The terms pericarditis refers to inflammation of the pericardium and myocarditis. Both can occur together in clinical practice, and hence the term myopericarditis is used.
Sometimes myopericarditis is used interchangeably with perimyocarditis. Myopericarditis is used when there are primarily pericarditis symptoms with evidence of the involvement of the myocardium as evidenced by cardiac biomarker elevation or imaging studies revealing normal wall motion. If there is evidence of regional wall motion abnormalities with reduced ventricular function, the term perimyocarditis has been used.
The majority of cases of myopericarditis are idiopathic as no definitive cause is identified even after extensive work up. Causes are divided into infectious and non-infectious causes. Most of the infectious causes are viral, and they are often attributed to the following viruses:
- Viruses: Coxsackievirus, adenoviruses, herpes viruses, echovirus, Ebstein-Barr virus, cytomegalovirus, influenza virus, hepatitis C virus, parvovirus B19
- Bacterial: Mycobacterium tuberculosis, Streptococcus, Staphylococcus, Haemophilus, Legionella, Mycoplasma
- Fungal: Histoplasma, Aspergillus, Blastomyces, coccidioidomycosis
- Parasites: Toxoplasma, amebic, Chaga disease
Drugs (cardiotoxic effects or hypersensitivity reactions): procainamide, isoniazid, hydralazine, alcohol, anthracycline, heavy metals
- Post-radiation to the chest cavity
- Systemic inflammatory diseases: Lupus, rheumatoid arthritis, scleroderma, Sjogren, mixed connective tissue disease
- Other inflammatory conditions: Granulomatosis, inflammatory bowel disease
- Metastatic cancers: Especially lung cancer, breast cancer, melanoma
- Primary cardiac tumors: Rhabdomyosarcoma
- Metabolic: Hypothyroidism, Renal failure/uremia
- Vaccine-associated myopericarditis
The exact incidence and prevalence of myopericarditis have not been established. Acute pericarditis is the admitting diagnosis in 0.1% of hospital admissions. It is estimated the incidence of myocarditis is around 1 to 10 cases per 100,000 persons. Vaccine-associated myocarditis has been reported in 0.01% of military recruits following smallpox vaccination.
History and Physical
Clinical features depend on the degree of the inflammation of the pericardium, myocardium, and acuity of the illness. The clinical presentation can differ from a subtle, self-limiting illness to severe cardiogenic shock and death.
Early symptoms included precordial chest pain, fatigue, dyspnea, palpitations, and fever. Patients may give symptoms suggestive of a viral prodrome (a runny nose, arthralgia, low-grade fever) 1 to 2 weeks preceding the presentation. In predominant pericardial involvement, they can describe the pain as sharp, worse with a cough or inspiration and relieved by sitting forward. If there is significant myocardial involvement, there may be a continuous pain, and sometimes, it is hard to differentiate from myocardial ischemia pain, especially in people with cardiovascular risk factors. They may also have predominant heart failure symptoms such as shortness of breath, orthopnea, pedal edema, and fatigue. Rare symptoms include arrhythmias, syncope, and sudden cardiac arrest. Physical examination findings can be variable, but common findings may include fever, pericardial friction rub and features of heart failure. Look for other signs of systemic illness which may contribute to the etiology.
Myopericarditis can result in elevation of markers of inflammation like erythrocyte sedimentation rate, C-reactive protein, white blood cell and cardiac biomarkers suggesting myocardial involvement. All the patients should also have routine blood work to rule out thyroid pathology, hepatitis, and renal function. Workup with serological markers is undertaken in patients with persistent symptoms or associated with signs of such illness. Routine viral serologies are unlikely to be helpful in the diagnostics process as the results from these studies rarely alter the treatment.
Typical ECG findings of pericarditis include diffuse concave ST-segment elevation and PR depression. Depending on the degree of myocardial involvement, these classic changes may or may not be present with diffuse T-wave changes or inversions. Even though ST-segment changes are diffuse in most cases, it is not uncommon to see localized ECG changes (inferolateral or anterolateral) depending on the degree of the involvement. Common arrhythmias include supraventricular or ventricular ectopic beats or non-sustained ventricular arrhythmias.
Chest x-ray is normal in most of the self-limiting and minor forms of the disease or may reveal an enlarged cardiac silhouette suggesting significant fluid accumulation in the pericardial space in some patients. There may be signs of heart failure as well in some very sick patients.
