Cardiac fibrosis
Synonyms and keywords:Davies disease

# Overview
Cardiac fibrosis refers to an abnormal thickening of the heart valves due to inappropriate proliferation of cardiac fibroblasts.Endomyocardial fibrosis(EMF) chareacterized by deposite of fibrous  and thickening of the endocardium and myocardium ventricles that leading restrictive cardiomyopathy.It is a neglected disorder that usually prodominant in tropical areas of developing and low -income countries.

# Historical Perspective
First description for endomyocardial fibrosis was in 1948 by Davies in Uganda.
Endomyocardial fibrosis was first discovered  in 1984 in Ugenda.
There have been several outbreaks of endomycardial fibrosis, including Africa,Asia,and South America.

# Classification
Myocardial fibrosis may be classified  into two groups:interstitial fibrosis (diffuse),replacement fibrosis (scar) .
The interstitial fibrosis divided in to two subclasses:reactive and infiltrative.

# Pathophysiology
It is thought that cardiac fibrosis is the result of remodeling of extracellular and deposition of extracellular matrix that causes to impaired muscles function.
Also cardiac fibrosis is mediated by  cardial fibrosis include Eosinophilia, Infectious such as toxoplasma, malaria,helminthic parasites,rheumatic fever,Environmental exposure such as cerium ,casava ,Immunologic and Genetic.

# Causes
The most common cause of cardiac fibrosis is  cardiac fibrotic scars that usually  occure after myocardial infarction . other causes of cardiac fibrotic scars include , hypertensive heart disease,diabetic hypertrophic cardiomyopathy and ,idiopathic dilated cardiomyopathy.

# Differentiating ((Page name)) from other Diseases
cardiac fibrosis must be differentiated from other diseases that cause heart failure,premature death in children and adults.

# Epidemiology and Demographics
Endomyocardial fibrosis is the most cause of heart failure,that up 20% of cases are in endmic area.
Recently the incidence of EMF has been decreased.
In one of the researches in 2008  the prevalence of EMF was 19.8%.
The incidence of EMF is approximately 10,000,000 people worldwide.
The incidence of endomycardial fibrosis is more common in Africa,Asia and South America.
The most cases in Uganda,Mozambique and some west of Africa are Endemic for EMF.
Although sporadic cases have been reported in Congo and Malawi.
In India the most cases are in rain forest area of Kerala State.
In china we can see the high frequency of EMF in Guanxi.
And in south of America , Brazil and Colombia are the most frequent area.
Patients of all age groups may develop EMF butcommonly affects young adults between 10 to 30 years old with poor socioeconomic status.
In some researches EMF affects men and women equally but prevalence of EMF in Uganda,is 2-fold higher in women of childbearing age .
The majority of EMF  cases are reported in Africa.

# Risk Factors
Common risk factors in the development of cardiac fibrosis may be occupational, environmental, genetic, and viral.
Common risk factors in the development of cardiac fibrosis include Obesity, Metabolic dysfunction,DM,HTN.

# Screening
There is insufficient evidence to recommend routine screening for cardiac fibrosis but in high risk individuals or in endemic areas we can use echocardiography as screening.

# Natural History, Complications, and Prognosis
Common complications of cardiac fibrosis  include stifness of left ventricle,heart failure,arrihythmias.
Prognosis is very poor.

# Diagnosis

## Diagnostic Study of Choice
The diagnosis and severity of cardiac fibrosis  according to classification is made when we have 2 major criteria or 1 major criterion with 2 minor criteria.(table below)

## History and Symptoms
EMF symptoms divided in to two phases:Acute , Chronic
The most common symptoms of acute  include febrile illness ,pancarditis,eosinophilia,dyspenea,itching, pericardial effusion.
Symptoms of chronic phase deponds on biventricular  or isolated  right/left ventricular involvement(RV heart failure/LV heart failure).
In right ventricular involvement  most common symptoms are :systemic venous HTN,facial edema,exophtalmos,jugular vein distention,gross hepatomegaly,splenomegaly and abdomonal swelling.

## Physical Examination
Patients with cardiac fibrosis usually appear ill. 
Common physical examination findings of cardial fibrosis causing heart failure include,facial and periorbital edema, acscites,jvp distention,fever,pericardial friction rub due to pericarditis and non specific signs in chronic phase are clubbing of digits,growth retardation ,testicular atrophy, cachexia.

## Laboratory Findings
Biomarkers of fibrosis can be detected in blood tests.
In 2015 Begon Lopez and teammates have been researched on using collagenouse biomarkers for diagnos of cardiac fibrosis.
According to this article there is two type of collagen-derived serum peptids have been related to cardiac fibrosis:
Carboxy-terminal propeptide of procollagen type 1(P1CP),Amino-terminal propeptide of procollagen type 3(P3NP).

## Electrocardiogram
An ECG may be helpful in the diagnosis of cardiac fibrosis. Findings on an ECG include RA abnormality,peaking and increased P wave amplitude, if right ventricle involved we will have R wave in lead V1, and AF is common in patients with cardiac fibrosis.

## X-ray
An x-ray may be helpful in the diagnosis of cardiac fibrosis. Findings on an x-ray  include cardiomrgaly and may we have silhouette oligemic pulmonary fields.

## Echocardiography or Ultrasound
Findings on an echocardiography suggestive of endomyocardial fibrosis include small ventricle with obliteration of the apex with large atrium .
Oblitration by fibrosis at ventricular apex can change ventricular  morphology,that called mushroom sign.

## CT scan
Cardiac calcifications or intracavitary thrombi may be finded in cardiac  CT scan.

## MRI
Cardiac MRI may be helpful in the diagnosis of EMF. Findings on MRI diagnostic of EMF include small ventricles with hypertrophy in the apical,also shows hypoperfused myocardial areas and  we can see fibrosis in late -gadolinium -enhancement 
images.

## Other Imaging Findings
There are no other imaging findings associated with cardiac fibrosis.

## Other Diagnostic Studies
There are no other diagnostic studies associated with cardiac fibrosis.

# Treatment

## Medical Therapy
Supportive therapy  of heart failure for EMF includes diuretics, angiotensin-converting enzyme inhibitors with combination therapy with asprin or anticoagulation.

## Surgery
Surgery is the mainstay of treatment for cardiac fibrosis.
Compare with medical therapy , surgery can increase patients survival.Surgery of EMF include endocardiotomy and valve replacement or repair.

## Primary Prevention
There are no established measures for the primary prevention of cardiac fibrosis.

## Secondary Prevention
Effective measures for the secondary prevention of cardiac fibrosis include screening echocardiography in endemic areas.