Definition:
Naxos Disease is an autosomal recessive modification of Arrhythmogenic right ventricular dysplasia (ARVD, also known as Arrhythmogenic right ventricular cardiomyopathy or ARVC). It is a very rare syndrome characterized primarily by the heart disease and wooly hair.
Diagnosis:
In order to make the diagnosis of Naxos Disease being a variant of ARVD includes the following:
* Right Ventricular Angiography, which is considered to be the gold standard in the diagnosis of all ARVD.
* Electrocardiogram (EKG or ECG), which has the ability to record the electrical activity of the heart overtime.
* Echocardiography is also called as the ultrasound of the heart.
* Cardiac MRI
Treatment:
Treatment of Naxos Disease is achieved through antiarrhythmic drugs and heart transplants.
Symptoms and Signs:
The symptoms of Naxos Disease are more critical and severe compared to autosomal dominant Arrhythmogenic right ventricular dysplasia (ARVD). The following are some of the symptoms of this disorder:
* Heart disease
* Wooly hair
* Thickened skin on soles
* Thickened skin on palms
* Fainting
* Ventricular tachycardia
* Rough hair
* Dry hair
* Bristly hair
Causes:
Being a variant of ARVD, the real cause of Naxos Disease is still unknown; however there are two pathological patterns seen in Arrhythmogenic Right Ventricular Dysplasia (ARVD), fatty infiltration involving a partial or near completion of myocardium with fatty tissue without wall thinning and fibro-fatty infiltration, which involves replacement of myocytes with fibrofatty tissue.
The information on this site is for research purposes only and can not substitute for the advice of a medical professional. Ailments.com is not run by doctors and does not engage in the practice of medicine. Our site is not a medical authority, it is just a research tool for you to use in preparation for consulting with a doctor. We can not guarantee our information is accurate or up to date. Even if a statement made about a medical condition is accurate, it may not apply to you or your symptoms, so you should always consult a doctor. Nothing on our site should be construed as an attempt to offer or render a medical opinion or otherwise engage in the practice of medicine.
