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

^*Corresponding author for this work

Cardiology

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

14 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	R01HL128598

Keywords

defibrillators, implantable
genetic testing
knowledge
long-QT syndrome
politics

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10.1161/CIRCEP.120.009726

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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

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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,

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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

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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

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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.

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AN - SCOPUS:85111087303

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ER -

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

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