Dr. Bolin, May 2007


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Dr. Bolin, May 2007

  1. 1. Clinical Pathologic Conference Division of Cardiology Andrew Bolin May 11 th , 2007
  2. 2. The Case <ul><li>Chief Complaint: Irregular Heart Beat, Dyspnea and Fatigue. </li></ul><ul><li>HPI: A 74 year old white female with a history of paroxysmal atrial fibrillation for 7 years is referred to the cardiology clinic. </li></ul><ul><li>The patient had intermittent paroxysms of atrial fibrillation while taking flecainide for 5 years. In the month prior to referral, she was hospitalized twice for rapid atrial fibrillation. </li></ul>
  3. 3. The Case <ul><li>Before the first hospitalization, the patient reported symptoms of: paroxysmal nocturnal dyspnea, orthopnea and dyspnea on exertion. </li></ul><ul><li>During the first hospitalization, flecainide was discontinued and warfarin and metoprolol were started. She ruled out for an acute myocardial infarction and a dipyridamole thallium study did not demonstrate ischemia. An echo-cardiogram revealed normal left ventricular function and a mildly enlarged left atrium. </li></ul>
  4. 4. The Case <ul><li>Two weeks prior to her second hospitalization, the patient experienced the sudden onset of: fatigue, decreased endurance, irregular heart beat, constant chest pressure and profound dyspnea on exertion. </li></ul><ul><li>Prior to this illness, the patient could ambulate for thirty minutes without fatigue or dyspnea. On the day of consultation she experienced dyspnea on walking 20 yards. </li></ul><ul><li>Review of Symptoms: Positive for: 10 pound weight gain, diffuse chronic myalgias, and anxiety </li></ul>
  5. 5. Past Medical History <ul><li>Hypertension for 11 years </li></ul><ul><li>Breast Cancer: underwent left mastectomy and chemotherapy 14 years before. No radiation therapy. </li></ul><ul><li>Recurrent Urinary Tract Infections </li></ul>
  6. 6. Social History Family History <ul><li>Married to retired Methodist minister </li></ul><ul><li>Two adult children </li></ul><ul><li>No alcohol, tobacco or illicit drugs </li></ul><ul><li>No unusual exposures </li></ul><ul><li>Mother died of congestive heart failure at age 79 </li></ul><ul><li>Sister breast cancer </li></ul><ul><li>Sister “bone cancer” </li></ul>
  7. 7. Medications <ul><li>Metoprolol 25 mg BID </li></ul><ul><li>Coumadin </li></ul><ul><li>Hydrochlorothiazide 25 mg Daily </li></ul><ul><li>Celexa 10 mg Daily </li></ul><ul><li>Aspirin 81 mg Daily </li></ul><ul><li>Bactrim DS three days per week </li></ul><ul><li>Allergies: Macrodantin </li></ul>
  8. 8. Physical Exam <ul><li>Blood Pressure 148/78 (in both arms) Pulse 90 Respirations 18 </li></ul><ul><li>Neck: normal jugular venous pressure, symmetric brisk carotid upstrokes without bruits, no lymphadenopathy </li></ul><ul><li>Lungs: clear to auscultation, dullness to percussion in right base </li></ul>
  9. 9. Physical Exam <ul><li>Cardiac: normal point of maximal impulse, normal S1, physiologically split S2, no murmurs, rubs or gallops, strong and symmetric peripheral pulses </li></ul><ul><li>Benign Abdominal Exam </li></ul><ul><li>Extremities: no clubbing, cyanosis or edema </li></ul><ul><li>Musculoskeletal: tenderness to palpation in trapezius and shoulder girdle bilaterally </li></ul>
