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KMID : 0882420130840020265
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Korean Journal of Medicine
2013 Volume.84 No. 2 p.265 ~ p.268
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Permanent Pacemaker Implantation in a Patient with MELAS Syndrome
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Min Ji-Hyun
Ahn Jong-Hwa
Lee Jeong-Mi
Park Jeong-Rang
Koh Jin-Sin
Hwang Jin-Yong
Kwak Choong-Hwan
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Abstract
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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.
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KEYWORD
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MELAS syndrome, Cardiac sinus arrest, Syncope, Cardiomyopathy, Hypertrophic, Cardiac pacemaker, Artificial
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