Myocarditis and Pericarditis
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1 Myocarditis and Pericarditis Elias V. Haddad, M.D., FACC Advances in Cardiovascular Care 2012 Kingston, Jamaica December 7, 2012
2 Financial Disclosures No disclosures relevant to this presentation
3 Learning Objectives Recognize the major etiologies, clinical manifestations and treatment modalities in myocarditis Discuss the role of endomyocardial biopsy (EMB) in the work-up of myocarditis Summarize a modern management strategy for acute and relapsing pericarditis
4 Myocarditis Pericarditis Myopericarditis
5 Definition of Myocarditis Inflammation of the myocardium An inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent myocytes Myocardial inflammation that leads to progressive heart failure and/or arrhythmia
6 A Simple Problem That is often under-diagnosed and under-treated
7 Etiologies of Myocarditis Viral Enterovirus Adenovirus Coxsackievirus HCV HIV Parvovirus Rabies virus Yellow fever Bacterial Fungal Mycobacterial Streptococcal species (GAS) Mycoplasma pneumoniae Treponema pallidum Aspergillus Candida Coccidiodes Cryptococcus Histoplasma Mucormycosis Protozoal Trypanosoma cruzi (Chagas) Toxins Anthracyclines Cocaine Interleukin-2 Hypersensitivity Sulfonamides Cephalosporins Diuretics Digoxin TCA Dobutamine Methyldopa Spironolactone Immunologic syndromes Churg-Strauss Inflammatory bowel disease Giant Cell myocarditis Diabetes mellitus Sarcoidosis Systemic lupus erythematosus Thyrotoxicosis Takayasu s arteritis Wegener s granulomatosis
8 Etiologies of Myocarditis Viral Enterovirus Adenovirus Coxsackievirus HCV HIV Smallpox Vaccination Bacterial Mycobacterial Streptococcal species Mycoplasma pneumoniae Treponema pallidum Fungal Aspergillus Candida Coccidiodes Cryptococcus Histoplasma Protozoal Trypanosoma cruzi (Chagas) Parasitic Schistosomiasis Larva migrans Toxins Anthracyclines Cocaine Interleukin-2 Hypersensitivity Sulfonamides Cephalosporins Diuretics Digoxin TCA Dobutamine Immunologic syndromes Churg-Strauss Inflammatory bowel disease Giant Cell myocarditis Diabetes mellitus Sarcoidosis Systemic lupus erythematosus Thyrotoxicosis Takayasu s arteritis/kawasaki arteritis Wegener s granulomatosis
9 Etiologies of Myocarditis Meta-analysis of 12 studies performing PCR on biopsy specimens (N=289) 11 of 12 studies found a significantly increased enterovirus RNA in cases vs controls Odds ratio of 4.4 Baboonian Heart 2007
10 Etiologies of Myocarditis Multicenter study of 624 patients with biopsyproven myocarditis 38% found to have viral genome (adenovirus, enterovirus, cytomegalovirus) in biopsy specimens Bowles JACC 2003
11 Etiologies of Myocarditis Department of Defense Smallpox Vaccination Clinical Evaluation Unit Increased incidence of myocarditis in 2002 Incidence of 7.8/100,000 Eckart JACC 2004
12 Clinical Presentation Asymptomatic Chest Pain Dyspnea Fulminant
13 Diagnostic Evaluation EKG Normal Non-specific T-wave inversion Conduction abnormalities ST elevation mimicking acute MI Echo Useful to rule-out other causes of cardiomyopathy Evaluate LV function and chamber sizes
14 Diagnostic Evaluation Use of Cardiac Biomarkers CK/CKMB have low predictive value Troponin T sensitivity 53%, specificity 94%, positive predictive value 93% Smith Circulation 1997 Troponin I (Multicenter Myocarditis Treatment trial) positive predictive value 82% Lauer JACC 1997
15 Diagnostic Evaluation Contrast enhanced cardiac MRI Focal myocardial enhancement (edema) and wall motion abnormalities strongly suggest myocarditis Mid-wall striated late gadolinium enhancement is associated with active myocarditis by the Dallas histopathologic criteria
