From Pulmonary Embolism to Chronic Thromboembolic Pulmonary Hypertension

Transcription

1 From Pulmonary Embolism to Chronic Thromboembolic Pulmonary Hypertension Dr Rachel Davies Respiratory Physician National Pulmonary Hypertension Service Hammersmith Hospital Royal College of Physicians Regional Update in Medicine 27 th April 2017

2 Natural History of Pulmonary Embolism Sudden Death 1 Post-Mortem Diagnosis 1 Hospitalized 1 Cohen et al, Stein et al, Pepke-Zaba et al, 2011

3 Natural History of Pulmonary Embolism Sudden Death 1 Post-Mortem Diagnosis 1 Hospitalized 7-11% Mortality 2 Commenced on anticoagulation Discharged 1 Cohen et al, Stein et al, Pepke-Zaba et al, 2011

4 Natural History of Pulmonary Embolism Sudden Death 1 Post-Mortem Diagnosis 1 Hospitalized 7-11% Mortality 2 Commenced on anticoagulation Chronic Symptoms 3 Discharged 1 Cohen et al, Stein et al, Pepke-Zaba et al, 2011

5 WHO Classification of Pulmonary Hypertension European Heart Journal 2015; eurheartj.ehv317

6 Prevalence of Chronic Thromboembolic Pulmonary Hypertension in the UK

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8 Prevalence of Chronic Thromboembolic Pulmonary Hypertension in the UK NPHS Services

9 Pulmonary Embolism Clinics Imperial Trust model Patients seen at 6 weeks to check compliance with anticoagulation Seen at three months with a haematologist Assessment of possible provoking factors of incident event Assessment of clinical status - VQ scan If breathless echocardiogram and cardiopulmonary exercise test

10 Case October 2012 calf pain 4 weeks later short of breath March 2013 diagnosed with pulmonary embolism Commenced on warfarin Reviewed in local respiratory clinic at 3 months Ongoing breathlessness

11 Referred to QA Joint Pulmonary Hypertension Clinic WHO Functional Class III Exertional Dizziness Swollen ankles PMH Nil other than PE 2013 DH Warfarin O/E JVP not raised; mild peripheral oedema O2 Sats 96% on air; HS Loud P2

12 Peak Tricuspid Regurgitant Velocity

13 European Heart Journal 2015; eurheartj.ehv317

14 ESC/ERS Echo Guidance European Heart Journal 2015

15 Presence of other signs of PH European Heart Journal 2015

16 Case Peak TR Velocity 3.6m/s ~ RVSP 60mmHg

17 Four Chamber View

18 Case

19 Radiographic Features of CTEPH PA > Ao Extrinsic Thrombus Web Webs

20 Common Pitfall

21 Ventilation Perfusion Scan

22 Case mpa 60mmHg RA 6 mmhg PAWP 8mmHg Derived indices: Units Pulmonary flow (l/min) 4.92 L/min PFI (l/min/m 2 ) 2.54 L/min/m 2 PVR (Wood U)* 11 WU *calculated using: PCWP

23 Pathogenesis

24 Pathogenesis

25 Risk Factors for the Development of CTEPH Previous venous thromboembolism Some Procoagulant States (phospholipid antibodies, intrinsic abnormalities in fibrinogen,?elevated Factor VIII levels) History of malignancy History of splenectomy Presence of a ventriculoatrial shunt Presence of an infected pacemaker Chronic inflammatory conditions (osteomyelitis, inflammatory bowel disease) Hypothyroidism

26 Anticoagulation Lifelong anticoagulation First line warfarin with a target INT of 2-3 We do use Direct Oral Anticoagulants in patients intolerant of warfarin therapy

27 Assessing Operability for Pulmonary Endarterectomy Severity of clinical symptoms Accessibility of thromboembolic lesions Degree of impairment of haemodynamics - pulmonary vascular resistance (1500 Dynes) Presence of co-morbidities Mayer et al, 2011

28 Pulmonary Endarterectomy Surgery

29 Pulmonary Endarterectomy Surgery

30 Pulmonary Endarterectomy Surgery

31 Pulmonary Endarterectomy Surgery

32 Case

33 Case Pre-PEA Post-PEA Pre-PEA Post-PEA Mean PAP 60mmHg 22mmHg Cardiac Output 4.9 l/min 7.4 l/min Pulmonary Vascular Resistance 11 Wood units 1.6 Wood units

34 International CTEPH Registry Data Long Term Survival 89% 70% 4.7% in hospital mortality post pulmonary endarterectomy Delcroix et al, 2016

35 UK CTEPH Data Condliffe et al, AJRCCM 2008

36 UK Post-Pulmonary Endarterectomy Functional Status Condliffe et al, AJRCCM 2008

37 UK CTEPH Data Condliffe et al, AJRCCM 2008

38 Rationale for Treatment with PAH Therapy in Non-Operable or Post-Op CTEPH Vasculopathy Acute vasodilator response Low circulating Endothelin 1 levels

39 Novel Treatment of Non-operable CTEPH Riociguat Soluble GC Stimulator

40 Novel Treatment of Non-operable CTEPH Riociguat Soluble GC Stimulator Ghofrani, N Engl J Med 2013; 369:

41 Balloon Pulmonary Angioplasty

42 Summary ESC/ERS Treatment Recommendations European Heart Journal 2015; eurheartj.ehv317

43 UK Pulmonary Hypertension Service

44 UK Pulmonary Hypertension Service