  10. 10. Studies <ul><li>Electrocardiogram: atrial fibrillation, incomplete right bundle branch block and nonspecific ST-T abnormalities </li></ul><ul><li>Chest X-ray: possible right pleural effusion </li></ul><ul><li>Transesophageal Echocardiogram: unusual thickening of left atrial walls, small pericardial effusion </li></ul>
  11. 11. Studies <ul><li>Chest CT: pericardial effusion and bilateral pleural effusions, biapical pleuroparenchymal scarring </li></ul><ul><li>Cardiac MRI: atrium unremarkable </li></ul><ul><li>Labs: CBC, E-group, BUN, Creatinine, TSH, Troponin and CK were all within normal limits. </li></ul>
  12. 12. Right Thoracentesis <ul><li>Consistent with Transudative Effusion </li></ul><ul><li>WBC 485, Lymphocytes 89% </li></ul><ul><li>Flow Cytometry: Consistent with a Reactive Effusion. </li></ul><ul><li>Cytology: No Malignant Cells </li></ul>
  13. 13. Case Summary <ul><li>Elderly White Female with A-fib </li></ul><ul><li>Progressive Symptoms of CHF Over Weeks Without Echocardiographic Evidence of LV Dysfunction </li></ul><ul><li>Transudative Lymphocytic Bilateral Pleural Effusions </li></ul><ul><li>Biapical Pleuroparenchymal Scarring </li></ul><ul><li>Pericardial Effusion Without Evidence of Tamponade </li></ul><ul><li>Incomplete Right Bundle Branch Block </li></ul><ul><li>Abnormal Left Atrial Walls </li></ul>
  14. 14. Case Summary <ul><li>The Patient Has a Pathologic Process Involving the Pericardium and Myocardium that is Not Related to Valvular or Ischemic Disease. Additionally, Noted is an Echocardiogram Demonstrating Highly Abnormal Left Atrial Walls. </li></ul>
  15. 15. Causes of Lymphocytic Pleural Effusions <ul><li>CHF (Only Transudate) </li></ul><ul><li>Neoplasm </li></ul><ul><li>Fungal Infection </li></ul><ul><li>TB </li></ul><ul><li>Sarcoidosis </li></ul><ul><li>Rheumatoid Arthritis </li></ul><ul><li>Hepatic Hydrothorax </li></ul><ul><li>Yellow Nail Syndrome </li></ul><ul><li>Chylothorax </li></ul>
  16. 16. Causes of Left Atrial Enlargement <ul><li>Valvular Disease </li></ul><ul><li>Myocardial Infarction </li></ul><ul><li>Obstructive Cardiomyopathy </li></ul><ul><li>Hypertension </li></ul><ul><li>Infiltrative Cardiomyopathy </li></ul><ul><li>Inflammatory Cardiomyopathy </li></ul><ul><li>Primary or Metastatic Tumors </li></ul>
  17. 17. Differential Diagnosis for Pericardial Effusion <ul><li>Infectious </li></ul><ul><li>Malignant </li></ul><ul><li>Autoimmune </li></ul><ul><li>Cardiomyopathy </li></ul><ul><li>Drug Induced </li></ul><ul><li>Cardiac </li></ul><ul><li>Constrictive Pericarditis </li></ul><ul><li>Radiation </li></ul><ul><li>Trauma </li></ul><ul><li>Metabolic: Hypothyroidism Uremia Ovarian- Hyperstimulation- Syndrome </li></ul>
  18. 18. Pericardial Effusion <ul><li>All Causes of Pericardial Effusion Can Also Cause Pleural Effusions. </li></ul><ul><li>All Causes of Pericardial Disease Can Also Include the Myocardium to Varying Degrees. Termed Myopericarditis or Perimyocarditis. </li></ul>
