Permanent Pacemaker Implantation in a Patient with MELAS Syndrome.
- Author:
Ji Hyun MIN
1
;
Jong Hwa AHN
;
Jeong Mi LEE
;
Jeong Rang PARK
;
Jin Sin KOH
;
Jin Yong HWANG
;
Choong Hwan KWAK
Author Information
1. Department of Internal Medicine, Gyeongsang National University School of Medicine, Jinju, Korea. cwakch@korea.com
- Publication Type:Case Report
- Keywords:
MELAS syndrome;
Cardiac sinus arrest;
Syncope;
Cardiomyopathy, Hypertrophic;
Cardiac pacemaker, Artificial
- MeSH:
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
- From:Korean Journal of Medicine
2013;84(2):265-268
- CountryRepublic of Korea
- Language:Korean
-
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.