동기능 부전을 동반한 MELAS 증후군 1예

• Published in: The Korean journal of medicine pp. 265 - 268
• Main Authors: 민지현, 안종화, 이정미, 박정랑, 고진신, 황진용, 곽충환
• Format: Journal Article
• Language: Korean
• Published: 대한내과학회 01.02.2013
• Subjects: 내과학
• ISSN: 1738-9364; 2289-0769
• DOI: 10.3904/kjm.2013.84.2.265

MELAS 증후군은 비교적 드문 질환으로 비후성 심근증, WPW 증후군, 심장전도장애 등의 심장 관련 증상이 발생할 수 있다. 동기능 이상과 동반된 MELAS 증후군은 거의 알려진바 없어, 실신을 동반한 동정지로 영구형 심박동기 시술을 시행한 MELAS 증후군 환자 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. A 35-year-old male patient with heart and renal failure and pneumonia was transferred to our department due to recurrent cardiac standstill with syncope. He had been diagnosed as and treated for MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome for the past 3 years. Electrocardiography (ECG) showed the Wolff-Parkinson-White pattern, and an echocardiogram showed hypertrophic cardiomyopathy. He developed syncopal attacks intermittently, and ECG monitoring showed intermittent bradycardia. His Holter monitoring showed several episodes of 5-16 seconds of sinus arrest. We conducted an electrophysiological study to evaluate the arrhythmia. During atrial and ventricular extra-stimuli, cardiac standstill developed several times, and the duration of pauses varied from 2.5 to 5.5 seconds. Abrupt asystolic events also developed accompanying syncopal attacks that were not related to the extra-stimuli. We decided to implant a permanent pacemaker. The patient’s syncopal episodes disappeared after implantation of a DDD type pacemaker. KCI Citation Count: 0