  19. 19. Infectious Pericardial Effusion <ul><li>Viral </li></ul><ul><li>Pyogenic/Bacterial </li></ul><ul><li>Fungal </li></ul><ul><li>Parasitic </li></ul><ul><li>Tuberculous </li></ul><ul><li>Any Infectious Agent Can Affect the Pericardium </li></ul>
  20. 20. Viral Pericarditis <ul><li>Most Common Infectious Pericarditis </li></ul><ul><li>Usually Cause Acute Pericarditis with: Fever, Rub, Typical ECG Changes, Leukocytosis, </li></ul><ul><li>3:1 Male to Female Ratio, Predominately in Young Adults </li></ul><ul><li>Usually Self Limited Resolving Within 2 Weeks </li></ul><ul><li>Preceded by Upper Respiratory or GI Illness by 1-3 Weeks </li></ul><ul><li>50% Have Recurrence Within 8 Months </li></ul><ul><li>Common Viruses: Enteroviruses, Coxsackie A&B, Adenovirus, Rhinovirus, Echovirus type 8, and Influenza </li></ul><ul><li>Diagnosis of Exclusion </li></ul>
  21. 21. Pyogenic/Bacterial Pericarditis <ul><li>Fulminant Course </li></ul><ul><li>Symptoms Arise Over a Few Days and Usually Lead to Tamponade and Sepsis </li></ul><ul><li>Commonly Pneumococus, Staph, Strep </li></ul><ul><li>Typically Associated with High Fever (Virtually All Cases), Toxicity, Tachycardia, Rub </li></ul><ul><li>Maybe More Insidious Course in the Elderly </li></ul><ul><li>Usually Require Systemic Antibiotics and Pericardial Drainage With Exploration </li></ul><ul><li>In the Antibiotic Era Usually Associated with Dialysis, Thoracic Surgery and Chemotherapy. </li></ul>
  22. 22. Fungal Pericarditis <ul><li>Usually Occurs in Immunocompromised </li></ul><ul><li>Histoplasma: Usually Young Males in Endemic Areas, Often Benign Course Remitting Spontaneously, May Have Mediastinal Lymphadnopathy and Pleural Effusions </li></ul><ul><li>Coccidiomycosis: Usually in Patients with Widely Disseminated Infection </li></ul><ul><li>Candida & Aspergillus: Opportunistic, Necrotizing and Leading to Thrombosis </li></ul>
  23. 23. Parasitic Pericarditis <ul><li>Clinical Course Resembles that of Pyogenic Pericarditis </li></ul><ul><li>Residents or Travelers to Endemic Areas </li></ul><ul><li>Usually Have an Identifiable Primary Source i.e. Liver or Lung </li></ul><ul><li>Eosinophillia </li></ul>
  24. 24. Primary Tumors <ul><li>75% Benign, 50% of Benign Tumors Are Myxomas </li></ul><ul><li>25% Malignant </li></ul><ul><li>95% of Malignant Tumors Are Sarcomas </li></ul><ul><li>5% of Malignant Tumors Are Lymphomas </li></ul><ul><li>Symptoms Related to Location and Size </li></ul><ul><li>The Pathology of the Tumor Can Be Speculated by Appearance on Imaging and Anatomic Location </li></ul>
  25. 25. Myxomas <ul><li>Occur in 3 rd -6 th Decades </li></ul><ul><li>Female Predominance 60-70% </li></ul><ul><li>80% Found in the Left Atrium </li></ul><ul><li>Presentation: Obstruction of Mitral or Tricuspid Valve 67% (Occasionally Positional), Embolization 29%, 15% of Cases Have Audible Tumor Plop </li></ul><ul><li>Constitutional Symptoms: Fever, Fatigue, Weight Loss, Myalgias, and Arthralgias </li></ul><ul><li>Not Commonly Associated With Pericardial Effusion </li></ul>
  26. 26. Myxomas <ul><li>Echo Demonstrates Endocardial Mass, Rarely May Be Intramural </li></ul><ul><li>Treatment is Surgical Resection, Though Myxoma May Recur 1-5% </li></ul><ul><li>10% May Be Familial </li></ul>
