Management of Congenital Long-QT Syndrome: Commentary From the Experts

Elizabeth S. Kaufman; Lee L. Eckhardt; Michael J. Ackerman; Peter F. Aziz; Elijah R. Behr; Marina Cerrone; Mina K. Chung; Michael J. Cutler; Susan P. Etheridge; Andrew D. Krahn; Steven A. Lubitz; Marco V. Perez; Silvia G. Priori; Jason D. Roberts; Dan M. Roden; Eric Schulze-Bahr; Peter J. Schwartz; Wataru Shimizu; M. Benjamin Shoemaker; Raymond W. Sy; Jeffrey A. Towbin; Sami Viskin; Arthur A.m. Wilde; Wojciech Zareba

doi:10.1161/CIRCEP.120.009726

Management of Congenital Long-QT Syndrome: Commentary From the Experts

Elizabeth S. Kaufman^*
, Lee L. Eckhardt
, Michael J. Ackerman
, Peter F. Aziz
, Elijah R. Behr
, Marina Cerrone
, Mina K. Chung
, Michael J. Cutler
, Susan P. Etheridge
, Andrew D. Krahn
, Steven A. Lubitz
, Marco V. Perez
, Silvia G. Priori
, Jason D. Roberts
, Dan M. Roden
, Eric Schulze-Bahr
, Peter J. Schwartz
, Wataru Shimizu
, M. Benjamin Shoemaker
, Raymond W. Sy

Jeffrey A. Towbin, Sami Viskin, Arthur A.m. Wilde, Wojciech Zareba

^*Corresponding author for this work

Clinical Departments

Case Western Reserve University
University of Wisconsin-Madison
Mayo Clinic Rochester, MN
Mayo Clinic College of Medicine and Science
Cleveland Clinic Foundation
St. George's University of London
New York University
Primary Children's Medical Center
University of Utah
University of British Columbia
Massachusetts General Hospital
Stanford University
IRCCS Istituti Clinici Scientifici Maugeri S.p.A. SB - Pavia
University of Pavia
Hamilton Health Sciences
Vanderbilt University
University of Münster
IRCCS Istituto Auxologico Italiano - Milano
Nippon Medical School
Royal Prince Alfred Hospital
The University of Sydney
University of Tennessee Health Science Center
University of Amsterdam
University of Rochester

Research output: Contribution to journal › Review article › peer-review

21 Scopus citations

Abstract

While published guidelines are useful in the care of patients with long-QT syndrome, it can be difficult to decide how to apply the guidelines to individual patients, particularly those with intermediate risk. We explored the diversity of opinion among 24 clinicians with expertise in long-QT syndrome. Experts from various regions and institutions were presented with 4 challenging clinical scenarios and asked to provide commentary emphasizing why they would make their treatment recommendations. All 24 authors were asked to vote on case-specific questions so as to demonstrate the degree of consensus or divergence of opinion. Of 24 authors, 23 voted and 1 abstained. Details of voting results with commentary are presented. There was consensus on several key points, particularly on the importance of the diagnostic evaluation and of β-blocker use. There was diversity of opinion about the appropriate use of other therapeutic measures in intermediate-risk individuals. Significant gaps in knowledge were identified.

Original language	English
Pages (from-to)	E009726
Journal	Circulation: Arrhythmia and Electrophysiology
Volume	14
Issue number	7
DOIs	https://doi.org/10.1161/CIRCEP.120.009726
State	Published - 1 Jul 2021

Funding

Funders	Funder number
National Heart, Lung, and Blood Institute	R01HL139731, R01HL128598, R01HL139738

Keywords

defibrillators, implantable
genetic testing
knowledge
long-QT syndrome
politics

Access to Document

10.1161/CIRCEP.120.009726

Cite this

Kaufman, E. S., Eckhardt, L. L., Ackerman, M. J., Aziz, P. F., Behr, E. R., Cerrone, M., Chung, M. K., Cutler, M. J., Etheridge, S. P., Krahn, A. D., Lubitz, S. A., Perez, M. V., Priori, S. G., Roberts, J. D., Roden, D. M., Schulze-Bahr, E., Schwartz, P. J., Shimizu, W., Shoemaker, M. B., ... Zareba, W. (2021). Management of Congenital Long-QT Syndrome: Commentary From the Experts. Circulation: Arrhythmia and Electrophysiology, 14(7), E009726. https://doi.org/10.1161/CIRCEP.120.009726

@article{5b3103afc433435d9e346c91bba8d92d,

title = "Management of Congenital Long-QT Syndrome: Commentary From the Experts",

abstract = "While published guidelines are useful in the care of patients with long-QT syndrome, it can be difficult to decide how to apply the guidelines to individual patients, particularly those with intermediate risk. We explored the diversity of opinion among 24 clinicians with expertise in long-QT syndrome. Experts from various regions and institutions were presented with 4 challenging clinical scenarios and asked to provide commentary emphasizing why they would make their treatment recommendations. All 24 authors were asked to vote on case-specific questions so as to demonstrate the degree of consensus or divergence of opinion. Of 24 authors, 23 voted and 1 abstained. Details of voting results with commentary are presented. There was consensus on several key points, particularly on the importance of the diagnostic evaluation and of β-blocker use. There was diversity of opinion about the appropriate use of other therapeutic measures in intermediate-risk individuals. Significant gaps in knowledge were identified.",