An echocardiogram is routinely performed and in most patients may be normal with normal heart function with a trace or no significant pericardial effusion. Researchers have described increased pericardial brightness as a marker of the pericardial inflammation, but this is a non-specific finding with limited specificity.
Some patients may have significant pericardial fluid accumulation with or without hemodynamic compromise (tamponade physiology). An echocardiogram will also assess the left and right ventricular function and associated valvular heart disease abnormalities. Patients with reduced left ventricular function are preferably managed in tertiary centers, and these patients have high morbidity and mortality.
Coronary angiography is not indicated in young patients with typical features of myopericarditis, but in patients with risk factors for atherosclerotic cardiovascular disease it may be difficult to differentiate from myocardial ischemia based on non-invasive tests and would need cardiac catheterization to rule out acute coronary syndrome.
Cardiac Magnetic Resonance Imaging
Cardiac magnetic resonance imaging (CMR) can be a very good diagnostic test in these patients to assess the degree of myocardial and pericardial involvement. In patients with myopericarditis, subepicardial or mid-myocardial inflammatory changes are seen along with myocardial edema in different vascular territories, as opposed to subendocardial or transmural myocardial enhancement in one arterial territory in acute coronary syndrome. CMR will also assess the left ventricular function. Pericarditis can be diagnosed on CMR by the presence of noncalcified pericardial thickening with pericardial effusion.
Endomyocardial biopsy may be needed in a few selective sick patients, who exhibit clinical signs of continued deterioration despite standard supportive care. In these patients, endomyocardial biopsy is recommended if it may alter treatment options, for example for giant cell arteritis).
The diagnosis of myopericarditis is suspected based on the history of pleuro-pericarditis chest pain, findings on the clinical exam like pericardial friction rub associated with typical changes of EKG and elevated cardiac biomarkers. Elevated markers of inflammation will support the diagnosis of myopericarditis and echocardiogram is done to assess the left ventricular function and pericardial involvement. In patients with atherosclerotic risk factors, they will need cardiac catheterization to rule out obstructive epicardial coronary artery disease. Cardiac magnetic resonance imaging is a useful test to assess the degree of myocardial involvement especially in patients with hemodynamic instability or symptoms of heart failure or cardiac arrhythmias.
Acute pericarditis is diagnosed in the presence of 2 or more of the following features: Pleuro-pericarditis chest pain, pericardial friction rub on the exam, ECG changes (diffuse concave ST-segment elevation and PR depression), or pericardial effusion.
Myopericarditis is diagnosed in the presence of one additional feature: Elevated cardiac biomarkers, presumed new Left ventricular systolic dysfunction based on echocardiography or CMR, myocardial inflammation by CMR.
Pearls and Other Issues
In patients with risk factors for atherosclerotic vascular disease, it can be confused with the acute coronary syndrome.
Patients usually present with complaints of chest pain, shortness of breath, fever and on examination may have a pericardial friction rub.
Laboratory tests will be positive for markers of inflammation like erythrocyte sedimentation rate, C-reactive protein, and white cell count.
Cardiac biomarkers will be elevated in patients with myopericarditis. An electrocardiogram may have changes of pericarditis, but in patients with significant pericardial effusion and myocarditis, typical changes may not be seen.
An echocardiogram is routinely performed to assess the degree of pericardial effusion and left the ventricular function.
Cardiac magnetic resonance imaging may provide, additional information to quantify the extent of inflammation in the myocardium.
Patients with predominant pericarditis features are treated with high dose non-steroidal anti-inflammatory drugs, while in patients with predominant myocarditis features, these drugs have to be used with caution.
Enhancing Healthcare Team Outcomes
An interprofessional approach to myopericarditis is recommended.
Myopericarditis is not an uncommon problem and is usually of viral in etiology. The condition has diverse presentations and hence is best managed in an interprofessional team that includes an intensivist, cardiologist, radiologist, infectious disease specialist, physical therapist, and an internist. Even though most cases of myopericarditis have a good prognosis, the clinical focus should be on identifying the patients with large pericardial effusions or patients with significantly lowered systolic function as these patients need aggressive medical therapies to prevent morbidity and mortality. In the absence of data from randomized trials, treatment recommendations of myopericarditis are based on expert opinion.