  27. 27. Benign Papillary Fibroelastomas <ul><li>2 nd Most Common Benign Tumor </li></ul><ul><li>Predominantly Affect Valves, Account for 75% of Valvular Tumors </li></ul><ul><li>Often Asymptomatic, But May Present with Dyspnea, Embolization, or Chest Pain </li></ul><ul><li>Visible By Echo as Mobile Endocardial Mass </li></ul>
  28. 28. Other Benign Cardiac Tumors <ul><li>All Are Rare </li></ul><ul><li>Rhabdomyomas: Most Present in Childhood </li></ul><ul><li>Fibromas: Most Present in Childhood </li></ul><ul><li>Hemangiomas: Have Characteristic Findings on Imaging </li></ul><ul><li>Lipomas: Have Characteristic Findings on Imaging </li></ul><ul><li>Teratomas: Have Characteristic Findings on Imaging, Most Present in Childhood </li></ul>
  29. 29. Malignant Sarcomas <ul><li>Angiosarcomas: Most “Common” Malignant Primary Cardiac Tumor </li></ul><ul><li>Men:Women 3:1, 65-90% Have Metastases at Presentation </li></ul><ul><li>80% Occur In the Right Atrium (Other Sarcomas Are Usually in the Left Atrium) </li></ul>
  30. 30. Malignant Sarcomas <ul><li>Rhabdomyosarcoma: 2 nd Most “Common” Malignant Primary Cardiac Tumor </li></ul><ul><li>Can Occur at Any Age </li></ul><ul><li>Most Have Aggressive Features Seen on Imaging (Invasion of Local Structures) </li></ul>
  31. 31. Primary Cardiac Lymphomas <ul><li>By Far Most Commonly Occur in the Right Atrium </li></ul><ul><li>More Common in AIDS and Immunocompromised Patients </li></ul><ul><li>Most Are Fatal Shortly After Diagnosis </li></ul>
  32. 32. Metastatic Tumors <ul><li>20 Times More Common than Primary Tumors </li></ul><ul><li>Lung, Breast, Leukemia, Lymphoma, Melanoma, Kaposi’s Sarcoma </li></ul><ul><li>Presenting Symptoms Based on Location of Tumor, Usually Causes Pericardial Effusion </li></ul><ul><li>If the Patient Is Symptomatic the Tumor Is Virtually Always Visible on Echo, CT or MRI </li></ul><ul><li>Most Patients Have Mediastinal Lymphadnopathy </li></ul><ul><li>Most Have Aggressive Features Seen on Imaging (Invasion of Local Structures) </li></ul>
  33. 33. Autoimmune Pericardial Effusion <ul><li>Rheumatoid Arthritis </li></ul><ul><li>SLE </li></ul><ul><li>Scleroderma </li></ul><ul><li>Rheumatic Fever </li></ul><ul><li>Wegener’s Granulomatosis </li></ul><ul><li>Ankylosing Spondylitis </li></ul><ul><li>Inflammatory Bowel Disease </li></ul>
  34. 34. Cardiomyopathies <ul><li>Ischemic </li></ul><ul><li>Dilated </li></ul><ul><li>Hypertrophic </li></ul><ul><li>Valvular </li></ul><ul><li>Hypertensive </li></ul><ul><li>Restrictive or Infiltrative </li></ul><ul><li>Inflammatory </li></ul>
  35. 35. Restrictive Cardiomyopathy <ul><li>Excessively Rigid Ventricular Walls Cause Diastolic Dysfunction </li></ul><ul><li>Systolic Function Is Not Impaired </li></ul><ul><li>Commonly Present with Dyspnea, Fatigue and Chest Pain </li></ul><ul><li>Commonly Associated with A-Fib </li></ul><ul><li>Difficult to Distinguish Clinically from Constrictive Pericarditis </li></ul>
  36. 36. Restrictive Cardiomyopathy <ul><li>Storage Diseases </li></ul><ul><li>Infiltrative </li></ul><ul><li>Endomyocardial </li></ul>
