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

Circ Arrhythm Electrophysiol. 2021 Jul;14(7):e009726. doi: 10.1161/CIRCEP.120.009726. Epub 2021 Jul 9.

Authors

Elizabeth S Kaufman¹, Lee L Eckhardt^#², Michael J Ackerman^{3

4

5}, Peter F Aziz⁶, Elijah R Behr^{7

8}, Marina Cerrone⁹, Mina K Chung¹⁰, Michael J Cutler¹¹, Susan P Etheridge¹², Andrew D Krahn¹³, Steven A Lubitz¹⁴, Marco V Perez¹⁵, Silvia G Priori^{8

16

17}, Jason D Roberts¹⁸, Dan M Roden¹⁹, Eric Schulze-Bahr^{8

20}, Peter J Schwartz^{8

21}, Wataru Shimizu²², M Benjamin Shoemaker²³, Raymond W Sy^{24

25}, Jeffrey A Towbin²⁶, Sami Viskin²⁷, Arthur A M Wilde^{8

28

29}, Wojciech Zareba³⁰

Affiliations

¹ Heart & Vascular Center, MetroHealth Campus, Case Western Reserve University, Cleveland, OH (E.S.K.).
² Cellular and Molecular Arrhythmia Research Program, Division of Cardiovascular Medicine, Department of Medicine, University of Wisconsin, Madison (L.L.E.).
³ Departments of Cardiovascular Medicine (M.J.A.), Mayo Clinic, Rochester, MN.
⁴ Pediatric and Adolescent Medicine (M.J.A.), Mayo Clinic, Rochester, MN.
⁵ Molecular Pharmacology and Experimental Therapeutics (M.J.A.), Mayo Clinic, Rochester, MN.
⁶ Pediatric Electrophysiology and Inherited Arrhythmia Clinic, Cleveland Clinic Children's, OH (P.F.A.).
⁷ Cardiovascular Clinical Academic Group, Institute of Molecular and Clinical Sciences, St George's, University of London, St George's University Hospitals NHS Foundation Trust, United Kingdom (E.R.B.).
⁸ ERN GUARDHEART members of the European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (http://guardheart.ern-net.eu) (E.R.B., S.G.P., E.S.-B., P.J.S., A.A.M.W.).
⁹ Inherited Arrhythmias Clinic, Leon H. Charney Division of Cardiology, New York University Grossman School of Medicine (M.C.).
¹⁰ Heart, Vascular and Thoracic Department, Department of Cardiovascular Medicine, Cleveland Clinic, OH (M.K.C.).
¹¹ Intermountain Medical Center Heart Institute, Intermountain Medical Center, Murray, UT (M.J.C.).
¹² Department of Pediatrics, Division of Pediatric Cardiology, University of Utah, Salt Lake City (S.P.E.).
¹³ Center for Cardiovascular Innovation, Division of Cardiology, University of British Columbia, Vancouver, Canada (A.D.K.).
¹⁴ Cardiac Arrhythmia Service and Cardiovascular Research Center, Massachusetts General Hospital, Boston (S.A.L.).
¹⁵ Stanford Center for Inherited Cardiovascular Diseases, Stanford University, Palo Alto, CA (M.V.P.).
¹⁶ Istituti Clinici Scientifici Maugeri, Pavia, Italy (S.G.P.).
¹⁷ Department of Molecular Medicine, University of Pavia, Italy (S.G.P.).
¹⁸ Population Health Research Institute, McMaster University and Hamilton Health Sciences, Ontario, Canada (J.D.R.).
¹⁹ Senior Vice-President for Personalized Medicine, Vanderbilt University Medical Center, Nashville, TN (D.M.R.).
²⁰ Institute for Genetics of Heart Diseases, University Hospital Münster, Germany (E.S.-B.).
²¹ Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy (P.J.S.).
²² Department of Cardiovascular Medicine, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan (W.S.).
²³ Department of Medicine, Division of Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN (M.B.S.).
²⁴ Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, Australia (R.W.S.).
²⁵ Sydney Medical School, University of Sydney, NSW, Australia (R.W.S.).
²⁶ Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis (J.A.T.).
²⁷ Tel Aviv Sourasky Medical Center and Sackler School of Medicine, Tel Aviv University, Israel (S.V.).
²⁸ Department of Clinical and Experimental Cardiology, University of Amsterdam Heart Center, the Netherlands (A.A.M.W.).
²⁹ Department of Clinical and Experimental Cardiology, Amsterdam, the Netherlands (A.A.M.W.).
³⁰ Clinical Cardiovascular Research Center, University of Rochester Medical Center, NY (W.Z.).

^# Contributed equally.

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.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

MeSH terms

Adrenergic beta-Antagonists / therapeutic use*
Consensus*
Diagnostic Techniques, Cardiovascular*
Disease Management*
Humans
Long QT Syndrome / congenital*
Long QT Syndrome / diagnosis
Long QT Syndrome / surgery

Substances

Adrenergic beta-Antagonists

Abstract

Publication types

MeSH terms

Substances

Grants and funding