Abstract
Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. The hair phenotype is unique, characterized by congenital woolly, curly, rough, and light-colored scalp hair and sparse eyebrows. However, arrhythmias and severe cardiomyopathies are causes of severe life threatened intracardiac thrombus. Thrombus therapy needs private care and sedative, operative processes need to give close attention to these patients. In this case report, sedation given a patient who having severe congestive heart failure with huge mural thrombus in left ventriculus has been presented.
Highlights
Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair
Naxos disease is a rare autosomal recessive disease that consists of an associated triad of woolly hair, thickened palms and soles, and heart involvement
A nine years old girl patient who has treated with anticongestive therapy for dilated CMP and arrhythmias due to Naxos disease since two years admitted to hospital due to palpitation, syncope and edema
Summary
Naxos disease is a rare autosomal recessive disease that consists of an associated triad of woolly hair, thickened palms and soles (keratoderma), and heart involvement. Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. Sedation given a patient who having severe congestive heart failure with huge mural thrombus in left ventriculus has been presented.
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