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Rheumatic Myocarditis

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Description: Rheumatic myocarditis is an inflammatory condition of the heart muscle that occurs as a complication of rheumatic fever, often resulting from a streptococcal infection.
Type: Rheumatic myocarditis is an inflammatory condition of the heart muscle that occurs as a complication of rheumatic fever, which itself is a result of an autoimmune response to a group A Streptococcus infection. Rheumatic myocarditis is not inherited and does not involve genetic transmission. Instead, it is an acquired condition following a streptococcal infection.
Signs And Symptoms: The signs and symptoms associated with myocarditis are varied, and relate either to the actual inflammation of the myocardium or to the weakness and dysfunction of the heart muscle that is secondary to the inflammation. While myocarditis may develop over periods ranging from hours to months, patients typically present with signs and symptoms that resemble heart failure, including the following:
Since myocarditis is often due to a viral illness, many patients experience symptoms consistent with a recent viral infection including a fever, rash, loss of appetite, abdominal pain, vomiting, diarrhea, joint pains, and easily becoming tired. Additionally, myocarditis is often associated with pericarditis, and many people with myocarditis present with signs and symptoms that suggest myocarditis and pericarditis at the same time.Children primarily present with the aforementioned symptoms associated with a viral infection. Later stages of the illness can involve the respiratory system and lead to increased work of breathing. These are often mistaken for asthma.Myocarditis can be distinguished as either fulminant or acute based on the severity of symptoms on presentation, as well as the time course over which symptoms develop and persist. This categorization can help predict the treatment, outcomes, and complications of myocarditis.
Fulminant myocarditis is defined as sudden and severe myocarditis that is associated with signs and symptoms of heart failure while at rest. More specifically, fulminant myocarditis is characterized by a distinct, rapid onset of severe heart failure symptoms, such as shortness of breath and chest pain, that develop over the course of hours to days. Additionally, treatment requires the use of medications or mechanical devices to improve heart function.Acute non-fulminant myocarditis has a less distinct onset in contrast to fulminant myocarditis, and evolves over days to months. While the symptoms of acute myocarditis overlap with those of fulminant myocarditis, they do not typically occur at rest, and treatment does not require the use of mechanical circulatory support.
Prognosis: The prognosis associated with myocarditis is stratified by the severity and time course along which symptoms develop. In addition to symptom severity, there are also several indicators of heart function that can be used to predict patient outcomes, many of which are part of the standard evaluation of patients presenting with cardiovascular dysfunction. Most people with myocarditis have an uncomplicated, self-limited and mild course while making a full recovery. However, those with myocarditis that present with a decreased ejection fraction, or those who present with heart failure, advanced atrioventricular block, with sustained ventricular arrhythmias or with hemodynamic instability have a worse prognosis with an increased risk of death or need for heart transplantation.An electrocardiogram is one of the most common screening tools used in cases of suspected cardiac pathology, such as myocarditis. The findings that correlate with poorer outcomes are non-specific and include widened QRS complexes and QT intervals, partial or complete atrial-ventricular heart block, and malignant ventricular arrhythmias like sustained ventricular tachycardia or ventricular fibrillation. Electrocardiogram findings of ST elevations with upward concavity and an early repolarization pattern, however, were associated with a better cardiovascular prognosis in general.In cases of acute myocarditis, cardiac magnetic resonance imaging can reveal several prognostic indicators that, similar to ECGs, are non-specific and reflect poorer cardiac physiology. Late gadolinium enhancement on cardiac MRI demonstrates perturbations in extracellular volume as a result of cell necrosis or edema, and is significantly associated with increases in all-cause mortality, cardiovascular mortality, and major adverse cardiovascular events. The association was strongest with any late gadolinium enhancement, but remained true for findings of anterolateral-specific enhancement. A similar relationship was found between a left ventricular ejection fraction < 50%, increased mortality, and increased major adverse cardiovascular events.Myocarditis has been reported to be a major cause of sudden cardiac death (SCD) in infants, adolescents, and young adults, but the reported rates show wide variation (1 to 14 percent) among young people depending on differences in SCD definition and classification/ definition of myocarditis post-mortem as well as heterogeneity of study populations.In fulminant myocarditis, in which an inflammatory cytokine storm occurs, cardiac functions decline rapidly and the death rate is high.
