Restrictive Cardiomyopathy

The restrictive cardiomyopathy is a form of Cardiomyopathie (literally, disease of the cardiac muscle ) secondary to a loss of the flexibility of the walls of the cardiac cavities, mainly of the ventricles, (reduction in cardiac compliance), which involves a resistance to flow normal Sang uin.

Epidemiology

August 1st

Physiopathology

Physiopathological mechanisms

The rate/rhythm and the contractility of the heart can be normal, but the walls " enraidies" cardiac cavities prevent them from filling correctly of blood.
Les walls of the ventricles less flexible but is not necessarily thickened.

Causes

It can be Idiopathique (no cause is identified), or secondary with various pathologies such as a endomyocardic Fibrose (tropical, Syndrome hypereosinophilic idiopathic), a infiltrative disease (Amylose, Sarcoïdose), a metabolic disease (Maladie of Left-handed person, Mucopolysaccharidose S, Maladie of Fabry, syndrome carcinoïde). It was also described in the continuations of Radiothérapie.

The subjects reached of a idiopathic form can have a family history of Cardiomyopathie.

Diagnosis

The functional signs are not specific and gather Asthénie (tiredness), Dyspnée (breathlessness), Palpitation S, edema. The diagnosis is made by the cardiac echography. The other examinations (the electrocardiogram, and the cardiac Catheterization) are not necessary to the diagnosis but appreciate its repercussion.

Assumption of responsibility

The heart Transplant is the only treatment allowing a prolonged survival. There does not exist curator treatment. The goal of the treatment is to improve the Symptôme S.
Chez the patients presenting of the disorders of the cardiac rhythm or of the turbid of conduction can be proposed a Pacemaker.

Evolution and complications

The idiopathic restrictive cardiomyopathy of the child is almost always fatal. Only the heart transplant is possible. The complications are mainly the embolic Troubles cardiac rhythm and complications .

Notes and references of the article

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