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Anaesthesia in Naxos disease: First case report

Hüseyin Yildiz, Emin Silay, Ismail Coskuner, Kemal Ozyurt, Seref Olgar, Nimet Senoglu, Hafize Oksuz


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.


intracardiac mural thrombus; congestive heart failure; Naxos disease; anaesthesia

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