ΔΙΑΓΝΩΣΗ ΚΑΙ ΘΕΡΑΠΕΙΑ ΤΗΣ ΧΡΟΝΙΑΣ ΘΡΟΜΒΟΕΜΒΟΛΙΚΗΣ ΥΠΕΡΤΑΣΗΣ (CTEPH)

Transcription

1 Aristotle University of Thessaloniki Cardiology Clinic, AHEPA Hospital ΔΙΑΓΝΩΣΗ ΚΑΙ ΘΕΡΑΠΕΙΑ ΤΗΣ ΧΡΟΝΙΑΣ ΘΡΟΜΒΟΕΜΒΟΛΙΚΗΣ ΥΠΕΡΤΑΣΗΣ (CTEPH) Charalampos I. Karvounis, MD Professor of Cardiology Aristotle University of Thessaloniki

2 Disclosures Honoraria for lectures: MSD, Bayer, Actelion, Elpen, Pfizer, Novartis, Sanofi, Servier.

3 Female 42y

4 Background 2006 Healthy until summer Aug 2006 Bronchitis followed by progressive dyspnea Oct 2006 Pulmonology: normal lung function, asthma. No improvement under asthma treatment Dec 2006 Cardiology: normal left and right heart function Mar 2007 Psychiatry: normal

5 Background April June 2007 Progressive dyspnea (NYHA FC II III) Oct 2007 Pulmonology/Cardiology (University Hospital): Normal lung function: po2 67 mmhg, pco2 31 mmhg Echo: RV enlargement; spap 72 mmhg + RAP, TAPSE: 21 mm CTPA: no thrombosis Diagnosed as idiopathic PAH and started on bosentan (no RHC!)

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7 Initial assessment 2010 Dyspnoea in mild exertion WHO class III BMI: 31kg/cm 2 SAT 88%, no clubbing HR 88/min, BP 110/80mmHg Loud P2 Mild JVP elevation No ascites / mild peripheral edema 6MWT (without oxygen) Pre Post SpO HF Borg 2 8 Total walked distance 287m

8 ECG

9 ESC/ERS guidelines 2015

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11 Lung perfusion scan: High risk of PE/CTEPH with normal ventilation and perfusion defects on right anterior, posterobasal and laterobasal segmental and subsegmental branches and left posterior, posterobasal and segmental and subsegmental brances P V P V

12 CTPA

13 CTPA

14 HRCT

15 ALK1, ENG, KCNK3, CAV-1 ESC/ERS guidelines 2015

16 RHC (Fick method)

17 Pulmonary angiogram

18 Pulmonary angiogram

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20 CTEPH algorithm Anticoagulation was added No change after 3 months Operability assessment by an expert center abroad

21 Patient was assessed as inoperable (distal disease, very high PVR disproportionate with obstructions) Treprostinil was added to bosentan, dose uptitrated to 90ng/kg/min Gradually improved, still NYHA Ill, 6MWT 380m

22 RHC

23 2 years later Further clinical deterioration (NYHA FC IV; 6MWD 185 m Deterioration of RV function (TTE) Second operability assessment (Discussion about surgical treatment (PEA vs LTx) with patient Patient s wish for PEA (surgical risk up to 20%)

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25 Work-up abroad RHC CI: 1.9 l/min/m2 mpap: 49mmHg PVR: 9.9 W pulmonary angiogram Diffuse segmental and distal subsegmental disease

26 Pulmonary endarterectomy, June 2014

27 KM survival estimates Operated vs non-operated Cumulative Survival p=< (log rank test) 0.00 patients at risk at the end of the time period Months from Diagnosis Operated, N=404 Non operated, N=275 Simonneau G, et al. ATS (accepted).

28 MR Angiography pre-op. post-op.

29 July 2015 Patient feeling much better Now in WHO class II 6MWT = 410 m Under acenocoumarol, no targeted PH therapy

30 RHC

31 Diagnosis: Residual PH post PEA Patient was started on riociguat 1 mg tid uptitrated uneventfully to 2.5 mg tid Patient improved, 6MWT = 480 m

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33 Balloon angioplasty of PA-branches (BPA) as standalone- or hybrid-procedure

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37 Take home messages Differentiating CTEPH vs IPAH is crucial (different treatment pathways, CTEPH is potentially curable) In patients with unexplained dyspnea, echocardiography and V/ Q scan are essential screening methods for CTEPH A negative CT scan does not rule out operable CTEPH. Lung perfusion scan should precede. Patients should be referred to expert centers with experience in CTEPH imaging Diagnosis and evaluation of operability are based on RHC and pulmonary angiography (CT and/or MRI conventional)

38 Take home messages A multidisciplinary expert team is mandatory for all types of CTEPH treatment The evaluation of operability is subjective and influenced by experience (what is distal?) In case of inoperability, a second opinion is recommended PEA surgery can also be successful in high-risk patients

39 Take home messages Riociguat is the 1 st licensed drug for persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) after surgical treatment or inoperable CTEPH

40 ΕΥΧΑΡΙΣΤΩ ΠΟΛΥ ΓΙΑ ΤΗΝ ΠΡΟΣΟΧΗ ΣΑΣ