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Assessing the Right Ventricle in Pulmonary Arterial Hypertension: Getting to the Heart of the Matter

Assessing the Right Ventricle in Pulmonary Arterial Hypertension: Getting to the Heart of the Matter. Vallerie V. McLaughlin, MD Professor of Medicine Director, Pulmonary Hypertension Program Department of Internal Medicine Division of Cardiovascular Medicine

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Assessing the Right Ventricle in Pulmonary Arterial Hypertension: Getting to the Heart of the Matter

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  1. Assessing the Right Ventricle in Pulmonary Arterial Hypertension:Getting to the Heart of the Matter Vallerie V. McLaughlin, MD Professor of Medicine Director, Pulmonary Hypertension Program Department of Internal Medicine Division of Cardiovascular Medicine University of Michigan Health System Ann Arbor, Michigan

  2. Goals • Summarize the role of diagnostic testing to evaluate the right ventricle in patients with PAH • Explore emerging as well as existing diagnostic tools • Evaluate the relevance of the diagnostic findings in risk stratification and the utilization of appropriate therapies

  3. Disclosures Vallerie V. McLaughlin, MD has disclosed the following relevant financial relationships: Served as a consultant and/or on a speakers bureau and/or has received grants/research support from: Actelion Pharmaceuticals, Ltd; Bayer Healthcare Pharmaceuticals; Gilead Sciences, Inc.; Novartis Pharmaceuticals Corporation; United Therapeutics Corporation

  4. Hemodynamic Definition of PH/PAH PH Mean PAP ≥ 25 mm Hg Mean PAP ≥ 25 mm Hg plusPCWP/LVEDP ≤ 15 mm Hg PAH ACCF/AHA CECD includes PVR > 3 Wood units PH = pulmonary hypertension; PAH = pulmonary arterial hypertension; PAP = pulmonary arterial pressure; PCWP = pulmonary capillary wedge pressure; LVEDP = left ventricular end-diastolic pressure; ACCF = American College of Cardiology Foundation; AHA = American Heart Association; CECD = Clinical Expert Consensus Document; PVR = pulmonary vascular resistance McLaughlin VV, et al. JAm Coll Cardiol. 2009;53:1573-1619. Badesch D, et al. J Am Coll Cardiol. 2009;54:S55-S66.

  5. Clinical Classification of PH PAH Idiopathic PAH Heritable Drug- and toxin-induced Persistent PH of newborn Associated with: Connective tissue disease HIV infection Portal hypertension Congenital heart disease Schistosomiasis Chronic hemolytic anemia • 1’. Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis • PH Due to Left Heart Disease • Systolic dysfunction • Diastolic dysfunction • Valvular disease Simonneau G, et al. J Am Coll Cardiol. 2009;54:S43-S54.

  6. Clinical Classification of PH (cont) • 3. PH Due to Lung Diseases and/or Hypoxia • Chronic obstructive pulmonary disease • Interstitial lung disease • Other pulmonary diseases with mixed restrictive and obstructive pattern • Sleep-disordered breathing • Alveolar hypoventilation disorders • Chronic exposure to high altitude • Developmental abnormalities • 4. Chronic Thromboembolic PH • 5. PH With Unclear or MultifactorialMechanisms • Hematologic disorders • Systemic disorders • Metabolic disorders • Others Simonneau G, et al. J Am Coll Cardiol. 2009;54:S43-S54.

  7. Reversible Disease Normal Irreversible Disease Pathogenesis of PAH Risk Factors andAssociated Conditions Collagen Vascular Disease Congenital Heart Disease Portal Hypertension HIV Infection Drugs and Toxins Pregnancy Vascular Injury Endothelial Dysfunction ↓ Nitric Oxide Synthase ↓ Prostacyclin Production ↑ Thromboxane Production ↑ Endothelin 1 Production Vascular Smooth Muscle Dysfunction Impaired Voltage-Gated Potassium Channel (KV1.5) Disease Progression Loss of Response to Short-Acting Vasodilator Trial 3 1 2 Susceptibility Abnormal BMPR2 Gene Other Genetic Factors Smooth muscle hypertrophy Adventitial and intimal proliferation In situ thrombosis Adventitia Smooth muscle hypertrophy Media Intima Plexiform lesion Early intimal proliferation Gaine S. JAMA.2000;284:3160-3168.

  8. French Registry: Kaplan-Meier Survival Estimates in Combined PAH Population vs NIH-Predicted 100 Observed 80 60 Survival (%) Predicted (NIH Registry) 40 20 0 0 12 24 36 Time (months) No. at risk: All patients 56 69 98 113 120 127 133 Humbert M, et al. Circulation. 2010;122:156-163.

  9. Survival of Patients With Idiopathic PAH According to NYHA FC at Diagnosis 100 NYHA FC I/II 80 NYHA FC III 60 40 NYHA FC IV 20 N = 190 0 36 12 24 0 Time (months) FC = functional class Humbert M, et al. Circulation.2010;122:156-163.