  37. 37. Restrictive Cardiomyopathy Inherited Storage Disorders <ul><li>Hemochromatosis: Deposition of Iron in Liver, Pancreas, Gonads and Myocardium </li></ul><ul><li>Fabry: Intracellular Accumulation of Glycosphingolipids in the Skin, Kidneys and Myocardium </li></ul><ul><li>Gaucher: Accumulation of Cerebrosides in the Spleen, Liver, Bone Marrow, Lymph Nodes, Brain and Myocardium </li></ul><ul><li>Glycogen Storage Diseases: Survival to Adulthood Uncommon Except in Type III, Where Infiltration of the Myocardium is Usually Not Clinically Relevant </li></ul>
  38. 38. Restrictive Cardiomyopathy Infiltrative Disorders <ul><li>Amyloid </li></ul><ul><li>Sarcoid </li></ul><ul><li>Carcinoid Heart Disease: Serotonin Producing Tumor Causing Cutaneous Flushing, Diarrhea, Bronchoconstriction and Right Sided Endocardial Plaques Composed of Fibrous Tissue </li></ul>
  39. 39. Restrictive Cardiomyopathy Endocardial Fibrotic Diseases <ul><li>Endomyocardial Disease (Africa, Eosinophilia) </li></ul><ul><li>Loffler Endocarditis (Eosinophilia) </li></ul><ul><li>Endomyocardial Fibrosis (Africa) </li></ul>
  40. 40. Myocarditis <ul><li>Primary </li></ul><ul><li>Lymphocytic </li></ul><ul><li>Acute Rheumatic Fever </li></ul><ul><li>Eosinophilic </li></ul><ul><li>Lyme </li></ul><ul><li>Chagas </li></ul><ul><li>HIV </li></ul><ul><li>Drugs </li></ul><ul><li>Radiation </li></ul><ul><li>Giant Cell </li></ul>
  41. 41. Myocarditis <ul><li>Inflammation of the Myocardium Associated with Injury to Myocytes </li></ul><ul><li>Presents as an Acute Illness, Often Congestive Heart Failure, Chest Pain and Fatigue </li></ul><ul><li>Moderate Elevation of Cardiac Biomarkers, Nonspecific ST-T Changes, Arrhythmias, Nonspecific Echo </li></ul><ul><li>Diagnosis by Endomyocardial Biopsy </li></ul>
  42. 42. Myocarditis <ul><li>Primary or Acute Myocarditis Associated with Viral Infection (20 Viruses). Most Commonly Enteroviruses (Coxsackie). Viral Etiology Suggested by Antecedent Upper Respiratory or GI Illness. </li></ul><ul><li>Acute Viral Myocarditis is Associated with Lymphocytic Infiltrate </li></ul>
  43. 43. Inflammatory Myocarditis <ul><li>Lymphocytic Myocarditis: Most Will Improve Over 1-6 Months, a Minority Will Fail to Clear a Cardiotropic Virus or Develop Persistent Inflammation That Leads to Chronic Cardiomyopathy, Heart Block or Ventricular Arrhythmias. </li></ul><ul><li>Myocarditis Treatment Trial: Prospective Randomized, Double-Blind, Placebo-Controlled Trial of Prednisone and Cyclosporine or Azathioprine for the Treatment of Biopsy Proven Lymphocytic Myocarditis in Acute CHF. There Was No Benefit from Immunosuppression. </li></ul><ul><li>Immune Modulation for Acute Cardiomyopathy: Evaluated the Role of IVIG and Found no Benefit. </li></ul><ul><li>May Lead to Dilated Cardiomyopathy </li></ul>
  44. 44. Inflammatory Myocarditis <ul><li>Acute Rheumatic Fever: Pancarditis Occurring in 3% of Untreated Streptococal A Pharyngeal Infections </li></ul><ul><li>Hypersensitivity Myocarditis (Drug Induced): Tricyclics, Penicillins, Antipsychotics, Present with Rash & Fever </li></ul><ul><li>Eosinophilic Myocarditis: Occurs in Conjunction with: Hypereosinophilic Syndrome, Churg-Strauss Syndrome & Loffler’s Endomyocardial Fibrosis. Peripheral Eosinophilia </li></ul><ul><li>Lyme Myocarditis: Borrelia burgdorferi, Often Develop Heart Block or Arrhythmias, </li></ul><ul><li>Chagas Cardiomyopathy: Trypanosoma cruzi (Protozoan), Endemic to Central & South America </li></ul><ul><li>HIV Myocarditis </li></ul>
