About fibroelastic endocarditis
What is fibroelastic endocarditis?
Endomyocardial fibrosis (EMF) is a progressive disease of unknown origin (idiopathic) that may seriously affect the heart. Its most obvious feature is a gross change in the makeup of the lining of the heart cavities (the endocardium) of one or both of the lower chambers of the heart (the ventricles) leading to the replacement of normal cells with fibrous tissue (fibrosis). This process is progressive and leads to the narrowing (constriction) of the right or left ventricular cavities. It may involve the valves between the chambers of the heart as well as the tendon-like cords that fix the valves to the ventricles (chordae tendineae).
Loeffler's disease is a disease of the heart much like endomyocardial fibrosis. Some clinicians regard it as an early stage of EMF, although this idea remains controversial. Loeffler's disease is a rare disorder of unknown origin, characterized by abnormal increases in the number of particular white blood cells (eosinophilia), and like EMF, gross fibrosis of the endocardium, and inflammation of small blood vessels (arteritis).
What are the symptoms for fibroelastic endocarditis?
The main microscopic feature of endomyocardial fibrosis (as well as of Loeffler’s disease) is fibrosis of the inner lining of the heart cavities (the endocardium). This means that the normal endocardium is replaced by a thick, inelastic tissue. The fibrotic lesions may be over 1 cm thick and may extend finger-like projections into the heart muscle (the myocardium).
Fibrosis frequently affects the heart asymmetrically. It may specifically involve one or more of the following areas: the top (apex) of the left ventricle, the back (posterior) wall of the left ventricle including the fibrous cords that connect the valves to the ventricles (the chordae tendineae), and the top (apex) of the right ventricle, extending backwards to encase the muscle and cords (chordae tendineae) attaching the heart valve (tricuspid).
If fibrosis of the left ventricle is predominant, then blood flow from the right side of the heart is reduced often with mitral valve failure causing back-flow (regurgitation) of blood. The results may include pulmonary venous hypertension and left ventricular enlargement. Abnormal heartbeat patterns (atrial fibrillation or atrial arrhythmia) are common. Difficulty in breathing (dyspnea) especially, but not exclusively, on physical exertion is the major physical sign.
If fibrosis of the right ventricle is predominant, then circulation is restricted often with tricuspid valve failure, causing backflow (tricuspid regurgitation). Enlargement of the heart (cardiomyopathy) because of right atrial dilatation is often seen. Facial Swelling (edema), Swelling of the legs, enlargement of the spleen and liver (hepatosplenomegaly), and an accumulation of fluid in the abdominal cavity (ascites) are to be expected.
Biventricular fibrosis with circulation features is a mixture of the two forms listed above. That is, the symptoms are a combination of left and right ventricular fibrosis.
The extracardiac manifestations of Loeffler’s disease include emboli to the brain (stroke), spotty (petechial) hemorrhages, and an enlarged liver (hepatomegaly).
What are the causes for fibroelastic endocarditis?
At this time, clinicians believe that an as yet unknown immunological process is the preferred explanation for the cause of most cases of endomyocardial fibrosis and Loeffler’s disease. In the past, the cause of both conditions was attributed to the presence of the filaria worm in patients or to poor nutrition. Widespread infection with such worms and poor diets are typical in the tropical regions in which these disorders are more common. Eosinophils have been observed in some cases of endomyocardial fibrosis, suggesting a form of hypersensitivity may play a role in select cases. In children, endomyocardial fibrosis has been associated with the mumps virus.
What are the treatments for fibroelastic endocarditis?
Responses to medical treatment are generally poor and unproven. For patients with severe symptoms, surgical treatment may be pursued when other treatments have not been successful. These procedures, however, are not without risk. Mortality as a consequence of surgery may be as high as 20%. Successful surgery reduces symptoms and increases survival times and rates.
Surgery is usually designed (1) to take out the fibrous endomyocardium so that the ventricles can be filled with blood once more; (2) to repair or replace the mitral or tricuspid valve (or both), if one or another is involved; and (3) to leave a portion of fibrous endocardium in place to prevent postoperative heart block.
What are the risk factors for fibroelastic endocarditis?
Endocardial fibroelastosis is a rare disorder that affects males and females in equal numbers. Fewer than 1 percent of infants and children with congenital heart disease are diagnosed with this disorder. A 1964 study suggested an incidence of 1 in 5,000 live births. However, in the United States there has been a marked reduction in incidence since then for reasons that are not known. The disorder is extremely rare.
Is there a cure/medications for fibroelastic endocarditis?
The cause of fibroelastic endocarditis/Endomyocardial fibrosis (EMF), is not known, but it can be treated using antibiotics.
- If you have fibroelastic endocarditis, it's important to take your medication as prescribed by your doctor.
- The treatment for fibroelastic endocarditis will depend on what type of valve is affected and how severe the infection is.
- In some cases, surgery may be needed to replace damaged tissue or repair damaged valves.
- The first step in treating fibroelastic endocarditis is making sure that the infection is under control.
- Few Medications can help manage the symptoms and reduce the chance of complications.
- These medications include pain relievers like ibuprofen (Advil) or naproxen (Aleve), antibiotics to treat infection, blood thinners like warfarin (Coumadin) to decrease blood clotting, beta-blockers to control blood pressure and slow down your heart rate.
- Once the infection has been treated, you'll need to take medication for at least six months to prevent the bacteria from returning.
- Your doctor will decide on the best treatment plan for your specific case, and you should always follow their instructions closely.
- If you have any questions about your treatment plan, don't hesitate to ask your doctor!
Difficulty breathing (dyspnea),Breathlessness,Grunting sounds during breathing,Coughing,Irritability,Weakness,Pale facial appearance (pallor)
Pulmonary venous hypertension,Left ventricular enlargement,Abnormal heartbeat patterns (atrial fibrillation or atrial arrhythmia)