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Young Soldier With A Failing Heart Manju Goyal, M.D. Walter Reed Army Medical Center April 2008 Case HPI: 20 year-old male with cough, shortness of breath, intermittent chest pressure and palpitations x 4 days PMhx/PSHx/Shx/Fhx/Meds: negative EXAM: Vitals: 145, 90/58, 95% ra, afebrile Cardiovascular: tachycardic, systolic murmur best heard at the apex, no JVD Lungs: CTAB Extremities: no edema Case LABS: CBC - nml BMP - nml D-dimer - nml BNP - 397 LFTs - 88/136 Cardiac enzymes - 115/2.2/<0.01 Case EKG – sinus tachycardia at 131, inferolateral TWI CXR – AP film with just an enlarged cardiac silhouette Young patient in SHOCK with concerning cardiac exam and EKG Case ECHO: - Severely dilated left ventricle but normal wall thickness No LV thrombus EF in the 10-15% range Severe global hypokinesis, with mild posterior wall contractility. Moderate to severe MR due to annular dilatation New onset of Dilated Cardiomyopathy (DCM) Dilated Cardiomyopathy www.uptodate.com Review of 1230 Patients with DCM Idiopathic — 50 percent Myocarditis — 9 percent Ischemic heart disease — 7 percent Infiltrative disease — 5 percent Peripartum cardiomyopathy — 4 percent Hypertension — 4 percent HIV infection — 4 percent Connective tissue disease — 3 percent Substance abuse — 3 percent Doxorubicin — 1 percent Other — 10 percent NEJM 2000 Importance of Etiology NEJM 2000 Additional Tests LABS: Cardiac CATH: ESR - 33 Ferritin - nml TSH - nml ACE level - nml RF - nml ANA - negative Lyme titers - negative HIV - negative Normal Coronaries What’s the differential? Any further tests? Review of 1230 Patients with DCM Idiopathic — 50 percent Myocarditis — 9 percent Ischemic heart disease — 7 percent Infiltrative disease — 5 percent Peripartum cardiomyopathy — 4 percent Hypertension — 4 percent HIV infection — 4 percent Connective tissue disease — 3 percent Substance abuse — 3 percent Doxorubicin — 1 percent Other — 10 percent Endomyocardial Biopsy NEJM 2000 Biopsy Results Dr. Brendan Graham Dept. of Pathology Normal Myocardium Biopsy – 4x Biopsy – 20x Biopsy – 40x Case of Viral Myocarditis Other infectious etiologies ruled out by special stains/cultures Dallas Criteria: Lymphocytic infiltrates of varying severity Myocyte necrosis and cytoskeletal disorganization Interstitial fibrosis seen with subacute/chronic cases Objectives: Myocarditis Review etiology and pathophysiology Clinical Manifestations Role of different diagnostic modalities Therapy 1. 2. Cardiovascular support for an unstable patient (i.e. indications for VAD, ECMO) Role of immunosuppressive/modulating therapies Prognosis Myocarditis Definition: Non-ischemic myocardial inflammation resulting from a variety of infectious, immune and toxic insults. Epidemiology Precise incidence and prevalence unknown Lack of a non-invasive “gold standard” test for diagnosis Not every suspected myocarditis case gets a biopsy Biopsy itself has low sensitivity Present in 1-9% of routine postmortem examinations1 Accounted for 20% of sudden cardiac deaths in military recruits2 1. Circulation 1976 2. Ann Intern Med 2004 Etiology Infectious VIRUSES (adeno, coxsackie) Bacterial Fungal Protozoal (Chagas disease) Helminths Non-infectious Toxins/Drugs (alcohol, anthracyclines) Systemic disorders (sarcoid, lupus, scleroderma) Etiology Etiology Braunwald 2007 Pathophysiology of Viral Myocarditis Braunwald 2007 Viral Phase Virus enters (GI/Lungs) Activates proteases damages cytoskeletan Activates tyrosine kinases immune system turns ON Replicates and persists chronic inflammation/fibrosis/DCM Braunwald 2007 Immune Response Autoimmune response: auto-antibodies to myosin and other cardiac proteins Overexpression of cytokines (IL-2, INF-γ, TNF-α) Braunwald 2007 Pathophysiology Clinical Presentation Acute Fulminant Nonspecific cardiac symptoms Heart failure, Acute MI, or SCD More common in children/teenagers +/- viral prodrome Cardiogenic shock +/- acute heart failure Chronic Subtle, insidious onset Biopsy doesn’t match Already have DCM the clinical severity. HF symptoms