  45. 45. Drug Induced Pericardial Effusion <ul><li>Procainamide </li></ul><ul><li>Hydralazine </li></ul><ul><li>Phenytoin </li></ul><ul><li>Isoniazide </li></ul><ul><li>Minoxidil </li></ul><ul><li>Methysergide ergot derivative </li></ul><ul><li>Phenylbutazone/ NSAID </li></ul><ul><li>Anticoagulants </li></ul><ul><li>Cromolyn Sodium </li></ul><ul><li>Dantrolene </li></ul><ul><li>Thrombotics </li></ul><ul><li>Penicillin </li></ul><ul><li>Doxorubicin/ Adriamycin </li></ul><ul><li>Cytoxan/ Cyclophosphamide </li></ul>
  46. 46. Cardiac Pericardial Effusions <ul><li>Myocardial Infarction </li></ul><ul><li>Postmyocardial Infarction “Dressler’s Syndrome” </li></ul><ul><li>Trauma </li></ul><ul><li>Aortic Dissection with Hemorrhage into Pericardial Space </li></ul><ul><li>Postpericardiotomy </li></ul>
  47. 47. Constrictive Pericarditis <ul><li>No Pericardial Thickening Was Noted on Imaging </li></ul><ul><li>Few Case Reports of Constriction Without Pericardial Thickening </li></ul><ul><li>Would Not Account for Abnormal Left Atrium Seen on Echo </li></ul>
  48. 48. Possible Etiologies for This Case <ul><li>Tuberculous Pericarditis </li></ul><ul><li>Cardiac Sarcoidosis </li></ul><ul><li>Cardiac Amyloidosis </li></ul><ul><li>Giant Cell Myocarditis </li></ul>
  49. 49. Tuberculous Pericarditis <ul><li>4-10% of All Acute Pericarditis is Caused by TB (Reported up to 80% in Some 3 rd World Countries) </li></ul><ul><li>1-4% of Patients with TB Have Pericardial Involvement </li></ul><ul><li>Etiology of 20% of Constrictive Pericarditis </li></ul><ul><li>Cases 93% of all Pericardial Effusions in HIV Patients </li></ul>
  50. 50. Four Pathologic Stages of TB Pericarditis <ul><li>I-Dry: Fibrin Deposition & Granulomatous Reaction. Usually Clinically Silent </li></ul><ul><li>II-Effusive: Serous Fluid Accumulation Caused by Hypersensitivity to Tuberculoprotein and Impaired Resorption </li></ul><ul><li>III- Absorptive: Effusion Resolves and Fibrous Tissue Replaces Granulomas, Pericardium Thickens </li></ul><ul><li>IV-Constrictive: Parietal Pericardial Calcification </li></ul>
  51. 51. Presentation of Tuberculous Pericarditis <ul><li>Dyspnea 45-90% </li></ul><ul><li>Chest Pain 40-75% </li></ul><ul><li>Orthopnea 20-65% </li></ul><ul><li>Distant Heart Sounds 25-55% </li></ul><ul><li>Rub 30-85% </li></ul><ul><li>Cough 50-95% </li></ul><ul><li>Fever 80-100% </li></ul>
  52. 52. Presentation of Tuberculous Pericarditis <ul><li>50% of Patient Have Slowly Progressive Insidious Presentation </li></ul><ul><li>95% Have Cardiomegally, 30% Have Active Pulmonary TB, 39-71% Have Pleural Effusions L>R. Bilateral Pleural Effusions More Common than Rub. </li></ul>
  53. 53. Tuberculous Pericarditis <ul><li>“ Because of the variable and nonspecific features of TB pericarditis, establishing the diagnosis on clinical grounds alone is impossible.” D.H. Spodick </li></ul><ul><li>Diagnosis by Pericardial Fluid or Biopsy: Positive AFB Smear, Culture, Caseating Granulomas, TB DNA PCR </li></ul>