Onset: Rheumatic myocarditis often develops as a result of rheumatic fever, which is a complication of untreated or poorly treated streptococcal throat infection. The onset of symptoms can vary but typically appears within 1 to 5 weeks after the initial streptococcal infection. The onset can be acute with symptoms such as fever, chest pain, palpitations, and fatigue.
Prevalence: The prevalence of rheumatic myocarditis is not specified as "nan" (not a number). Rheumatic myocarditis is a complication of rheumatic fever, a condition resulting from untreated or inadequately treated streptococcal throat infections. The exact prevalence can vary widely by geography, age group, and access to medical care, but it tends to be more common in developing countries where rheumatic fever is still prevalent.
Epidemiology: The prevalence of myocarditis is estimated to be about 1-10 cases per 100,000 persons per year, with higher estimates at 22 cases per 100,000 persons annually. The highest incidence of myocarditis is seen in men between the ages of 20 and 40. Fulminant myocarditis, the most severe subtype, has been shown to occur in up to 2.5% of known myocarditis presentations. When looking at different causes of myocarditis, viral infection is the most prevalent, especially in children; however, the prevalence rate of myocarditis is often underestimated as the condition is easily overlooked and is sometimes asymptomatic. Viral myocarditis being an outcome of viral infection depends heavily on genetic host factors and the pathogenicity unique to the virus. If one tests positive for an acute viral infection, clinical developments have discovered that 1-5% of said population may show some form of myocarditis.In regard to the population affected, myocarditis is more common in pregnant women, children, and those who are immunocompromised. Myocarditis, however, has shown to be more common in the male population than in the female. Multiple studies report a 1:1.3-1.7 female-male ratio of prevalence of myocarditis. In young adults, up to 20% of all cases of sudden death are due to myocarditis. Young males specifically have a higher incidence rate than any other population due to their testosterone levels creating a greater inflammatory response that increases the chance of cardiac pathologies. While males tend to have a higher risk of developing myocarditis, females tend to display more severe signs and symptoms, such as ventricular tachycardia and ventricular fibrillation, but do so at an older age. Among patients with HIV, myocarditis is the most common cardiac pathological finding at autopsy, with a prevalence of 50% or more.Myocarditis is the third most common cause of death among young adults with a cumulative incidence rate globally of 1.5 cases per 100,000 persons annually. Myocarditis accounts for approximately 20% of sudden cardiac death in a variety of populations, including adults under the age of 40, young athletes, United States Air Force recruits, and elite Swedish orienteers. With individuals who develop myocarditis, the first year is difficult as a collection of cases have shown there is a 20% mortality rate.
Intractability: Rheumatic myocarditis, which is inflammation of the heart muscle due to rheumatic fever, is not inherently intractable. It typically responds to appropriate treatment, which includes antibiotics to treat the underlying streptococcal infection and anti-inflammatory medications to reduce inflammation. Chronic or severe cases may require more extensive medical management and follow-up. Timely and proper treatment can significantly improve outcomes and prevent long-term complications.
Disease Severity: Rheumatic myocarditis is an inflammatory condition of the heart muscle caused by rheumatic fever, which is a complication of untreated or inadequately treated strep throat or scarlet fever. The severity of rheumatic myocarditis can vary, but it often leads to significant complications including arrhythmias, heart failure, and chronic rheumatic heart disease. Prompt treatment of streptococcal infections and ongoing prophylactic therapy are essential in managing disease severity and preventing long-term damage.
Healthcare Professionals: Disease Ontology ID - DOID:8481
Pathophysiology: Rheumatic myocarditis is an inflammatory condition of the heart muscle (myocardium) that occurs as a result of rheumatic fever. The pathophysiology involves an autoimmune response triggered by a Group A Streptococcus infection, usually following a case of strep throat or scarlet fever. The body's immune system mistakenly attacks its own tissues, including the heart, joints, skin, and brain. In the heart, this leads to inflammation and damage to the myocardium, as well as potential involvement of the heart valves and pericardium. This immune-mediated damage results in symptoms such as chest pain, fatigue, heart murmurs, and potentially heart failure if left untreated.
Carrier Status: Rheumatic myocarditis is an inflammatory condition of the heart muscle that occurs as a complication of rheumatic fever, which itself is a result of an autoimmune response to a streptococcal infection. There is no carrier status associated with rheumatic myocarditis, as it is not an infectious disease or a genetic condition.
