Korean J Med.  2013 Feb;84(2):265-268.

Permanent Pacemaker Implantation in a Patient with MELAS Syndrome

• Min JH
• Ahn JH
• Lee JM
• Park JR
• Koh JS
• Hwang JY
• Kwak CH

Abstract

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.

Keyword

MELAS syndrome; Cardiac sinus arrest; Syncope; Cardiomyopathy, Hypertrophic; Cardiac pacemaker, Artificial

MeSH Terms

Acidosis, Lactic
Arrhythmias, Cardiac
Bradycardia
Cardiomyopathy, Hypertrophic
Dichlorodiphenyldichloroethane
Electrocardiography
Electrocardiography, Ambulatory
Heart
Humans
Male
MELAS Syndrome
Muscular Diseases
Pacemaker, Artificial
Pneumonia
Renal Insufficiency
Sinus Arrest, Cardiac
Syncope
Dichlorodiphenyldichloroethane