keywords = "defibrillators, implantable, genetic testing, knowledge, long-QT syndrome, politics",

author = "Kaufman, \{Elizabeth S.\} and Eckhardt, \{Lee L.\} and Ackerman, \{Michael J.\} and Aziz, \{Peter F.\} and Behr, \{Elijah R.\} and Marina Cerrone and Chung, \{Mina K.\} and Cutler, \{Michael J.\} and Etheridge, \{Susan P.\} and Krahn, \{Andrew D.\} and Lubitz, \{Steven A.\} and Perez, \{Marco V.\} and Priori, \{Silvia G.\} and Roberts, \{Jason D.\} and Roden, \{Dan M.\} and Eric Schulze-Bahr and Schwartz, \{Peter J.\} and Wataru Shimizu and Shoemaker, \{M. Benjamin\} and Sy, \{Raymond W.\} and Towbin, \{Jeffrey A.\} and Sami Viskin and \{A.m. Wilde\}, Arthur and Wojciech Zareba",

note = "Publisher Copyright: {\textcopyright} 2021 American Heart Association, Inc.",

year = "2021",

month = jul,

day = "1",

doi = "10.1161/CIRCEP.120.009726",

language = "אנגלית",

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Kaufman, ES, Eckhardt, LL, Ackerman, MJ, Aziz, PF, Behr, ER, Cerrone, M, Chung, MK, Cutler, MJ, Etheridge, SP, Krahn, AD, Lubitz, SA, Perez, MV, Priori, SG, Roberts, JD, Roden, DM, Schulze-Bahr, E, Schwartz, PJ, Shimizu, W, Shoemaker, MB, Sy, RW, Towbin, JA, Viskin, S, A.m. Wilde, A & Zareba, W 2021, 'Management of Congenital Long-QT Syndrome: Commentary From the Experts', Circulation: Arrhythmia and Electrophysiology, vol. 14, no. 7, pp. E009726. https://doi.org/10.1161/CIRCEP.120.009726

TY - JOUR

T1 - Management of Congenital Long-QT Syndrome

T2 - Commentary From the Experts

AU - Kaufman, Elizabeth S.

AU - Eckhardt, Lee L.

AU - Ackerman, Michael J.

AU - Aziz, Peter F.

AU - Behr, Elijah R.

AU - Cerrone, Marina

AU - Chung, Mina K.

AU - Cutler, Michael J.

AU - Etheridge, Susan P.

AU - Krahn, Andrew D.

AU - Lubitz, Steven A.

AU - Perez, Marco V.

AU - Priori, Silvia G.

AU - Roberts, Jason D.

AU - Roden, Dan M.

AU - Schulze-Bahr, Eric

AU - Schwartz, Peter J.

AU - Shimizu, Wataru

AU - Shoemaker, M. Benjamin

AU - Sy, Raymond W.

AU - Towbin, Jeffrey A.

AU - Viskin, Sami

AU - A.m. Wilde, Arthur

AU - Zareba, Wojciech

PY - 2021/7/1

Y1 - 2021/7/1

N2 - While published guidelines are useful in the care of patients with long-QT syndrome, it can be difficult to decide how to apply the guidelines to individual patients, particularly those with intermediate risk. We explored the diversity of opinion among 24 clinicians with expertise in long-QT syndrome. Experts from various regions and institutions were presented with 4 challenging clinical scenarios and asked to provide commentary emphasizing why they would make their treatment recommendations. All 24 authors were asked to vote on case-specific questions so as to demonstrate the degree of consensus or divergence of opinion. Of 24 authors, 23 voted and 1 abstained. Details of voting results with commentary are presented. There was consensus on several key points, particularly on the importance of the diagnostic evaluation and of β-blocker use. There was diversity of opinion about the appropriate use of other therapeutic measures in intermediate-risk individuals. Significant gaps in knowledge were identified.

AB - While published guidelines are useful in the care of patients with long-QT syndrome, it can be difficult to decide how to apply the guidelines to individual patients, particularly those with intermediate risk. We explored the diversity of opinion among 24 clinicians with expertise in long-QT syndrome. Experts from various regions and institutions were presented with 4 challenging clinical scenarios and asked to provide commentary emphasizing why they would make their treatment recommendations. All 24 authors were asked to vote on case-specific questions so as to demonstrate the degree of consensus or divergence of opinion. Of 24 authors, 23 voted and 1 abstained. Details of voting results with commentary are presented. There was consensus on several key points, particularly on the importance of the diagnostic evaluation and of β-blocker use. There was diversity of opinion about the appropriate use of other therapeutic measures in intermediate-risk individuals. Significant gaps in knowledge were identified.

KW - defibrillators, implantable

KW - genetic testing

KW - knowledge

KW - long-QT syndrome

KW - politics

UR - https://www.scopus.com/pages/publications/85111087303

U2 - 10.1161/CIRCEP.120.009726

DO - 10.1161/CIRCEP.120.009726

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C2 - 34238011

AN - SCOPUS:85111087303

SN - 1941-3149

VL - 14

SP - E009726

JO - Circulation: Arrhythmia and Electrophysiology

JF - Circulation: Arrhythmia and Electrophysiology

IS - 7

ER -

Management of Congenital Long-QT Syndrome: Commentary From the Experts

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