PMID- 33133318
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201103
IS  - 1878-5409 (Electronic)
IS  - 1878-5409 (Linking)
VI  - 22
IP  - 5
DP  - 2020 Nov
TI  - Subcutaneous and transvenous implantable cardioverter defibrillator in high-risk 
      long-QT syndrome type 3 associated with Val411Met mutation in SCN5A.
PG  - 238-241
LID - 10.1016/j.jccase.2020.07.007 [doi]
AB  - Congenital long-QT syndrome type 3 (LQT3) with SCN5A-V411M mutation has been 
      reported as a malignant case of LQT3 with highest risk for sudden cardiac death 
      (SCD). Here, we present two cases of LQT3 with SCN5A-V411M who had been implanted 
      with subcutaneous (S-) or transvenous (TV-) implantable cardioverter 
      defibrillators (ICD). Case 1, a 2-year-old boy, although he had no symptoms, was 
      diagnosed as having LQT3 (V411M-SCN5A) due to family history. The QTc interval 
      was still longer than 500 ms during follow-up even under oral mexiletine. Case 2 
      (his aunt) diagnosed as LQT3 suffered from syncope caused by ventricular 
      fibrillation at 35-years-old despite taking mexiletine. Furthermore, case 1's 
      father and half-brother, both had the V411M mutation with LQT3, had suddenly 
      died. Thus, case 1 was recommended S-ICD when he was 15-years-old for primary 
      prevention of SCD but not necessary for pacing therapy, while, case 2 had been 
      implanted TV-ICD for secondary prevention of SCD. They had no event after ICD 
      implantation, however, case 2 had to have added an extra ICD-lead due to lead 
      failure when she was 44-years-old. The S-ICD may be a potent therapeutic option 
      for high-risk LQTS when patients are younger and do not need pacing therapy. 
      <Learning objective: In congenital long-QT syndrome (LQTS) type 3, some of the 
      first events are lethal, particularly, LQT3 with V411M-SCN5A mutation is the 
      highest risk for sudden cardiac death (SCD). Which implantable cardioverter 
      defibrillator (ICD), transvenous (TV-ICD) or subcutaneous (S-ICD) is better for 
      primary prevention of SCD in LQTS is still controversial. The S-ICD rather than 
      TV-ICD may have a potent benefit for high-risk LQTS when patients are younger and 
      do not need pacing therapy.>.
CI  - (c) 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights 
      reserved.
FAU - Yokoyama, Yasuhiro
AU  - Yokoyama Y
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Aiba, Takeshi
AU  - Aiba T
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Ueda, Nobuhiko
AU  - Ueda N
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Nakajima, Kenzaburo
AU  - Nakajima K
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Kamakura, Tsukasa
AU  - Kamakura T
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Wada, Mitsuru
AU  - Wada M
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Yamagata, Kenichiro
AU  - Yamagata K
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Ishibashi, Kohei
AU  - Ishibashi K
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Inoue, Yuko
AU  - Inoue Y
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Miyamoto, Koji
AU  - Miyamoto K
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Nagase, Satoshi
AU  - Nagase S
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Noda, Takashi
AU  - Noda T
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Yasuda, Satoshi
AU  - Yasuda S
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
FAU - Shimizu, Wataru
AU  - Shimizu W
AD  - Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan.
FAU - Kusano, Kengo
AU  - Kusano K
AD  - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular 
      Center, Suita, Japan.
LA  - eng
PT  - Case Reports
DEP - 20200811
PL  - Japan
TA  - J Cardiol Cases
JT  - Journal of cardiology cases
JID - 101549579
PMC - PMC7588482
OTO - NOTNLM
OT  - Implantable cardioverter defibrillator
OT  - Long-QT syndrome
OT  - SCN5A
OT  - Sudden cardiac death
EDAT- 2020/11/03 06:00
MHDA- 2020/11/03 06:01
PMCR- 2020/08/11
CRDT- 2020/11/02 06:16
PHST- 2020/04/28 00:00 [received]
PHST- 2020/06/20 00:00 [revised]
PHST- 2020/06/25 00:00 [accepted]
PHST- 2020/11/02 06:16 [entrez]
PHST- 2020/11/03 06:00 [pubmed]
PHST- 2020/11/03 06:01 [medline]
PHST- 2020/08/11 00:00 [pmc-release]
AID - S1878-5409(20)30086-4 [pii]
AID - 10.1016/j.jccase.2020.07.007 [doi]
PST - epublish
SO  - J Cardiol Cases. 2020 Aug 11;22(5):238-241. doi: 10.1016/j.jccase.2020.07.007. 
      eCollection 2020 Nov.