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What exactly is arrhythmogenic right ventricular cardiomyopathyand how does it manifest?

It probably sounds terrifying, but this condition is one ofthe most frequent causes of sudden death in young people. The real cause of itis not really known to the scientists, but it is known that children whose oneparent has it are at high risk of inheriting the same condition; to be moreprecise, there is 50 % chance that something like that will happen. As for themain characteristics, the fact is that in people who develop this condition,the normal muscle cells of the right ventricle are progressively being replacedby fat and fibrous tissue, and even though at the beginning only certain partsof the right ventricle are affected, over time the problem becomes global.

The symptoms that are related to arrhythmogenic rightventricular cardiomyopathy include rapid heartbeat, fainting, tiredness, light-headednessand other signs that indicate arrhythmia, although the signs that are typicalof cardiomyopathy (pain in the chest and shortness of breath, for instance) arerarely present. The worst symptom of this condition is sudden death. All ofthis makes the process of coming to a conclusion of the diagnosis, and practicallyimpossible at the early stages. Electrocardiogram, 2-dimensionalechocardiogram, several other tests and scans, and in some cases, even MRI is usually used for that. However, sometimes it is possible to miss it, even afterall these procedures are performed. It is necessary to confirm the presence offat in the right ventricle, the damage in that area and presence of familyhistory of this disease in order to confirm the diagnosis.

What treatment methods are available?

Since the greatest majority of the patients who suffer fromthis heart condition do not develop any symptoms except probably arrhythmia,their treatment is usually focused on the prevention or control of thatproblem. In case the medications do not show any positive results, it might benecessary to implant cardioverter defibrillator or to try with some othermethod of treatment. Catheter ablation is one of the options, and it consistsof inserting a catheter, which should deliver electrical energy to the rightside of the heart, create a small scar and thus prevent transmission of arrhythmia.Another option is surgical ablation, which sometimes requires an open-heartsurgery, but which consists of mapping, identifying and destroying theelectrical pathway of the arrhythmia. However, even with the treatment of the symptoms, it is impossible to cure this condition.

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