Mechanism: Most forms of myocarditis involve the infiltration of heart tissues by one or two types of pro-inflammatory blood cells, lymphocytes and macrophages plus two respective descendants of these cells, NK cells and macrophages. Eosinophilic myocarditis is a subtype of myocarditis in which cardiac tissue is infiltrated by another type of pro-inflammatory blood cell, the eosinophil. Eosinophilic myocarditis is further distinguished from non-eosinophilic myocarditis by having a different set of causes and recommended treatments.The pathophysiology of viral myocarditis is not well understood, but it is believed to involve cardiotropic viruses (viruses with a high affinity for the heart muscle) gaining entry to cardiac muscle cells, usually via binding to a transmembrane receptor. Over approximately the next 1–7 days the virus replicates and causes inflammation leadings to necrosis and apoptosis of cardiac muscle cells (myocytes) and activation of the innate immune system. Over the next 1–4 weeks, viral replication continues with subsequent activation of the acquired immune system leading to T cell infiltration and the formation of antibodies, including possibly auto-antibodies. Over the next few months to years, this process either resolves and concludes with viral clearance or it may progress to cause permanent heart damage such as dilated cardiomyopathy, ventricular dysfunction or other cardiomyopathies. Coxsackie B, specifically B3 and B5, has been found to interact with coxsackievirus-adenovirus receptor (CAR) and decay-accelerating factor (DAF). However, other proteins have also been identified that allow Coxsackieviruses to bind to cardiac cells. The natural function of CAR and mechanism that the Coxsackievirus uses to infect the cardiac muscle is still unknown. The mechanism by which coxsackie B viruses (CBVs) trigger inflammation is believed to be through the recognition of CBV virions by Toll-like receptors.The binding of many types of coronaviruses, including the SARS-CoV-2 virus, through ACE2 receptors present in heart muscle may be responsible for direct viral injury leading to myocarditis. In a study done during the 2002-2004 SARS outbreak, SARS viral RNA was detected in the autopsy of heart specimens in 35% of the patients in the Toronto, Canada area who had died due to SARS. It was also observed that an already diseased heart has increased expression of ACE2 receptor contrasted to healthy individuals which may lead to greater viral infiltration in the heart muscle. Hyperactive immune responses in COVID-19 patients may lead to the initiation of the cytokine storm. This excess release of cytokines may lead to myocardial injury. In addition to direct cardiac myocyte (heart muscle cell) damage due to SARS-CoV-2 viral infiltration and inflammation, there are other suspected mechanisms that Covid-19 may indirectly cause myocarditis. During COVID-19, the other indirect mechanisms thought to contribute to myocarditis include: oxygen supply-demand mismatch to the heart muscle leading to myocardial (heart muscle) injury; microvascular thrombi, or blood clots in the small blood vessels of the heart causing injury; the systemic hyperinflammatory state in Covid-19 leading to heart muscle injury; or the virus causing indirect damage to the heart by inducing auto-immune mediated damage to the heart muscle (and frequently other organs).
Treatment: While myocarditis has many etiologies and a variable constellation of signs and symptoms, many causes do not have a specific treatment thus the primary focus is on supportive care and symptom management. In some cases of biopsy-proven myocarditis, the causative cell type may indicate condition specific treatments that are beneficial. These treatments typically consist of corticosteroids, or immunosuppressants. Eosinophilic myocarditis, giant cell myocarditis and cardiac sarcoidosis are usually responsive to immunosuppressive treatments; in the form of glucocorticoids with or without azathioprine and cyclosporine. Some of these immune mediated forms of myocarditis require an extended course (maintenance course) of immunosuppressive therapy. It is recommended to rule out drugs and parasites as potential causes of eosinophilic myocarditis as these common causes of the variant can be effectively treated with discontinuation of the offending drug or specific anti-parasitic treatment respectively. Empiric IV glucocorticoids are indicated in acute myocarditis with cardiogenic shock, heart failure, ventricular arrhythmias or high degree AV block that is suspected due to auto-immune disease; but the European Society of Cardiology also recommends subsequent viral genome testing of endomyocardial biopsy specimens due to risk of viral activation, which may necessitate discontinuation of immunosuppression therapy.In a majority of cases, the main therapies are used to support patients and are dependent on the severity of symptoms and the time course across which myocarditis develops. Supportive therapies can be divided into two broad categories, medications and mechanical support.
Compassionate Use Treatment: Compassionate use treatment for rheumatic myocarditis, an inflammation of the heart muscle related to rheumatic fever, may include off-label or experimental therapies under strict regulatory oversight. Some investigational approaches or treatments used under compassionate use may involve:

