The long QT syndrome: A transatlantic clinical approach to diagnosis and therapy

Peter J. Schwartz, Michael John Ackerman

Research output: Contribution to journalArticle

139 Citations (Scopus)

Abstract

The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors still take place, both in the diagnostic process and in the therapeutic choices. The price of these errors is paid by the patients and their families. This review is not directed to the relatively small number of LQTS experts who know what to do. It does not deal with genetics, with epidemiology, or with the well-known clinical manifestations. We have focused solely on the approach to diagnosis and therapy and we have directed this review to the average clinical cardiologist who, in his/her practice, sees occasionally patients affected or suspected to be affected by LQTS; the cardiologist who may know enough to manage them but not enough to be completely confident on his/her most critical choices. We have provided our personal views without making any attempt to blend differences whenever present. On most issues we agree fully but where we do not, we make it clear to the reader by indicating who is thinking what. The result may be unconventional, but it mirrors the challenges, often severe, that we all face in managing and protecting these patients from sudden death while also helping them live and thrive despite their diagnosis. We trust that this unabashed presentation of our clinical approach will be useful for both cardiologists and patients.

Original languageEnglish (US)
Pages (from-to)3109-3116
Number of pages8
JournalBioinformatics
Volume29
Issue number24
DOIs
StatePublished - Dec 15 2013

Fingerprint

Long QT Syndrome
Therapy
Sudden Death
Epidemiology
Disorder
Mirror
Diagnostics
Therapeutics
Molecular Epidemiology
Review
Cardiologists

Keywords

  • Genetics
  • Implantable cardioverter defibrillator
  • Left cardiac sympathetic denervation
  • Long QT syndrome
  • QT interval
  • Sudden cardiac death

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Computational Theory and Mathematics
  • Computer Science Applications
  • Computational Mathematics
  • Statistics and Probability

Cite this

The long QT syndrome : A transatlantic clinical approach to diagnosis and therapy. / Schwartz, Peter J.; Ackerman, Michael John.

In: Bioinformatics, Vol. 29, No. 24, 15.12.2013, p. 3109-3116.

Research output: Contribution to journalArticle

@article{98cb83e3d4474eeb8de9eccebedde092,
title = "The long QT syndrome: A transatlantic clinical approach to diagnosis and therapy",
abstract = "The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors still take place, both in the diagnostic process and in the therapeutic choices. The price of these errors is paid by the patients and their families. This review is not directed to the relatively small number of LQTS experts who know what to do. It does not deal with genetics, with epidemiology, or with the well-known clinical manifestations. We have focused solely on the approach to diagnosis and therapy and we have directed this review to the average clinical cardiologist who, in his/her practice, sees occasionally patients affected or suspected to be affected by LQTS; the cardiologist who may know enough to manage them but not enough to be completely confident on his/her most critical choices. We have provided our personal views without making any attempt to blend differences whenever present. On most issues we agree fully but where we do not, we make it clear to the reader by indicating who is thinking what. The result may be unconventional, but it mirrors the challenges, often severe, that we all face in managing and protecting these patients from sudden death while also helping them live and thrive despite their diagnosis. We trust that this unabashed presentation of our clinical approach will be useful for both cardiologists and patients.",
keywords = "Genetics, Implantable cardioverter defibrillator, Left cardiac sympathetic denervation, Long QT syndrome, QT interval, Sudden cardiac death",
author = "Schwartz, {Peter J.} and Ackerman, {Michael John}",
year = "2013",
month = "12",
day = "15",
doi = "10.1093/eurheartj/eht089",
language = "English (US)",
volume = "29",
pages = "3109--3116",
journal = "Bioinformatics",
issn = "1367-4803",
publisher = "Oxford University Press",
number = "24",

}

TY - JOUR

T1 - The long QT syndrome

T2 - A transatlantic clinical approach to diagnosis and therapy

AU - Schwartz, Peter J.

AU - Ackerman, Michael John

PY - 2013/12/15

Y1 - 2013/12/15

N2 - The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors still take place, both in the diagnostic process and in the therapeutic choices. The price of these errors is paid by the patients and their families. This review is not directed to the relatively small number of LQTS experts who know what to do. It does not deal with genetics, with epidemiology, or with the well-known clinical manifestations. We have focused solely on the approach to diagnosis and therapy and we have directed this review to the average clinical cardiologist who, in his/her practice, sees occasionally patients affected or suspected to be affected by LQTS; the cardiologist who may know enough to manage them but not enough to be completely confident on his/her most critical choices. We have provided our personal views without making any attempt to blend differences whenever present. On most issues we agree fully but where we do not, we make it clear to the reader by indicating who is thinking what. The result may be unconventional, but it mirrors the challenges, often severe, that we all face in managing and protecting these patients from sudden death while also helping them live and thrive despite their diagnosis. We trust that this unabashed presentation of our clinical approach will be useful for both cardiologists and patients.

AB - The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors still take place, both in the diagnostic process and in the therapeutic choices. The price of these errors is paid by the patients and their families. This review is not directed to the relatively small number of LQTS experts who know what to do. It does not deal with genetics, with epidemiology, or with the well-known clinical manifestations. We have focused solely on the approach to diagnosis and therapy and we have directed this review to the average clinical cardiologist who, in his/her practice, sees occasionally patients affected or suspected to be affected by LQTS; the cardiologist who may know enough to manage them but not enough to be completely confident on his/her most critical choices. We have provided our personal views without making any attempt to blend differences whenever present. On most issues we agree fully but where we do not, we make it clear to the reader by indicating who is thinking what. The result may be unconventional, but it mirrors the challenges, often severe, that we all face in managing and protecting these patients from sudden death while also helping them live and thrive despite their diagnosis. We trust that this unabashed presentation of our clinical approach will be useful for both cardiologists and patients.

KW - Genetics

KW - Implantable cardioverter defibrillator

KW - Left cardiac sympathetic denervation

KW - Long QT syndrome

KW - QT interval

KW - Sudden cardiac death

UR - http://www.scopus.com/inward/record.url?scp=84890060481&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84890060481&partnerID=8YFLogxK

U2 - 10.1093/eurheartj/eht089

DO - 10.1093/eurheartj/eht089

M3 - Article

C2 - 23509228

AN - SCOPUS:84880064816

VL - 29

SP - 3109

EP - 3116

JO - Bioinformatics

JF - Bioinformatics

SN - 1367-4803

IS - 24

ER -