  54. 54. Treatment Tuberculous Pericarditis <ul><li>Antibiotics: Isoniazid, Rifampin, Pyrazinamide, Ethambutol </li></ul><ul><li>Corticosteroids: Reduces Mortality and Need for Subsequent Pericardiocentisis </li></ul>
  55. 55. Cardiac Sarcoidosis <ul><li>Causes Restrictive Cardiomyopathy </li></ul><ul><li>Clinically Relevant in 5%, 25% Incidentally Noted at Autopsy </li></ul><ul><li>69% of Clinically Relevant Cardiac Sarcoidosis Patients Have No Other Manifestations of the Disease at Presentation </li></ul><ul><li>Presentation: Complete Heart Block (Be Suspicious in Young Patients With Complete Heart Block), Ventricular Arrhythmia, Congestive Heart Failure Systolic or Diastolic Dysfunction </li></ul>
  56. 56. Cardiac Sarcoidosis <ul><li>Echo Usually Reveals Hyperechogenic Left Ventricular Walls, May Develop Ventricular Aneurysm From Myocardial Scar Tissue </li></ul><ul><li>Diagnosis Based on Clinical Suspicion in a Patient with Known Sarcoid or by Endomyocardial Biopsy in a Patient with No Evidence of Sarcoid in Other Organs. Biopsy Consistent with Sarcoid 30% of Cases. </li></ul><ul><li>Treat with Steroids </li></ul>
  57. 57. Cardiac Amyloidosis <ul><li>Causes Restrictive Cardiomyopathy </li></ul><ul><li>Myocyte Destruction and Replacement with Amyloid Fibrils (Beta Pleated Sheets) Leading to Progressive Thickening of the Ventricular Walls. </li></ul><ul><li>More Common in Primary (AL) Amyloidosis, a Plasma Cell Dyscrasia Where Immunoglobulins Form Amyloid Protein </li></ul>
  58. 58. Cardiac Amyloidosis <ul><li>EKG: Often Have Bundle Branch Block, Low Voltage Despite Thick Ventricles, or A-Fib </li></ul><ul><li>Most Patients with Amyloidosis, Even Those with no Cardiac Symptoms, Have Abnormal Echocardiograms with: Ventricular Wall Thickening (70%), Isolated Septal Wall Thickening (30%), Diastolic Dysfunction (57%), Systolic Dysfunction (27%), Pericardial Effusion (40%), Myocardial Sparkling Pattern in 2D (27%) </li></ul>
  59. 59. Cardiac Amyloidosis <ul><li>Diagnosis with Tissue Biopsy Revealing Amyloidosis, Often Abdominal Fat Pad </li></ul><ul><li>Prognosis Less Than 1 Year, Survival at 5 Years <5% </li></ul><ul><li>Treatment: Primary Amyliodosis Limited Benefit of Alkylating Agents </li></ul><ul><li>Secondary Amyloidosis: Treat Underlying Disease </li></ul>
  60. 60. Giant Cell Myocarditis <ul><li>Causes Inflammatory Cardiomyopathy </li></ul><ul><li>Unknown Etiology </li></ul><ul><li>Causes Progressive Left Ventricular Failure and Arrhythmias, Over Weeks to Months </li></ul><ul><li>Rare, 80 Cases Reported by 1997 </li></ul><ul><li>Average Age 43 (Infants to Elderly) Usually Affects Otherwise Healthy Patients </li></ul><ul><li>Equal Predominance of Men and Women </li></ul><ul><li>90% Are Caucasian </li></ul>
  61. 61. Giant Cell Myocarditis <ul><li>Diagnosed by Endomyocardial Biopsy Demonstrating: Diffuse Myocardial Necrosis with Multinucleated Giant Cells in the Absence of Sarcoid Like Granuloma. Inflammatory Infiltrate in Close Apposition to Myocyte Necrosis. Negative Culture and Stains for Infection, No Viral Particles on Electron Microscopy. </li></ul>
