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
Disclosures Sponsored to attend scientific meetings, or honoraria by: Actelion Bayer MSD Galenica GSK Pfizer
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)
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
Patient case The patient presented a syncopal episode (during his first 6MWT) and he was transferred immediately to our Cardiology Unit
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
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
Patient case Jan. 2015 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
Patient case What is the Diagnosis? acute pulmonary embolism acute pulmonary embolism on chronic CTEPH
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
Patient case Jan. 2015 May 2015 Right Heart Catheterization RAP 5 9 mmhg PAP 70/30/44 81/27/45 mmhg (S/D/M) PAWP 10 12 mmhg CO 5,4 l/min CI 2,4 l/min/m 2 PVR 6,1 Wood Units SvO2 71 %
Patient case Perfusion lung scanning multiple bilateral segmental perfusion defects
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
Patient case The patient was evaluated for potential pulmonary endarterectomy
Patient case The patient was evaluated for potential pulmonary endarterectomy He underwent Bilateral Pulmonary Endarterectomy (PEA) in Germany (October 2015)
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
Patient case 10 months later
Patient case 10 months later TTE epasp: 50 mmhg
post PEA
post PEA
pre post 1 year
post PEA
Patient case PEA Jan. 2015 May 2015 Oct. 2016 Right Heart Catheterization RAP 5 9 9 mmhg PAP 70/30/44 81/27/45 61/27/38 mmhg (S/D/M) PAWP 10 12 15 mmhg CO 5,4 6,8 l/min CI 2,4 3 l/min/m 2 PVR 6,1 3,4 Wood Units SvO2 71 75 %
Patient case Jan. 2015 May 2015 Oct. 2016 Right Heart Catheterization RAP 5 9 9 mmhg PAP 70/30/44 81/27/45 61/27/38 mmhg (S/D/M) PAWP 10 12 15 mmhg CO 5,4 6,8 l/min CI 2,4 3 l/min/m 2 PVR 6,1 3,4 Wood Units SvO2 71 75 % Oct. 2016: initiation of specific treatment with riociguat (stimulator of soluble guanylate cyclase)
Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015
Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015
Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015
Recommendations for chronic thromboembolic pulmonary hypertension PH guidelines 2015
N Engl J Med 2013;369:319-29
N Engl J Med 2013;369:319-29
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: 372 80
Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy Results From the United Kingdom National Cohort From 1997 to 2012 880 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: 1761-1771
Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy Results From the United Kingdom National Cohort Circulation. 2016; 133: 1761-1771
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
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
CTEPH a late complication of (unresolved) acute pulmonary embolism? DVT EMBOLUS ACUTE PE CTEPH IN TRANSIT
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
Delcroix, Kerr, & Fedullo: CTEPH Epidemiology and Risk Factors Annals ATS July 2016
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
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
Delcroix, Kerr, & Fedullo: CTEPH Epidemiology and Risk Factors Annals ATS July 2016
F.A. Klok et al. / Blood Reviews 28 (2014) 221 226
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
Ventriculoatrial shunts Myeloproliferative syndromes Chronic inflammation, autoimmunity High risk of persisting thrombi Splenectomy Central venous catheters Hypercoagulability (antiphospholipid syndrome, FVIII)
Lang, Dorfmuller, & Noordegraaf: Pathobiology of CTEPH. Annals ATS July 2016
Treatment algorithm for chronic thromboembolic pulmonary hypertension. PH guidelines 2015