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Pulmonary Hypertension-Whats New PowerPoint Presentation

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This medical PowerPoint presentation talks about the pulmonary hypertension, its risk factors, recommendations regarding new therapeutic targets and treatment strategies available, and the diagnosis and treatment of pulmonary hypertension in left heart disease.

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Slide 1-

Pulmonary Hypertension: What’s New? Guidance from the CCS/CTS Position Statement on Pulmonary Hypertension Panel March 31, 2021
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© Canadian Cardiovascular Society. 2021. All rights reserved. Distribution, transmission or republication is strictly prohibited without the prior written permission of the Canadian Cardiovascular Society. For medical institution internal education or training (i.e. grand rounds, medical college/classroom education, etc.), kindly consider and implement the following: Include the citation for the Canadian Journal of Cardiology Add ‘Reproduced with permission from the Canadian Cardiovascular Society. [insert year].’ Avoid use of CCS logos or trademarks on slides, tools or publications that are not the property of the CCS Do not modify the slide content and, the layout and CCS branding on the Guideline-related slides Guideline recommendations must be reproduced exactly as published For an industry supported or involved program, permissions are required: If content is published in the Canadian Journal of Cardiology, permissions must be sought through our publisher Elsevier (www.onlinecjc.com) If content is property of the CCS, contact guidelines@ccs.ca
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We are grateful to our partners for their commitment to the CCS. The Canadian Cardiovascular Society is grateful to our partner(s) for their commitment to building knowledge and expertise of the heart team. This program was made possible in part by Janssen. CCS thanks Janssen for their dissemination support so that healthcare providers can benefit from this important information.
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Our Faculty Dr. Lisa Mielniczuk Dr. Naushad Hirani Dr. Ali KAPASI Dr. Sanjay MEHTA MODERATOR
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Disclosure of potential conflicts of interest Lisa Mielniczuk Consulting Fees/Honoraria: Actelion, Amgen, Bayer, Johnson & Johnson, Novartis, PHA Canada, Servier Clinical Trials: Actelion, Amgen, Bayer, Johnson & Johnson, Novartis, PHA Canada Ali Kapasi Consulting Fees/Honoraria: Actelion, Bayer, Boehringer Ingelheim, GlazoSmithKline, Roche Clinical Trials: Actelion, Bayer, Boehringer Ingelheim, Sanofi Naushad Hirani Consulting Fees/Honoraria: Actelion/Janssen, Bayer Clinical Trials: Bayer, Bellerophon, Gilead, Gossamer Bio, United Therapeutics Sanjay Mehta Consulting Fees/Honoraria: Actelion/Janssen, Bayer, Natco Clinical Trials: Actelion/Janssen, Bellerophon, Eiger, Gilead, Reata, United Therapeutics
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Dr. Lisa Mielniczuk MD MSc FRCPC Ottawa ON MODERATOR Cardiologist and Clinician Investigator, University of Ottawa Heart Institute Director, Heart Failure Program Medical Director, Pulmonary Hypertension Clinic
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Overall Learning Objectives Recognize pulmonary hypertension and identify risk factors, Examine recommendations regarding new therapeutic targets and treatment strategies available, and Review an approach to the diagnosis and treatment of pulmonary hypertension in left heart disease.
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A clinical case: Mr. CB – A 56-year-old male Relevant past medical history HTN Dyslipidemia Hypothyroidism Non-smoker Obesity Presents with 18 months of gradually worsening SOB One syncopal episode on year prior 4-5 presyncopal episodes 3 months prior
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A clinical case: Mr. CB – A 56-year-old male Presented to emergency with right HF Initial testing Transferred to our hospital for diagnosis and management NYHA FC III CT scan chest – no evidence of PE PFTs: no evidence of airflow obstruction or abnormal lung volumes 6 minute walk test 100m NTproBNP 700 ng/mL
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ECG
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CXR
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V/Q scan
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Echocardiogram
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Initial considerations
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Right heart catheterization PAP 82/41/57 PCWP 14/17/10 RAP 15/13 PA 49% CO 2.75 CI 1.26 PVR 17 (1367 dsc)
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Cardiac MRI
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Dr. Naushad Hirani MD FRCPC FCCP Calgary AB Diagnostic Approach Clinical Assistant Professor, Division of Respiratory Medicine, University of Calgary Southern Alberta Pulmonary Hypertension Clinic
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Learning Objectives: Diagnostic Approach Discuss the diagnosis of pulmonary hypertension using echocardiography, MRI and right heart catheterization Recognize special groups of patients who may need to be screened for pulmonary hypertension
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Diagnosis of pulmonary hypertension Modified from ESC/ERS PH Guidelines 2015 Echocardiographic probability of PH Symptoms, signs and history suggestive of PH
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Echocardiographic probability of PH Modified from ESC/ERS PH Guidelines 2015
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Secondary signs of PH Ventricles Pulmonary Artery RA/IVC RVd/LVd > 1.0 Septal flattening, LVEI > 1.1 RVOT AT < 105 ms, midsyst notching PI early diastolic vel. > 2.2 RA area > 18 cm2 IVC > 21 mm/ dec. inspire. collapse British Society Echo Guidelines 2010. ESC/ERS PH Guidelines 2015
Slide 23-

