Anaesthesia in Naxos disease: First case report

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

doi:10.17305/bjbms.2013.2421

Authors

Hüseyin Yildiz Department of Anaesthesiology and Reanimation, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Emin Silay Department of Dermatology, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Ismail Coskuner Department of Anaesthesiology and Reanimation, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Kemal Ozyurt Department of Dermatology, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Seref Olgar Department of Paediatrics, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Nimet Senoglu Department of Anaesthesiology and Reanimation, Faculty of Medicine, Kahramanmaras Sutcu Imam University
Hafize Oksuz Department of Anaesthesiology and Reanimation, Faculty of Medicine, Kahramanmaras Sutcu Imam University

DOI:

https://doi.org/10.17305/bjbms.2013.2421

Keywords:

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

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.