What is congenital endocardial Fibroelastosis?

Endocardial fibroelastosis (EFE) is a rare heart disorder that affects infants and children. It is characterized by a thickening within the muscular lining of the heart chambers due to an increase in the amount of supporting connective tissue (inelastic collagen) and elastic fibers.

What does Fibroelastosis mean?

: a condition of the body or one of its organs (as the left ventricle of the heart) characterized by proliferation of fibroelastic tissue — see endocardial fibroelastosis.

What is Subendocardial fibrosis?

Endomyocardial fibrosis (EMF), also referred to as Loffler and Davies disease, is a form of restrictive cardiomyopathy of unknown etiology characterized by endocardial fibrosis of the apices and inflow tracts of the right ventricle, left ventricle, or both, consequently leading to ventricular obliteration and …

What are the signs and symptoms of left-sided heart failure?

Left-sided Heart Failure Symptoms

Shortness of breath.
Difficulty breathing when lying down.
Weight gain with swelling in the feet, legs, ankles.
Fluid collection in the abdomen.
Fatigue or a general feeling of weakness.

Can you live a long life with pulmonary fibrosis?

When you do your research, you may see average survival is between three to five years. This number is an average. There are patients who live less than three years after diagnosis, and others who live much longer.

What causes Pleuroparenchymal Fibroelastosis?

Etiology. The cause of idiopathic pleuroparenchymal fibroelastosis is unknown, but clinical data suggest a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms are also thought to play a role in this disease.

How serious is myocardial fibrosis?

Myocardial fibrosis is an important part of cardiac remodeling that leads to heart failure and death. Myocardial fibrosis results from increased myofibroblast activity and excessive extracellular matrix deposition. Various cells and molecules are involved in this process, providing targets for potential drug therapies.

What are the symptoms of cardiac fibrosis?

The lungs and the lymph glands between the lungs are frequently affected, and symptoms may include coughing and difficulty breathing. The heart may also be affected, resulting in abnormal heartbeat patterns (conduction block, atrial arrhythmia or ventricular arrhythmia) and/or heart failure.

Can you have fibrosis of the heart?

Cardiac fibrosis is a scarring event in the cardiac muscle that is characterized by an increased collagen type I deposition as well as cardiac fibroblast activation and differentiation into myofibroblasts [5].

What is the definition of endocardial fibroelastosis?

General Discussion. Endocardial fibroelastosis (EFE) is a rare heart disorder that affects infants and children. It is characterized by a thickening within the muscular lining of the heart chambers due to an increase in the amount of supporting connective tissue (inelastic collagen) and elastic fibers. The normal heart has four chambers.

How does endocardial fibroelastosis affect infants and children?

Is there a last resort for endocardial fibroelastosis?

A last resort form of treatment is heart transplant. An infant with dilated, failing heart was no rarity on the pediatric wards of hospitals in the mid-twentieth century. On autopsy, most of these patients’ hearts showed the thickened endocardial layer noted above.

How did Coxsackie virus lead to endocardial fibroelastosis?

Evidence gradually accumulated as to the role of infection as one such type of stress. The studies of Fruhling and colleagues in 1962 were critical. They followed a series of epidemics of Coxsackie virus infection in their part of France. After each epidemic there were increased numbers of cases with EFE coming to autopsy.