Recommendation: Echo for identification of PH We recommend transthoracic echocardiography for initial assessment of all patients with clinically suspected PH or unexplained dyspnea We recommend complete echo assessment when assessing for PH including: estimation of sPAP using the TRV jet, measurement of IVC size and degree of inspiratory collapse, assessment of “secondary” signs of PH, such as right atrial or right ventricular (RV) enlargement, RV hypertrophy, septal flattening, and RV dysfunction Strong Recommendation, Moderate-Quality Evidence
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Echo: Screening populations at risk for PAH Connective tissue disease (CTD) Scleroderma (SLE, overlap syndromes) Adults with congenital heart disease Hepatic Cirrhosis Porto-pulmonary hypertension Liver transplantation – contraindicated if mPAP >35 HIV Infection
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Consider other diagnoses or ongoing follow up Echo probability of PH Symptoms, signs and history suggestive of PH Consider left heart disease and lung disease Modified from ESC/ERS PH Guidelines 2015 High/Intermediate Low
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Left heart disease: HFpEF Normal HFrEF HFpEF Melenovsky V, et al. Eur Heart J 2014; 35: 2452-3462 Fayyaz AU, et al. Circulation 2018; 137: 1796-1810
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Approach to the classification of PH Pulmonary Hypertension Chronic Thromboembolic Disease Lung Disease and/or Hypoxemia Left-Sided Heart Dysfunction Pulmonary Arterial Hypertension WHO Group 1 Idiopathic Connective Tissue Disease Portal Hypertension Congenital Heart Disease COPD Interstitial Lung Disease Sleep Apnea Systolic Diastolic Valvular WHO Group 4 WHO Group 3 WHO Group 2
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Multimodality testing for classification of PH
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Consider other diagnoses or ongoing follow up Echo probability of PH Symptoms, signs and history suggestive of PH Consider left heart disease and lung disease Modified from ESC/ERS PH Guidelines 2015 High/Intermediate Low Refer to expert center for multimodality assessment, diagnosis and Rx V/Q scan to screen for chronic thromboembolic PH (CTEPH)
Slide 30-

Right heart catheterization Should be done after noninvasive workup For diagnosis of PAH mPAP >20 mmHg PAWP ≤15 mmHg PVR ≥3 WU (240 DSC) Recommend that RHC is performed at PH Centre with technical expertise and experience with diagnosis of PH
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Reference level Mid thoracic height best approximates position of left atrium This should be the reference level used at RHC Kovacs et al. Eur Respir J 2013;42:1586-94
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End expiratory measurements Using mean vs. End-expiratory changes PH classification in 25-30% of cases Ryan et al., Am Heart J. 2012;163:589-94 LeVarge et al, Eur Respir J. 2014;44:425-34 End-expiratory wedge Computer mean wedge
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Cardiac MRI Accurate assessment of chamber size, function, shunts Cannot replace RHC RVEDV can enhance risk assessment Useful when CHD is suspected Second line imaging for difficult cases Lewis RA, et al. Am J Resp Crit Care Med Feb 2020
Slide 34-

