAWP mmhg CO 5,4 6,8 l/min CI 2,4 3 l/min/m 2 PVR 6,1 3,4 Wood Units SvO % Oct. 2016: initiation of specific treatment with riociguat (stimulator of soluble guanylate cyclase)

28 Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015

29 Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015

30 Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015

31 Recommendations for chronic thromboembolic pulmonary hypertension PH guidelines 2015

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33 N Engl J Med 2013;369:319-29

34 N Engl J Med 2013;369:319-29

35 Predictors of long-term outcomes in pts treated with riociguat for CTEPH: data from the CHEST-2 open-label, randomised, long-term extension trial Lancet Respir Med 2016; 4:

36 Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy Results From the United Kingdom National Cohort From 1997 to pts with CTEPH underwent PEA at Papworth 51% of pts had an mpap 25 mm Hg RHC at 3 to 6 months post-pea Circulation. 2016; 133:

37 Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy Results From the United Kingdom National Cohort Circulation. 2016; 133:

38 Chronic thromboembolic pulmonary hypertension (CTEPH) Group 4 PH (Nice pulmonary hypertension classification) Diagnosis confirmed by: precapillary pulmonary hypertension (RHC) at least one segmental perfusion defect at scintigraphy and typical findings at conventional or CT pulmonary angiography after at least 3 months of effective anticoagulation

39 CTEPH is a dual vascular disorder 1. Stenoses, webs, & occlusions predominate in large & medium-sized pulmonary arteries at the sites of previous pulmonary emboli 2. A secondary vasculopathy resembling the pulmonary arteriopathy encountered in other forms of pulmonary hypertension predominates in low-resistance vessels

40 CTEPH a late complication of (unresolved) acute pulmonary embolism? DVT EMBOLUS ACUTE PE CTEPH IN TRANSIT

41 History of pts with CTEPH 56% deep venous thrombosis 75% acute pulmonary embolism 41% anatomically massive PE 33% recurrent PE 25% no history of symptomatic PE only a small percentage of pts with acute PE develop CTEPH

42 Delcroix, Kerr, & Fedullo: CTEPH Epidemiology and Risk Factors Annals ATS July 2016

43 Complete resolution of thrombi is usually not achieved after acute PE 30 50% of pts have persistent defects 1 year after diagnosis few of these pts met the hemodynamic definition of CTEPH

44 Complete resolution of thrombi is usually not achieved after acute PE 30 50% of pts have persistent defects 1 year after diagnosis few of these pts met the hemodynamic definition of CTEPH The term chronic thromboembolic pulmonary vascular disease (CTEPVD) has been proposed persistent perfusion defects exercise limitation no pulmonary hypertension at rest

45 Delcroix, Kerr, & Fedullo: CTEPH Epidemiology and Risk Factors Annals ATS July 2016

46 F.A. Klok et al. / Blood Reviews 28 (2014)

47 Pathophysiology of CTEPH VTE Infection, inflammation Immunity Genetic predisposition Acute PE Incomplete resolution and organization of thrombus Lack of thrombus angiogenesis In situ thrombosis Development of fibrotic stenoses/occlusions Adaptive vascular remodeling of resistance vessels CTEPH

48 Ventriculoatrial shunts Myeloproliferative syndromes Chronic inflammation, autoimmunity High risk of persisting thrombi Splenectomy Central venous catheters Hypercoagulability (antiphospholipid syndrome, FVIII)

49 Lang, Dorfmuller, & Noordegraaf: Pathobiology of CTEPH. Annals ATS July 2016

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51 Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015

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