MYOCARDITIS

DR GOURANGA KUMAR SAHA MBBS,MD,FESC,FACC Associate Professor Cardiology NICVD, DHAKA

Definition of Myocarditis

Myocarditis is clinically and pathologically defined as inflammation of the myocardium in the absence of the predominant acute or chronic ischaemia characteristic of CAD. It is a clinical syndrome of non-ischaemic myocardial inflammation resulting from a heterogeneous group of infectious, immune and nonimmune diseases . Histopathologically, it is characterised by an inflammatory cellular infiltrate with or without evidence of myocyte injury

clinicopathologic classification :

Fulminant myocarditis - Follows a viral prodrome; distinct onset of illness comprising severe cardiovascular compromise with ventricular dysfunction and multiple foci of active myocarditis; either resolves spontaneously or results in death. Acute myocarditis - Less distinct onset of illness, with established ventricular dysfunction; may progress to dilated cardiomyopathy Chronic active myocarditis - Less distinct onset of illness, with clinical and histologic relapses; development of ventricular dysfunction associated with chronic inflammatory changes (including giant cells) . Chronic persistent myocarditis - Less distinct onset of illness; persistent histologic infiltrate with foci of myocyte necrosis without ventricular dysfunction despite symptoms (eg, chest pain, palpitations)

Aetiology of Myocarditis
Infectious causes: Viral: *Influenza A *Adenovirus *Coxsackie B virus *Hepatitis C virus *HIV *Echovirus *EBV *CMV *Parvovirus

Aetiology of Myocarditis(continued)
Bacterial: Mycobacterial Streptococcal species Mycoplasma pneumoniae Treponema pallidum TB Staphylococcal species Clostridium species Neisseria gonorrhea

Aetiology of Myocarditis(continued)
Mycotic: Aspergillus species Candida species Coccidiomycosis Cryptococcus species Histoplasma species Sporotrichosis species Blastomycosis

Aetiology of Myocarditis(continued)
Protozoal: Trypanosoma cruzi (Chagas disease) African trypanosomiasis (sleeping sickness) Toxoplasmosis Malaria Parasitic Schistosomiasis Larva migrans

Aetiology of Myocarditis(continued)

Helminthic :
*Trichinosis *Echinococcosis *schistosomiasis *heterophyiasis *cysticercosis *visceral larva migrans *filariasis

Aetiology of Myocarditis(continued)
Drugs (usually causing hypersensitivity myocarditis) Chemotherapeutic drugs - Doxorubicin and anthracyclines, streptomycin, cyclophosphamide, interleukin-2, anti-HER-2 receptor antibody/Herceptin Antibiotics - Penicillin, chloramphenicol, sulfonamides Antihypertensive drugs - Methyldopa, spironolactone Antiseizure drugs - Phenytoin, carbamazepine Amphetamines, cocaine, catecholamines

Aetiology of Myocarditis(continued)

Chemicals - Hydrocarbons, carbon monoxide, arsenic, lead, phosphorus, mercury, cobalt Physical agents (radiation, heatstroke, hypothermia) Acute rheumatic fever Systemic inflammatory disease - Giant cell myocarditis, sarcoidosis, Kawasaki disease, Crohn disease, systemic lupus erythematosus, ulcerative colitis, Wegener granulomatosis, thyrotoxicosis, scleroderma, rheumatoid arthritis Peripartum cardiomyopathy Posttransplant cellular rejection

Pathophysiology

Damage occurs through the following mechanisms:

Direct cytotoxic effect of the causative agent Secondary immune response, which can be triggered by the causative agent Cytokine expression in the myocardium (eg, tumor necrosis factor-alpha, nitric oxide synthase) Aberrant induction of apoptosis 3

Pathophysiology

Acute phase (first 2 wk): Myocyte destruction is a direct consequence of the offending agent, which causes cell-mediated cytotoxicity and cytokine release, contributing to myocardial damage and dysfunction. Detection of the causal agent is uncommon during this stage. Chronic phase (>2 wk): Continuing myocyte destruction is autoimmune in nature, with associated abnormal expression of human leukocyte antigen (HLA) in myocytes (and in the case of viral myocarditis, persistence of viral genome in myocardium).