High levels of cytokines reversible cardiac depression better prognosis Biopsy with fibrosis usually Diagnosis Symptoms: non-specific Laboratory Testing: also non-specific Positive cardiac biomarkers ECG: T wave inversion, ST segment elevation, bundle branch blocks ECHO Differentiate fulminant from acute myocarditis Detect thrombi, valvular abnormalities, and pericardial involvement Rule out other cardiomyopathies (HOCM, Takotsubo) Diagnosis: Cardiac MRI Non-invasive Visualize entire myocardium Use to guide biopsy Follow disease course and response to therapy RV LV RV LV WITHOUT Contrast WITH Contrast Eur Heart J 1994 Diagnosis: Coronary Angiography Rule out other congenital, rheumatic, or ischemic heart disease Determine need for inotropic or mechanical support based on hemodynamic parameters Elevated pulmonary artery pressures are independent predictors of mortality Diagnosis: Endomyocardial Biopsy Although controversial, still the current gold-standard test for diagnosis 1-6% complication rate Consider when suspicious for: Giant cell myocarditis Hypersensitivity/eosinophilic myocarditis Cardiac involvement in a systemic disease All other patients, consider only if pt is deteriorating When to consider biopsy? Mayo Clin Proc 2001 Circulation 2007 Treatment Dr. Barnett Gibbs Dept. of Cardiology Treatment Treatment Treatment ABC’s Circulation: Intra-aortic balloon pump counterpulsation Ventricular assist device Cardiopulmonary assist device Intra-aortic balloon pump Electrocardiographic synchronized phased pulsation Inflation with aortic valve closure Deflation just before systole Reduce systolic arterial pressure (afterload) Augment diastolic arterial pressure Reduces myocardial oxygen consumption Enhances coronary blood flow Mean pressure unchanged Intra-aortic balloon pump Benefits: Diminish myocardial ischemia 10-20% increase in CO Diminish heart rate Increase urine output Risks: Damage/perforation of aorta Distal ischemia Thrombocytopenia Hemolysis Renal emboli Mechanical failure – balloon rupture Ventricular-assist device Centrifugal pump or Archimedes’ screw type Inflow from LV and outflow into aorta Has been used as a bridge in myocarditis until recovery or transplant * *Centrifugal pump vs. corkscrew Ventricular-assist device Centrifugal pump or Archimedes’ screw type Inflow from LV and outflow into aorta Has been used as a bridge in myocarditis until recovery or transplant Disadvantages: Surgical implantation infection thrombosis hemolysis Ventricular-assist device Infection: Review of 76 patients using LVAD to bridge to cardiac transplant LVAD-related infection: 38 patients (50%) 29 bloodstream infections (including 5 cases of endocarditis) 17 local infections CID. 2005;40:1108. Treatment Treatment ABC’s Circulation: Intra-aortic balloon pump counterpulsation Ventricular assist device Cardiopulmonary assist device Medical therapy ACE-inhibitors Beta-blockers Medical therapy Most therapy used in HF patients appears to benefit those with HF due to myocarditis – with the exception of digoxin ACE-inhibitors Beta-blockers No RCT reviewing spironolactone or ARBs but these as well as other HF meds have been used successfully in case reports Medical therapy Animal models appear to demonstrate improved function with use of ACE inhibitors 32 mice infected with Coxsakie B3 virus Randomized to captopril vs. placebo on day 3 This evidence has been extrapolated to humans Am Heart J. 1990;120:1377. Medical therapy Animal models appear to demonstrate improved function with use of beta-blockers Circulation. 