The necessity of implantable cardioverter defibrillators in patients with Kearns-Sayre syndrome - systematic review of the articles -. (15th March 2019)

• Record Type: Journal Article
• Authors: Imamura, Tomohiko; Sumitomo, Naokata; Muraji, Shota; Mori, Hitoshi; Osada, Yousuke; Oyanagi, Takayuki; Kojima, Takuro; Yoshiba, Shigeki; Kobayashi, Toshiki; Ono, Katsushige
• Is Part Of: International journal of cardiology. Volume 279(2019)
• Page Start: 105
• Page End: 111
• Publication Date: 2019-03-15
• Subjects: KSS Kearns-Sayre syndrome -- AVB atrioventricular block -- CAVB complete atrioventricular block -- PMVT polymorphic ventricular tachycardia -- MMVT monomorphic tachycardia -- VF ventricular fibrillation -- PMI pacemaker implantation -- ICD implantable cardioverter-defibrillator -- SCD sudden cardiac death -- MVP mitral valve prolapse -- MR mitral valve regurgitation -- CPEO chronic progressive external ophthalmoplegia -- AHA American Heart Association -- ACC American College of Cardiology -- HRS Heart Rhythm Society -- ESC European Society of Cardiology -- CLBBB complete left bundle brunch block -- CoQ10 Coenzyme Q10 -- ICU intensive care unit -- ECG electrocardiogram -- TdP torsade de pointes -- CPA cardiac pulmonary arrest -- DCM dilated cardiomyopathy -- LVNC left ventricular non-compaction cardiomyopathy -- CRBBB complete right bundle brunch block -- ATP anti-tachycardia pacing -- CRT-D cardiac resynchronization therapy with defibrillator -- S-ICD subcutaneous implantable cardioverter-defibrillator -- MELAS mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes -- WPW Wolff-Parkinson-White -- APD action potential duration -- EAD early afterdepolarization -- DAD delayed afterdepolarization; Implantable cardioverter defibrillator -- Ventricular fibrillation -- Long QT -- Kearns-Sayre syndrome -- Atrioventricular block; Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2018.12.064
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: The most common cardiac feature of Kearns-Sayre syndrome (KSS) is atrioventricular block (AVB), and pacemaker implantations (PMIs) are recommended for KSS patients with advanced AVB. However, some KSS patients develop fatal arrhythmias such as polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) and die suddenly even after PMIs. We report a patient with KSS who developed PMVT, VF, and QT prolongation, and was treated with mexiletine and successfully managed with an implantable cardioverter defibrillator (ICD). We reviewed the literature on arrhythmias in KSS published from 1975 to 2018. There were 112 patients with arrhythmia-associated KSS, 10 died, and 6 died suddenly after the PMI. The first manifestation of an arrhythmia was bundle branch block, then it progressed to AVB, and developed into complete AVB (CAVB) in about half the KSS patients. Ventricular arrhythmias were documented in 12 patients, and 8 were implanted with defibrillators afterwards. One patient after the implantation of a cardiac resynchronization therapy defibrillator (CRT-D) was treated for VF by an appropriate shock. This fact suggested that VF occurred even under proper pacing, and that defibrillators were effective. Pacemakers may suppress early afterdepolarizations (EADs) associated with a QT prolongation due to bradycardia. Similarly, mexiletine may suppress EADs by blocking the late sodium and Ca currents. Ventricular arrhythmias observed under suppression of EADs may be caused by delayed afterdepolarization (DADs) via an increasing intracellular Ca concentration due to mitochondrial dysfunction. Therefore, a PMI alone may not be sufficient to prevent sudden death, and an ICD implantation should be necessary. Highlights: The cause of sudden death of Kearns-Sayre syndrome may not be due to atrioventricular block but ventricular arrhythmias. EADs following bradycardia and DADs by a Ca loading due to mitochondrial abnormalities may cause the fatal arrhythmias. A pacemaker is not sufficient, and an ICD is necessary to prevent sudden death in patients with Kearns-Sayre syndrome.