1. **Immunosuppressive Therapy:** Drugs like corticosteroids (e.g., prednisone) or other immunosuppressive agents may be used to reduce inflammation in severe cases.

2. **Intravenous Immunoglobulin (IVIG):** Used in some cases to manage autoimmune and inflammatory conditions, though not specifically approved for rheumatic myocarditis.

3. **Biologics:** Monoclonal antibodies targeting specific immune pathways, such as TNF inhibitors or IL-6 inhibitors, might be considered in experimental settings to control inflammation.

Access to these treatments typically requires regulatory approval and is usually considered when other standard treatments have failed or are unavailable.
Lifestyle Recommendations: For rheumatic myocarditis, lifestyle recommendations typically include the following:

1. **Rest and Recovery**: Adequate rest is crucial to reduce cardiac workload and allow the heart to heal.
2. **Healthy Diet**: A balanced diet rich in fruits, vegetables, whole grains, and lean proteins. Limiting salt intake can help manage symptoms and reduce stress on the heart.
3. **Regular Medical Check-Ups**: Regular follow-up appointments with a healthcare provider to monitor heart function and progression of the disease.
4. **Medication Adherence**: Taking medications as prescribed by a healthcare provider to manage symptoms and prevent complications.
5. **Avoiding Alcohol and Tobacco**: Refraining from smoking and limiting alcohol intake, as both can exacerbate heart conditions.
6. **Physical Activity**: Gradual increase in physical activity as advised by a healthcare provider, starting with low-impact exercises and avoiding strenuous activities.
7. **Stress Management**: Practicing stress-reduction techniques such as meditation, yoga, or other relaxation methods.
8. **Vaccinations and Infections**: Staying up to date with vaccinations and avoiding infections, as infections can exacerbate rheumatic diseases.

Always consult with a healthcare provider for personalized recommendations.
Medication: The specific medications that are used to support patients are directly related to the cause of the symptom or sign. Just as the symptoms of myocarditis mirror those of congestive heart failure, so too do the therapies. Additionally, the order in which therapies are used depends on the degree of heart dysfunction, with stabilization of patient blood pressure and breathing taking highest priority when present. This can involve the use of inotropes, or medications that make the heart contract with greater force, as well as antiarrhythmic drugs such as adenosine or carvedilol. In patients that have stable and adequate heart function, further treatments are based on heart failure guidelines. ACE inhibitors or Angiotensin Receptor Blockers (ARBs) can have a protective benefit to the heart, so either are typically used in any patient with symptomatic myocarditis. Simultaneously, beta blockers are used in patients that can tolerate their heart beating at a slower rate. Shortness of breath at rest and swelling can be relieved with diuretics such as furosemide, and the addition of aldosterone receptor blockers can augment the diuresis while preventing the excess loss of potassium. In patients with symptoms while resting, additional medications can be added such as digoxin.
Repurposable Drugs: Rheumatic myocarditis, a form of myocarditis associated with rheumatic fever, can potentially benefit from drugs repurposed from other inflammatory and cardioprotective treatments. Some potential repurposable drugs include:

1. **Colchicine**: An anti-inflammatory drug used for gout and pericarditis that has shown benefits in reducing inflammatory responses.
2. **Methotrexate**: An immunosuppressant and chemotherapy agent used for rheumatoid arthritis and psoriasis that might help manage severe inflammatory components.
3. **Beta-blockers (e.g., Metoprolol)**: Often used in heart failure and hypertension management to reduce heart strain.
4. **ACE Inhibitors (e.g., Lisinopril)**: Used for heart failure and hypertension to help reduce cardiac workload and fibrosis.
5. **Statins (e.g., Atorvastatin)**: Known for their lipid-lowering effects and anti-inflammatory properties. They are often used to prevent cardiovascular events.

These drugs would be used adjunctively, based on the specific condition and needs of the patient, and always under the supervision of a healthcare professional.
Metabolites: Rheumatic myocarditis is an inflammation of the heart muscle caused by rheumatic fever, which is a sequel of group A Streptococcus infection. Here are some key metabolites associated with rheumatic myocarditis:

1. **C-Reactive Protein (CRP)**: Elevated levels indicate inflammation and are often used to monitor the activity of rheumatic fever and myocarditis.

2. **Cardiac Troponins (Troponin T and I)**: These are biomarkers for myocardial injury. Elevated levels are significant in detecting myocarditis.

3. **B-type Natriuretic Peptide (BNP)**: Increased levels can indicate cardiac dysfunction and are elevated in heart failure cases associated with myocarditis.

4. **Cytokines (e.g., IL-6, TNF-alpha)**: These inflammatory markers are involved in the pathogenesis of myocarditis and are often elevated during an acute inflammatory response.

5. **Lactate Dehydrogenase (LDH)**: LDH levels may be elevated due to myocardial tissue damage.

6. **Serum autoantibodies**: In some cases, autoantibodies such as anti-myosin and anti-cardiac troponin antibodies are detected, reflecting an autoimmune response.

No specific metabolites directly prevent or cure rheumatic myocarditis, but these markers are crucial for diagnosing and monitoring the disease. Treatment typically focuses on eradicating the streptococcal infection and managing inflammation and cardiac complications.
Nutraceuticals: There is no verified information supporting the specific use of nutraceuticals for treating rheumatic myocarditis. It is essential to follow conventional medical treatments and consult healthcare professionals for managing this condition.
Peptides: For rheumatic myocarditis, peptides such as antimicrobial peptides and certain immunomodulatory peptides may play roles in the body's response to infection and inflammation. Research into the use of peptides for therapeutic purposes in autoimmune and inflammatory diseases is ongoing. Regarding nanoparticles (nan), they are being studied for targeted drug delivery systems and diagnostic imaging, potentially offering new approaches for managing and treating rheumatic myocarditis by delivering drugs directly to the inflamed heart tissue and improving imaging for better diagnosis.