  62. 62. Giant Cell Myocarditis <ul><li>75% Present with CHF, 25% with Ventricular Arrhythmias, Sudden Cardiac Death (50%), Heart Block, Diffuse ST-T Abnormalities on EKG. </li></ul><ul><li>May be Localized Rather Than Diffuse in Earlier Stages. There Are Case Reports of Giant Cell Myocarditis Affecting Only the Atria. </li></ul><ul><li>20% Have Other Autoimmune Disease: Hashimoto Thyroiditis, RA, MG, Takayasu Arteritis, Alopecia, Vitiligo, Pernicious Anemia, Crohn’s Disease, Ulcerative Colitis, ITP, Celiac Disease </li></ul>
  63. 63. Giant Cell Myocarditis <ul><li>Survival 5.5 Months After Onset of Symptoms, Normally Succumb to CHF or Arrhythmia </li></ul><ul><li>Immunosuppresive Therapy with Corticosteroids, Cyclosporin, Azathioprine Increases Survival to 12 Months, Case Reports of Dramatic Improvement with Immunotherapy </li></ul><ul><li>Cardiac Transplantation (with 25% recurrence), 71% Survival at 5 Years </li></ul><ul><li>The Giant Cell Myocarditis Treatment Trial and Registry, Randomized Controlled Trial of T-Cell Targeted Treatment with Muromonab-CD3 and Cyclosporine </li></ul>
  64. 64. Possible Etiologies for This Case <ul><li>Tuberculous Pericarditis </li></ul><ul><li>No Fever, No Exposure, Not Immunocompromized, Would Not Explain Abnormal Atrium </li></ul><ul><li>Cardiac Sarcoidosis </li></ul><ul><li>No Evidence of Systemic Sarcoid, Normal Left Ventricular Walls </li></ul><ul><li>Cardiac Amyloidosis </li></ul><ul><li>No Low Voltage EKG, No Ventricular Wall Thickening </li></ul><ul><li>Giant Cell Myocarditis </li></ul><ul><li>Time Course Is Consistant with Our Patient, Could Account for Symptoms of CHF, Effusions, and Abnormal Left Atrium </li></ul>
  65. 65. Diagnosis <ul><li>Giant Cell Myocarditis </li></ul><ul><li>Diagnostic Test of Choice: </li></ul><ul><li>Endomyocardial Biopsy </li></ul>
  66. 66. References <ul><li>Spodick DH. The Pericardium. New York: Marcel Dekker; 1997 </li></ul><ul><li>Braunwald E, Fauci AS. Harrison’s Textbook of Internal Medicine. McGraw-Hill; 2001 </li></ul><ul><li>Up To Date, 2007 </li></ul><ul><li>Garay S, Rom WN. Tuberculosis. Boston: Libble, Brown & CO; 1996 </li></ul><ul><li>Hall HD, Hammar SP. Pulmonary Pathology. New York: Springer-Verlag; 1994 </li></ul><ul><li>Roth JA, Ruckdeschel JC. Weisenburger, T. H. Thoracic Oncology. Philadelphia: W.B. Saunders Company; 1995 </li></ul><ul><li>Murphy JG, Lloyd MA. Mayo Clinic Cardiology. Rochester: Mayo Clinic Scientific Press; 2007 </li></ul><ul><li>Zipes DP, Libby P, Bonow RO, Braunwald E. Braunwald’s Heart Disease. Philadelphia: Elsevier Saunders; 2005 </li></ul><ul><li>Silver MD, Gotlieb AI, Schoen FJ. Cardiovascular Pathology. Philadelphia: Churchill Livingstone; 2001 </li></ul><ul><li>Cooper LT, Berry GJ, et al: Idiopathic Giant-Cell Myocarditis. NE J Medicine, June, 1997 </li></ul><ul><li>Frustaci A, Chimenti C, et al: Giant Cell Myocarditis, Responding to Immunosuppressive Therapy. Chest, March, 2000 </li></ul><ul><li>Cooper LT: Giant Cell Myocarditis: Diagnosis and Treatment. Herz, May, 2000 </li></ul><ul><li>Rosenstein ED, Zucker MJ, et al: Giant Cell Myocarditis: Most Fatal of Autoimmune Diseases. Seminars in Arthrithritis and Rheumatism, August, 2000 </li></ul>
  67. 67. Special Thanks To: <ul><li>Dr. Erwin </li></ul><ul><li>Dr. Starr </li></ul><ul><li>Dr. Hurley </li></ul>