History

Patients may present with mild symptoms of chest pain (in concurrent pericarditis), fever, sweats, chills, and dyspnea . Population studies suggest that adults may present with few symptoms, rather than the acute toxic state of cardiogenic shock or frank heart failure (fulminant myocarditis) that is often associated with myocarditis. Symptoms of palpitations, syncope, or even sudden cardiac death may develop, due to underlying ventricular arrhythmias or atrioventricular block (especially in giant cell myocarditis). Adults may present with heart failure years after initial index event of myocarditis (as many as 12.8% of patients with idiopathic dilated cardiomyopathy had presumed prior myocarditis in one case series).

Physical

Patients with myocarditis usually present with signs and symptoms of acute decompensation of heart failure (eg, tachycardia, gallop, mitral regurgitation, edema) and pericardial friction rub in those with concomitant pericarditis

Physical

Sarcoid myocarditis - Lymphadenopathy, also with arrhythmias, sarcoid involvement in other organs (up to 70%) Acute rheumatic fever (usually affects heart in 50-90%) Associated signs such as erythema marginatum, polyarthralgia, chorea, subcutaneous nodules (Jones criteria) Hypersensitive/eosinophilic myocarditis - Pruritic maculopapular rash and history of using offending drug Giant cell myocarditis - Sustained ventricular tachycardia in rapidly progressive heart failure15 Peripartum cardiomyopathy - Heart failure developing in the last month of pregnancy or within 5 months following delivery

Laboratory Studies

CBC - Leukocytosis (may be eosinophilia) ESR (and other acute phase reactants eg. CRP.) Elevated cardiac enzymes (CK or cardiac troponins): These are an indicator for cardiac myonecrosis help identify those with resolution of viral myocarditis. The test has 89% specificity and 34% sensitivity Serum viral antibody titers for viral myocarditis

Imaging Studies

*Echocardiography:to exclude other causes of heart failure & to evaluate the degree of cardiac dysfunction . *Cardiac angiography: *(MRI): This imaging technique is used for assessment of the extent of inflammation and cellular edema, although it is still nonspecific. *Nuclear imaging: Antimyosin scintigraphy(sensitivity (91-100%) and specificity (31-44%) * Electrocardiogram - Often nonspecific (eg, sinus tachycardia, nonspecific ST or T-wave changes)

Procedures

Endomyocardial biopsy (EMB): Standard for diagnosis of myocarditis. Routine EMB in establishing diagnosis of myocarditis rarely is helpful clinically, however, since histologic diagnosis seldom has an impact on therapeutic strategies, unless giant cell myocarditis is suspected. Histologic Findings:Biopsy specimens from EMB should reveal the simultaneous findings of lymphocyte infiltration and myocyte necrosis.

Treatment :Medical Care

controlling or treating the underlying cause of the inflammation reducing the workload of the heart treating heart abnormalities that result from the inflammation

Medications

ACE inhibitors and beta-blockers Steroids and other medications Diuretics

Treatment :Medical Care

Withdrawal of the offending agent Anticoagulation may be advisable as a preventive measure Antiarrhythmics can be used cautiously: *Supraventricular arrhythmias should be converted electrically. *High-grade ventricular ectopy and ventricular tachyarrhythmia should be treated cautiously with beta blockers and antiarrhythmics *Patients are usually very sensitive to digoxin and should use it with caution and in low doses. *Complete heart block is an indication for temporary transvenous pacing. * Implantable defibrillators rarely are indicated in lymphocytic myocarditis

Surgical Care

Left ventricular assistive devices (LVADs) and extracorporeal membrane oxygenation may be indicated for short-term circulatory support if needed for cardiogenic shock. Cardiac transplantation :beneficial to those with biopsy-proven giant cell myocarditis; the 5-year survival rate after transplantation was 71%, despite a 25% incidence of posttransplantation recurrence,

Complications

Cardiogenic shock may occur in fulminant cases of myocarditis Severe heart block requiring permanent pacemaker placement occurred in 1% of patients in the Myocarditis Treatment trial.

Prognosis

The prognosis for long-term damage is not predictable. After the initial phase of myocarditis, some patients can experience complete recovery, others may develop chronic heart failure. some patients develop fulminant heart failure,

Prognosis

Patients who have survived fulminant myocarditis have a good prognosis :In a study of 147 cases of myocarditis monitored for an average of 5.6 years, 93% of the 15 patients with fulminant disease were alive without transplant 11 years after biopsy, compared with 45% of the 132 patients with less severe disease. Predictors of death or need for heart transplantation after acute myocarditis in multivariate analyses include syncope, low ejection fraction, and left bundle-branch block, all indicators of advanced cardiomyopathy

Thank you