A Congenital Deadly Association: Dilated Cardiomyopathy and Long QT Syndrome

  • Neiberg de Alcantara Lima Western Michigan University – Homer Stryker M.D. School of Medicine – Department of Internal Medicine – Kalamazoo/MI – USA.
  • Antonio Thomaz de Andrade Hospital de Messejana – Fortaleza/CE – Brazil.
  • Stela M V Sampaio Hospital de Messejana – Fortaleza/CE – Brazil.
  • Mark Loehrke Western Michigan University Homer Stryker – School of Medicine – Internal Medicine – Kalamazoo (MI), USA.
Keywords: Long QT Syndrome, Cardiomyopathies, Genetic Diseases, Ventricular Tachycardia, Channelopathies

Abstract

Long QT syndrome is one of the most feared entities in hospitalized patients due to the potential risk for ventricular tachycardia and sudden death. Association between channelopathies and congenital cardiomyopathy is a new entity that has been studied recently. We report an interesting case of this association that maybe related to a genetic mutation.

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References

El‐Sherif N, Turitto G, Boutjdir M. Acquired long QT syndrome and torsade de pointes. Pacing and Clinical Electrophysiol 2018;41(4):414-21.

Ogawa Y, Tanaka T, Kido S. Maximum QT c on Holter electrocardiography in children. Pediatr Int 2018;60(6):507-12.

Robyns T, Willems R, Vandenberk B, Ector J, Garweg C, Kuiperi C, Breckpot J, Corveleyn A, Janssens S, Heidbuchel H, Nuyens D. Individualized corrected QT interval is superior to QT interval corrected using the Bazett formula in predicting mutation carriage in families with long QT syndrome. Heart rhythm 2017;14(3):376-82.

Schwartz PJ, Crotti L. QTc behavior during exercise and genetic testing for the long-QT syndrome. Circulation 2011;124:2181-84.

Horner JM, Horner MM, Ackerman MJ. The diagnostic utility of recovery phase QTc during treadmill exercise stress testing in the evaluation of long QT syndrome. Heart Rhythm 2011;8(11):1698-704.

Wilde AA, Amin AS. Clinical spectrum of SCN5A mutations: long QT syndrome, Brugada syndrome, and cardiomyopathy. JACC Clin Electrophysiol 2018;4(5):569-79.

Asatryan B. Cardiac Sodium Channel Dysfunction and Dilated Cardiomyopathy: A Contemporary Reappraisal of Pathophysiological Concepts. J clin med 2019;8(7):1029.

Moreau A, Gosselin-Badaroudine P, Boutjdir M, Chahine M. Mutations in the voltage sensors of domains I and II of Nav1. 5 that are associated with arrhythmias and dilated cardiomyopathy generate gating pore currents. Front pharmacol 2015;6:301.

Amin AS. SCN5A-related dilated cardiomyopathy: what do we know? Heart rhythm 2014;11(8):1454-5.

Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. JACC 2018;72(14):91-220.

Published
24-10-2020
How to Cite
Lima, N. de A., Andrade, A. T. de, Sampaio, S. M. V., & Loehrke, M. (2020). A Congenital Deadly Association: Dilated Cardiomyopathy and Long QT Syndrome. JOURNAL OF CARDIAC ARRHYTHMIAS, 33(4). Retrieved from https://jca.org.br/jca/article/view/3426
Section
Clinical Arrythmia