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Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

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Presentation by Dr. Jonathan R. Lindner MD at the 13th Annual Cheri Woo Scleroderma Education Seminar on March 8, 2014 in Portland, Oregon. The seminar is a free public service hosted by the Oregon Chapter of the Scleroderma Foundation.

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Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

  1. 1. Cardiovascular Manifestations Systemic Sclerosis Jonathan R Lindner, MD M Lowell Edwards Professor of Cardiology Knight Cardiovascular Institute Oregon Health and Sciences University
  2. 2. Pathophysiology of SSc Inflammation Thrombosis Vasoconstriction Vessel wall hyperplasia Tissue injury and fibrosis Hypoxia
  3. 3. Cardiovascular Manifestations of SSc Valve disease? Pericardial disease Cor Pulmonale Myocarditis/ Cardiomyopathy Hypertrophy Conduction abnormalities Microvascular disease
  4. 4. Cardiac Complications: Scope of the Problem • Cardiac symptoms often go unnoticed • Symptoms attributed to lung or musculoskeletal disease • Recognized primary cardiac involvement in 20- 25% of those with diffuse SSc (much higher on autopsy series) • Presence of cardiac involvement is a poor prognostic feature and usually occurs in those with more advanced disease
  5. 5. Pulmonary Hypertension in SSc • High blood pressure in the lung arterial circulation. • Severe pulmonary hypertension affects 10- 12% of patients with SSc • Mortality 50% within 3 yrs • Most of the mortality is directly related to effects on the right ventricle • In those with long term survival there is considerable morbidity from the effects on the right ventricle
  6. 6. Koch ET, et al. Br J Rheumatol 1996;35:989 PAH and Survival in SSc
  7. 7. Right Heart Failure in SSc PAH • For any given increase in pulmonary pressure, the deleterious effect on the right heart is greater in SSc than in other diseases of PAH
  8. 8. Right Heart Failure in SSc PAH Right Heart FailureNormal
  9. 9. Secondary Tricuspid Regurgitation
  10. 10. Right Heart Failure: Symptoms and Complications • Fatigue, shortness of breath, exercise intolerance • Severe edema (swelling of legs, abdomen) • Liver dysfunction and cirrhosis • Gastrointestinal symptoms of bowel edema • Heart rhythm disorders (atrial fibrillation, ventricular tachycardia)
  11. 11. Risk Factors for PAH and Right Heart Failure • Late age of onset of SSc • Pre-existing lung disease, smoking • Raynaud’s • Certain antibodies (anti-U3RNP) • More severe SSc
  12. 12. How to Diagnose PAH
  13. 13. Treatment Options Pulmonary vasodilators: • Prostacyclin agonists • Phosphodiesterase-E5 inhibitors • Endothelin antagonists • Calcium channel blockers Immunosuppressive therapy Diuretics Oxygen Digoxin Lung transplantation Experimental: Ivadrabine, Tyrosine kinase inhibitors
  14. 14. Left Ventricular Dysfunction in SSc  Causes: - Heart inflammation/fibrosis - Small vessel dysfunction - “Raynaud’s” of the heart vessels  Occurs in approximately 5% of patients with SSc  Higher incidence with advanced age, hypertension, kidney disease, pulmonary disease, digital ulcers
  15. 15. Left Ventricular Dysfunction Normal Dysfunction
  16. 16. Myocyte Damage from Microvascular Disease
  17. 17. Myositis and Vasculitis Histology DE-Gd-MRI
  18. 18. Symptoms of LV Dysfunction • Shortness of breath • Fatigue, weakness • Cough, frothy sputum • Inability to sleep flat Symptoms of Coronary Vasospasm • Chest pain, acute shortneess of breath Symptoms of Myositis • Chest pain, fever, fatigue
  19. 19. Occult LV Dysfunction: Common First Manifestations • Stroke • Heart rhythm disturbance (atrial fibrillation, ventricular fibrillation) • Complications of poor blood flow (kidney dysfunction, confusion)
  20. 20. Diagnosis • Clinical suspicion • Echocardiogram • Once LV dysfunction is found, there is a workup for causes not related to SSc • Evaluation for myocarditis and microvascular dysfunction
  21. 21. RNI Detection of Perfusion Defects Stress Rest
  22. 22. MCE Evaluation of the Microcirculation
  23. 23. Treatment • Diuretics • ACE-inhibitors; Angiotensin receptor blocking agents • Beta blockers??? • If vasospasm suspected: calcium channel blocking vasodilators or long- acting nitroglycerine • ICD • Cardiac rehabilitation • If myositis: immunosupppressive therapy
  24. 24. Diastolic Heart Failure • No problem with the heart squeeze • Problem exists with the relaxation of the heart between squeezes • Due to fibrosis and enlarged heart cells that occurs with inflammation, early microvascular disease, renal disease, and hypertension
  25. 25. Diastolic Heart Failure
  26. 26. Pericarditis Sharp chest pain Positional pain Respiratory variation Fevers Shortness of breath Palpitations Symptoms
  27. 27. Pericarditis in SSc  Symptomatic pericarditis in 5-12%  Detected by imaging/autopsy in 33-70%  Common in limited scleroderma (CREST)  More common if there is PAH  Treatment with NSAIDs and/or steroids  Complications of disease: • Effusions (tamponade) • Constriction
  28. 28. Pericardial Effusion
  29. 29. Pericardial Effusion  Symptoms: chest pain, shortness of breath, dizziness, fatigue, swelling  When severe  cardiac collapse (tamponade)  Hemodynamically significant effusion in 10% of those with pericarditis  Can also be associated with renal disease
  30. 30. Pericardial Effusion: Detection 1. Clinical suspicion 2. Physical exam 3. Imaging
  31. 31. Pericardial Constriction • Encasement of the heart • Symptoms: fatigue, chest pain, swelling • Abdominal distention • Atrial fibrillation
  32. 32. Pericardial Constriction
  33. 33. Treatment for Complications • Drain fluid if it is causing more than mild symptoms or endangering heart function • For constriction, diuretics to unload the heart • Consider immunosuppressive therapy for constriction or refractory/recurrent effusion
  34. 34. What Does This Mean for You? 1. Awareness that there are cardiac manifestations in SSc is the first and most important step to discovering cardiovascular disease 2. Echocardiography is a common diagnostic test – it is generally part of the routine screening for pulmonary hypertension 3. More severe disease should lead to more frequent screening 4. Do not discount symptoms of shortness of breath, extreme fatigue, dizziness, chest pain 5. Aggressive treatment of hypertension 6. Other risk factor modification (exercise, smoking cessation, diet)

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