16 Role of Endomyocardial Biopsy ACC/AHA Guidelines for Heart Failure Management Class IIa recommendation for EMB- Endomyocardial biopsy can be useful in patients presenting with HF when a specific diagnosis is suspected that would influence therapy Class III for routine use in evaluation Hunt et al JACC 2009
17 Role of Endomyocardial Biopsy Dallas Criteria-standardized histopathologic definition of myocarditis Only 10% of patients with idiopathic heart failure will have a positive biopsy by the Dallas Criteria
18 Role of Endomyocardial Biopsy Indications for Endomyocardial Biopsy: Subacute or acute onset HF refractory to management Development of significant arrhythmias (heart block and VT) HF with rash, fever, peripheral eosinophilia History of lupus, scleroderma, polyarteritis nodosum New-onset cardiomyopathy with known amyloidosis, sarcoidosis, hemachromatosis Suspicion for Giant Cell Myocarditis (young age, rapid progression, progressive arrhythmias) Wu Mayo Clin Proc 2001
19 Role of Endomyocardial Biopsy Histopathology can predict transplant-free survival Lymphocytic, granulomatous and giant cell myocarditis on EMB predicted death or transplant Clinical variables (except PCWP >15) did not predict the primary endpoint Magnani Am Heart J 2006 Kindermann Circulation 2008
20 Treatment Modalities Supportive Care Supportive Care Supportive Care
21 Hemodynamic Support Modalities
22 Treatment Modalities After initial hemodynamic stabilization ACC/AHA guidelines for pharmacologic intervention in heart failure ϐ-adrenergic blockade ACE inhibitors/arb Aldosterone antagonists (spironolactone, eplerenone) Decision to implant an ICD should be deferred for several months if possible
23 Treatment Modalities Immunosuppression Myocarditis Treatment Trial* 111 patients Randomized to placebo or immunosuppression No difference in mortality or LVEF at 28 weeks *Immunosuppression may be of benefit in patients with myocarditis in the setting of systemic autoimmune diseases *Mason NEJM 1995
24 Acute Pericarditis Myocarditis Pericarditis Myopericarditis
25 A Simple Problem with a Simple Solution? The Problem The Solution Inflammation Reduce Inflammation Fluid Drain the fluid Constriction Remove the pericardium Adapted from Nishimura RA, Pericardial Disease 2011
26 A Simple Problem That is often undertreated and mismanaged
27 Pericardial Disease A 39 year old man presents with three days of severe chest pain that is pleuritic in nature. Improved by sitting up. He had an upper respiratory infection for the last week. Exam: HR 92 BP 120/77 JVP < 8 cm H20, brisk carotid upstrokes Normal heart sounds, no murmur Three-part friction rub heard at LLSB
28 Pericardial Disease
29 PR depression Diffuse ST-elevation
30 Other tests are optional unless Heart failure signs/symptoms Echo Pulses paradoxus Echo High-risk for CAD Cardiac biomarkers Recurrent pericarditis Autoimmune profile
31 Treatment DO USE NSAIDs or high-dose aspirin for one month, then taper slowly (can follow ESR) DO GIVE cochicine for the first 6 months to reduce risk of relapse DO NOT use steroids due to increased risk of relapsing pericarditis
32 Relapsing Pericaritis Usually due to use of steroids with rapid taper Management: High-dose aspirin with goal salicylate level VERY SLOW steroid taper, 1 mg per month Colchicine In refractory cases, consider pericardiectomy
33 Take-Home Points Myocarditis and Pericarditis are overlapping conditions sharing a number of characteristics Treatment of myocarditis remains supportive care with good heart failure management Use of steroids in pericarditis increases the risk of relapsing disease
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