Chronic Thromboembolic Pulmonary Hypertension Dr Dolores Taboada MD

Chronic Thromboembolic Pulmonary Hypertension Dr Dolores Taboada MD Consultant Cardiologist Pulmonary Vascular Disease Unit Papworth Hospital, Cambridge, UK

My disclosures • I have received honoraria from Actelion, Bayer, GSK, Lilly and Pfizer

Pulmonary hypertension is a haemodynamic state • Mean PAP ≥ 25 mm. Hg • Assessed by RHC Pulmonary Hypertension European Guidelines. Eur Heart Journal 2009; 30, 2493 -2537

Classification of Pulmonary Hypertension Kiely et al, BMJ 2013; 346: f 2028 doi: 10. 1136/bmj. f 2028 (Published 16 April 2013)

Chronic thromboembolic pulmonary hypertension 2 -10% Inappropriate thrombus resolution

Chronic thromboembolic pulmonary hypertension Vascular remodeling pulmonary hypertension right ventricular failure

Symptoms are unspecific – Breathlessness – Chest tightness – Fatigue – Palpitations – Syncope on exertion

Examination – Signs of PH • Loud P 2 • Pansystolic murmur • Right ventricular heave – Signs of RV failure • • Elevated JVP Peripheral edema Hepatomegaly Ascites

Risk factors – Recurrent VTE – Splenectomy – Inflammatory bowel disease – VA shunts – Infected PPM

ESC, European Society of Cardiology; ERS, European Respiratory Society. Galiè N et al. Eur Heart J 2016; 37: 67– 119. 11

Echocardiogram shows ‘pulmonary hypertension’

The Association of Pulmonary Hypertension Centres UK and Ireland Golden Jubilee National Glasgow Freeman Hospital Newcastle Mater Missericordiae Hospital Dublin Royal Hallamshire Hospital Sheffield Cambridge Hammersmith Hospital Royal Free Hospital Royal Brompton Hospital London Papworth Hospital CTEPH expert centre

Right heart catheterisation confirms PH Main Pulmonary Artery Right Ventricle Wedged PA Trace Right Atrium

Imaging confirms CTEPH

Pulmonary Endarterectomy • Surgical removal of occlusive thromboembolic material • Under deep hypothermic circulatory arrest • Potentially cure for CTEPH

Assessment of suitability for PEA Severity of pulmonary hypertension EP Location of thromboembolic material Coexistent medical conditions Symptoms and functional limitation

1/3 Non - operable Pepke-Zaba et al. Circulation 2011; 24: 1973 -81

Targeted medical therapy Lai et al. Circulation Research June 20, 2014

CTEPH – 2 compartments OCCLUDED Organized thromboemboli Changes similar to one seen in the PAH

Balloon pulmonary angioplasty - BPA

CTEPH • A complication of pulmonary embolism • Lifelong anticoagulation • Only potentially curable form of PH • Operability assessment at CTEPH expert centre

CTEPH – treatment options Pulmonary Endarterectomy Balloon Pulmonary Angioplasty Medical Therapy

Multidisciplinary CTEPH team -Papworth Hospital Chest Physicians Surgeons PEA Anaesthetists Dr J Pepke Zaba Mr David Jenkins Dr G Martinez Dr K Sheares Mr Choo Ng Dr J Arrowsmith Dr J Cannon Mr J Dunning Dr A Klein Dr M Toshner Mr S Tsui Dr K Valchanov Prof N Morrell Interventional Cardiologist Stephen Hoole Dr D Taboada Gerry Coghlan Radiologists PEA Specialist Nurses Dr N Screaton Anie Ponaberanam Dr A Ruggiero Liliana Almeida Dr A Rubino Dr K Salaunkey Papworth ICU team MDT coordinator Karin Johnson

Thanks