  10. McLaughlin VV, et al. JAm Coll Cardiol. 2009;53:1573-1619.

  11. Mild PAH Apical 4-chamber view Systole in short-axis view RV IVS LV Diastole in short-axis view TR Jet

  12. Moderate PAH Disease Systole Apical 4-Chamber View TR Jet Diastole

  13. Severe PAH and RV Failure Apical 4-Chamber View Systole TR Jet Diastole

  14. Tricuspid Annular Plane Systolic Excursion (TAPSE) • Contraction of the RV is mainly longitudinal, and the tricuspid annulus displaces toward apex during systole • Imaging through lateral RV free wall with M-mode assesses longitudinal displacement (excursion) of the tricuspid annulus • Less TAPSE occurs when RV function declines • Baseline TAPSE < 1.8 cm has negative prognostic implications Forfia PR, et al. Am J Respir Crit Care Med. 2006;174:1034-1041.

  15. Presymptomatic/ Compensated Symptomatic/ Decompensating Declining/ Decompensated Progression of PAH CO Symptom Threshold PAP Right Heart Dysfunction PVR Time

  16. Role of MRI in PAH Assessment • Quantify RV size, function, viability, and interaction with LV • Evaluate pulmonary vascular structure and function • Combining volumetric and flow to pressure measurements can improve RV function and afterload assessment • Application in PAH is still in growing phase . Vonk-Noordegraaf A, et al. Eur Heart J.2007;9(suppl H):H29-34.

  17. Cardiac MRI in PH Anterior Chest Wall Left Lung LV IVS RV Liver Normal short-axis cine MRI Short-axis cine in severe PH

  18. PAH Treatment Goals • Fewer/less severe symptoms • Improved exercise capacity • Improved hemodynamics • Prevention of clinical worsening • Improved quality of life • Improved survival

  19. PAH Determinants of Risk McLaughlin V, et al. J Am Coll Cardiol. 2009;53:1573-1619.

  20. PAH Determinants of Risk (cont) McLaughlin V, et al. J Am Coll Cardiol. 2009;53:1573-1619.

  21. What Is the Optimal Treatment Strategy? Acute Vasoreactivity Testing Positive Negative Oral CCB No Sustained Response Yes Continue CCB Anticoagulate ± Diuretics ± Oxygen ± Digoxin McLaughlin V, et al. J Am Coll Cardiol. 2009;53:1573-1619.

  22. ACCF/AHA Consensus PAH Treatment Algorithm Epoprostenol or treprostinil (IV), iloprost (inhaled), ERAs or PDE5 inhibitors (oral), treprostinil (SC) ERAs or PDE5 inhibitors (oral), epoprostenol or treprostinil (IV), iloprost (inhaled), treprostinil (SC) No Reassess – considercombination therapy Atrial septostomyLung transplant Investigational protocols Acute Vasoreactivity Testing Anticoagulants ± Diuretics ± Oxygen ± Digoxin Positive Negative Oral CCB Lower risk Higher risk Sustained Response Yes Continue CCB McLaughlin VV, et al. J Am Coll Cardiol. 2009;53:1573-1619.

  23. Longitudinal Evaluation of the Patient McLaughlin V et al. JAm Coll Cardiol. 2009;53:1573-1619.

  24. Longitudinal Evaluation (cont) McLaughlin V, et al. JAm Coll Cardiol. 2009;53:1573-1619.

  25. Prostacyclin Use in REVEAL® (N = 2438) Badesch DB, et al. Chest. 2010;137:376-387.

  26. Important Prognostic Variables • French Registry • Functional class • 6-minute walk • RAP • Cardiac index • Age • Gender • Etiology • REVEAL Registry • Functional class • 6-minute walk • PVR, RAP • Vitals • BNP • Pericardial effusion • DLCO • Age • Gender • Etiology DLCO = carbon-monoxide diffusing capacity Humbert M, et al. Circulation. 2010;122:156-163. Benza RL, et al. Circulation. 2010;122:164-172.

  27. Will a Change in Important Prognostic Variables Change Outcomes? • French Registry • Functional class • 6-minute walk • RAP • Cardiac index • Age • Gender • Etiology • REVEAL Registry • Functional class • 6-minute walk • PVR, RAP • Vitals • BNP • Pericardial effusion • DLCO • Age • Gender • Etiology Humbert M, et al. Circulation. 2010;122:156-163. Benza RL, et al. Circulation. 2010;122:164-172.

  28. Effective PAH Management: Early Intervention, Regular Monitoring, and Escalation of Treatment No functional impairment Functional Capacity Progressive remodeling and right heart failure in absence of treatment Late intervention Time

  29. Effective PAH Management: Early Intervention, Regular Monitoring, and Escalation of Treatment (cont) No functional impairment Will escalation of therapy and achievement of goals improve long-term outcomes? Functional Capacity Early intervention Progressive remodeling and right heart failure in absence of treatment Late intervention Time

  30. Candidate "Goals of Therapy" • Functional class I/II • 6-minute walk distance • Hemodynamics • RAP • Cardiac output/cardiac index • BNP • ? Echocardiography

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