Dr. Ali Kapasi MD FRCPC Edmonton AB Management of PH Associate Professor, Department of Medicine, Division of Pulmonary Medicine, University of Alberta Heart & Lung Transplantation @kapasi_am
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Learning Objectives: Management of PH Describe when PAH targeted therapies are indicated Develop and appreciation for how PAH targeted therapies are selected and adjusted
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PAH targeted therapies are only indicated in WHO group 1 and 4
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General medical therapies for PAH Avoidance of pregnancy Immunizations against influenza, pneumococcal infection (COVID-19?) Psychosocial support/counselling Supervised exercise training Excessive physical activity leading to distressing symptoms in not advised Supplemental O2 (if resting PaO2 consistently <60 mmHg) Spinal anesthesia is preferable to GA whenever possible for elective surgery
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General medical therapies for PAH Diuretics are indicated in patients with signs of RV failure and fluid retention Oral anti-coagulation should be considered in patients with IPAH, HPAH, and DPAH Correct anemia/Fe deficiency Avoid ACEi, ARBs, β-blockers, ivabradine where possible Tachyarrhythmias are better managed with amiodarone CCBs are reserved for patients with IPAH, HPAH, and DPAH who respond to acute vasoreactivity testing
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PAH targeted therapies Phosphodiesterase type 5 inhibitors Sildenafil Tadalafil Soluble guanylate cyclase stimulants Riociguat Prostanoid analogues Treprostinil IV/SC Epoprostenol IV Endothelin receptor antagonists Bosentan Ambrisentan Macitentan Prostacyclin receptor agonists Selexipag ORAL
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How do we deploy these drugs? We usually start with dual oral combination therapy Patients presenting in NYHA FC IV/with RHF are usually considered for parenteral prostanoids A third drug is usually introduced based on inadequate response to two drugs There are numerous tools for risk assessment in PAH
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What about unstable patients?
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Dr. Sanjay Mehta MDCM FRCPC London ON What about the other (most common) causes of PH? Director, Southwest Ontario PH Clinic, London Health Sciences Centre Professor of Medicine, Western University Eternal PHriend / Past-Chair / Founding Board Member, PHA Canada
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Learning Objectives: Other Causes of PH Recognize and diagnose chronic thromboembolic pulmonary hypertension (CTEPH) Appreciate the limitations of current treatment approaches for some types of pulmonary hypertension
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N Hirani et al / CCS - CTS, CJC 2020
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D Helmersen et al / CTS, CJRCCSM 2019
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WHO classification of PH Pulmonary Hypertension Chronic Thromboembolic Disease Lung Disease and/or Hypoxemia Left-Sided Heart Dysfunction Pulmonary Arterial Hypertension WHO Group 1 Idiopathic Connective Tissue Disease Portal Hypertension Congenital Heart Disease COPD Interstitial Lung Disease Sleep Apnea Systolic Diastolic Valvular WHO Group 4 WHO Group 3 WHO Group 2
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Global estimates of PH M Hoeper et al, Lancet Resp Med 2016
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WHO Group 2 PH due to left heart disease
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WHO Group 2 PH due to left heart disease Diagnosis / Management Disproportionate dyspnea, edema Echo identification of PH and/or RV failure Optimal heart failure management: especially aggressively DIURESE !!! Assess / treat possible underlying Hypoxemia Lung disease (eg. COPD, sleep apnea) Pulmonary emboli
Slide 50-

Management: WHO Group 2 (post-capillary) PH due to heart disease We recommend that management should focus on efforts to optimize LV filling pressures and treat the underlying causes of and contributors to left heart disease Strong Recommendation, Low-Quality Evidence We recommend against the routine use of PAH-targeted therapies Strong Recommendation, Low-Quality Evidence N Hirani et al / CCS - CTS, CJC 2020
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WHO Group 3 PH due to lung disease / hypoxemia
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WHO Group 3 PH due to lung disease / hypoxemia Diagnosis / Management Disproportionate dyspnea, edema Echo identification of PH and/or RV failure Optimal lung disease management Hypoxemia (resting, exertional, nocturnal eg. sleep apnea) Assess / treat contributing causes Identify and Rx pulmonary embolism Identify and Rx underlying heart disease / fluid overload
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Management: WHO Group 3 PH due to lung disease / hypoxemia Mild-moderate PH / absence of RV failure: We recommend against routine RHC and against the use of PAH-targeted therapy Strong Recommendation, Moderate-Quality Evidence Moderate-severe PH and/or RV failure: We suggest referral to a PH centre Strong Recommendation, Low-Quality Evidence N Hirani et al / CCS - CTS, CJC 2020
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WHO Group 4 chronic thromboembolic PH (CTEPH) The most important cause of PH A common cause of PH which is frequently curable Established diagnostic work-up Available, effective treatment options Anticoagulation PEA/Pulmonary endarterectomy surgery PH-specific medical therapy Balloon pulmonary angioplasty (BPA)
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Nuclear V/Q lung scan CTEPH
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WHO Group 4 CTEPH can be cured by pulmonary endarterectomy
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PEA surgery: outcome X – Untreated Historical Survival M Madani et al / UCSD, Ann Thoracic Surg 2012
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CTEPH: a role for PH-targeted medical therapy Distal vascular obstruction - inoperable Residual Post-Op PH Proximal vascular obstruction surgery eligible ‘Cured’ Rx: Riociguat, BPA, ? others
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Case wrap-up
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QUESTIONS?
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FACULTY On Thursday, April 15, 2021 from 8 PM – 9 PM EDT, join us for an interactive case-based webinar highlighting content from the 2021 Canadian Cardiovascular Society/Canadian Heart Failure Society Heart Failure Guidelines Update: Defining a New Pharmacologic Standard of Care for HFrEF Dr. Virani Dr. McDonald Dr. Ezekowitz CCS/CHFS HF Guidelines Webinar What’s new in 2021: An updated standard of care of HFrEF Register at CCS.ca