PMID- 30642644
OWN - NLM
STAT- In-Data-Review
LR  - 20190201
IS  - 1874-1754 (Electronic)
IS  - 0167-5273 (Linking)
VI  - 279
DP  - 2019 Mar 15
TI  - The necessity of implantable cardioverter defibrillators in patients with
      Kearns-Sayre syndrome - systematic review of the articles.
PG  - 105-111
LID - S0167-5273(18)35565-7 [pii]
LID - 10.1016/j.ijcard.2018.12.064 [doi]
AB  - 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.
CI  - Copyright (c) 2018 Elsevier B.V. All rights reserved.
FAU - Imamura, Tomohiko
AU  - Imamura T
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Sumitomo, Naokata
AU  - Sumitomo N
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan. Electronic
      address: sumitomo@saitama-med.ac.jp.
FAU - Muraji, Shota
AU  - Muraji S
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Mori, Hitoshi
AU  - Mori H
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Osada, Yousuke
AU  - Osada Y
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Oyanagi, Takayuki
AU  - Oyanagi T
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Kojima, Takuro
AU  - Kojima T
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Yoshiba, Shigeki
AU  - Yoshiba S
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Kobayashi, Toshiki
AU  - Kobayashi T
AD  - Department of Pediatric Cardiology, Saitama Medical University International
      Medical Center, 1397-1, Yamane, Hidaka, Saitama 350-1298, Japan.
FAU - Ono, Katsushige
AU  - Ono K
AD  - Department of Pathophysiology, Oita University, 1397-1, Yamane, Hidaka, Saitama
      350-1298, Japan.
LA  - eng
PT  - Journal Article
DEP - 20181227
PL  - Netherlands
TA  - Int J Cardiol
JT  - International journal of cardiology
JID - 8200291
OTO - NOTNLM
OT  - Atrioventricular block
OT  - Implantable cardioverter defibrillator
OT  - Kearns-Sayre syndrome
OT  - Long QT
OT  - Ventricular fibrillation
EDAT- 2019/01/16 06:00
MHDA- 2019/01/16 06:00
CRDT- 2019/01/16 06:00
PHST- 2018/10/01 00:00 [received]
PHST- 2018/12/06 00:00 [revised]
PHST- 2018/12/21 00:00 [accepted]
PHST- 2019/01/16 06:00 [pubmed]
PHST- 2019/01/16 06:00 [medline]
PHST- 2019/01/16 06:00 [entrez]
AID - S0167-5273(18)35565-7 [pii]
AID - 10.1016/j.ijcard.2018.12.064 [doi]
PST - ppublish
SO  - Int J Cardiol. 2019 Mar 15;279:105-111. doi: 10.1016/j.ijcard.2018.12.064. Epub
      2018 Dec 27.