1991;83:2021.. Treatment Treatment ABC’s Circulation: Intra-aortic balloon pump counterpulsation Ventricular assist device Cardiopulmonary assist device Medical therapy ACE-inhibitors Beta-blockers Immunosuppressive therapy Immunosuppressive Therapies Recent meta-analysis of placebo-controlled RCT of immune therapy for myocarditis Five trials; 316 total patients Single or combination immunosuppressive therapy Prednisone Azathioprine Cyclosporine IVIG Int Heart J. 2005;46:113. Immunosuppressive Therapies Int Heart J. 2005;46:113. Immunosuppressive Therapies End-points: All cause death Heart transplantation Secondary: Change in LVEF and LVEDD Summary: No statistically significant benefit in treatment of myocarditis with immunosuppressive therapy Int Heart J. 2005;46:113. NEJM. 2000;343:1388. Prognosis Review of 1230 patients with cardiomyopathy Idiopathic cardiomyopathy (n=616 patients) Peripartum cardiomyopathy (51) Myocarditis (111) Ischemic heart disease (91) Infiltrative myocardial disease (59) Hypertension (49) Human immunodeficiency virus (45) Connective-tissue disease (39) Substance abuse (37) Therapy with doxorubicin (15) Other causes (117) NEJM. 2000;342:1077. Prognosis Idiopathic CM acted as the reference category No difference in survival between idiopathic CM and cardiomyopathy due to myocarditis NEJM. 2000;342:1077. Prognosis NEJM. 2000;342:1077. Prognosis “Loose” rule of third’s… 1/3: recover 1/3: residual ventricular dysfunction 1/3: transplantation or death SUMMARY ABC’s Supportive therapy is mainstay therapy Most medical therapies for HF seem to benefit myocarditis patients with the exception of digoxin Immunosuppressive therapy does not seem to play a role in survival Back to the case Stabilized initially with LVAD and ECMO EF increased to 40-45% Started on coreg, lisinopril, and aldactone Multiple complications during the hospital course Cardiac tamponade s/p thoracotomy Hemorrhagic CVA s/p craniotomy, tracheostomy and a PEG Multiple Infections Currently, at a rehab facility due to residual neurologic deficit and deconditioning Conclusion Most common cause is viruses (adeno and coxsackie) Highly variable clinical manifestations Cardiac MRI looks promising for diagnosis Biopsy is the gold standard but should be pursued in only select patients Aggressive, supportive care is the first line therapy because of high incidence of recovery Immunosuppressive therapy does not affect mortality References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. Felker GM et al. Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med 2000 Apr; 342(15): 1077-84. Cooper LT et al. The Role of Endomyocardial Biopsy in the Management of Cardiovascular Disease. Circulation 2007 Nov; 116: 2216-2233. www.uptodate.com Baughman KL: Diagnosis of myocarditis: Death of Dallas criteria. Circulation 2006; 113:593. Wu LA et al. Current role of endomyocardial biopsy in the management of patients with dilated cardiomyopathy and myocarditis. Mayo Clin Proc 2001; 76:1030 Cooper LT et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Circulation 2007; 116: 2216 Libby: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. Goldberg LR et al. Predictors of adverse outcome in biopsy-proven myocarditis. JACC 1999; 33 Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann Intern Med. 2004;141:829–834. Blankenhorn MA, Gall EA. Myocarditis and myocardosis; a clinicopathologic appraisal. Circulation. 1956;13:217–223. Kuhl U, Pauschinger M, Seeberg B, et al. Viral persistence in the myocardium is associated with progressive cardiac dysfunction. Circulation. 2005;112:1965–1970. Fuse K, Kodama M, Okura Y, et al. Predictors of disease course in patients with acute myocarditis. Circulation. 2000;102:2829 –2835. Ellis CR, et al. Myocarditis basic and clinical aspects. Cardiology in Review 2007;15: 170–177 Biopsy - 2-5% complication rate - Venous access: inadvertent arterial puncture, pneumothorax, vasovagal reaction, or bleeding after sheath removal Procedure itself: arrhythmias, conduction abnormalities, and cardiac perforation to pericardial tamponade and rarely, death. Patchy infiltrates lower sensitivity Lateral wall most common hard to access Diagnosis Expanded Criteria Category II: Evidence of Cardiac dysfunction in the Absence of regional coronary ischemia Compatible with myocarditis = 3 positive categories Category I: Clinical symptoms Suspicious for myocarditis = 2 positive categories High probability of being myocarditis = all 4 categories positive Category III: Cardiac MRI Category IV: Myocardial biopsy - Pathological or Molecular Analysis