Beyond the length and look of repolarization: Defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome

Conor M. Lane, J. Martijn Bos, Ram K. Rohatgi, Michael John Ackerman

Research output: Contribution to journalArticle

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Abstract

Background: Little is known about the spectrum and prevalence of ECG features beyond the length and morphology of repolarization in patients with congenital long QT syndrome (LQTS). Objective: The purpose of this study was to characterize the full ECG phenotype of LQTS patients and evaluate differences by age and LQTS genotype. Methods: Retrospective review of 943 patients with LQTS (57% female; median age 25 years; interquartile range 9–34 years) was performed. Comprehensive analysis of their initial evaluation ECG was performed using definitions outlined in professional guidelines. Results: Bradycardia was common (n = 320 [34%]), regardless of beta-blocker use. Left-axis deviation (n = 33 [3.5%]) and bundle branch block (n = 5 [0.5%]) were uncommon. T-wave inversion (TWI) involving leads V1 and V3 was more common in LQTS type 2 compared to LQTS type 1 or type 3 (odds ratio [OR] for V1: 2.67, 95% confidence interval [CI] 1.8–3.9; OR for V3: 1.76, 95% CI 1.2–2.6), whereas TWI in leads III and aVF was most common in LQTS type 3 (OR for III: 2.38, 95% CI 1.4–4.2; OR for aVF: 3.14, 95% CI 1.6–6.4). Notched T waves were most apparent at younger ages (48% in patients age 4–10 compared to 12% in patients age >40: P <.0001). Conclusion: Beyond the QT interval and bradycardia, ECG abnormalities are uncommon in LQTS patients, and patients almost never have concomitant bundle branch block. Notably, 19% of LQTS patients overall and 27% of LQTS type 2 patients exhibit anterior TWI that would satisfy a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy, thus creating the potential for diagnostic miscues.

Original languageEnglish (US)
JournalHeart Rhythm
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Long QT Syndrome
Electrocardiography
Odds Ratio
Confidence Intervals
Bundle-Branch Block
Bradycardia
Romano-Ward Syndrome
Arrhythmogenic Right Ventricular Dysplasia
Genotype
Guidelines
Phenotype

Keywords

  • Arrhythmia
  • Electrocardiography
  • Long QT syndrome
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Beyond the length and look of repolarization : Defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome. / Lane, Conor M.; Bos, J. Martijn; Rohatgi, Ram K.; Ackerman, Michael John.

In: Heart Rhythm, 01.01.2018.

Research output: Contribution to journalArticle

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title = "Beyond the length and look of repolarization: Defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome",
abstract = "Background: Little is known about the spectrum and prevalence of ECG features beyond the length and morphology of repolarization in patients with congenital long QT syndrome (LQTS). Objective: The purpose of this study was to characterize the full ECG phenotype of LQTS patients and evaluate differences by age and LQTS genotype. Methods: Retrospective review of 943 patients with LQTS (57{\%} female; median age 25 years; interquartile range 9–34 years) was performed. Comprehensive analysis of their initial evaluation ECG was performed using definitions outlined in professional guidelines. Results: Bradycardia was common (n = 320 [34{\%}]), regardless of beta-blocker use. Left-axis deviation (n = 33 [3.5{\%}]) and bundle branch block (n = 5 [0.5{\%}]) were uncommon. T-wave inversion (TWI) involving leads V1 and V3 was more common in LQTS type 2 compared to LQTS type 1 or type 3 (odds ratio [OR] for V1: 2.67, 95{\%} confidence interval [CI] 1.8–3.9; OR for V3: 1.76, 95{\%} CI 1.2–2.6), whereas TWI in leads III and aVF was most common in LQTS type 3 (OR for III: 2.38, 95{\%} CI 1.4–4.2; OR for aVF: 3.14, 95{\%} CI 1.6–6.4). Notched T waves were most apparent at younger ages (48{\%} in patients age 4–10 compared to 12{\%} in patients age >40: P <.0001). Conclusion: Beyond the QT interval and bradycardia, ECG abnormalities are uncommon in LQTS patients, and patients almost never have concomitant bundle branch block. Notably, 19{\%} of LQTS patients overall and 27{\%} of LQTS type 2 patients exhibit anterior TWI that would satisfy a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy, thus creating the potential for diagnostic miscues.",
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N2 - Background: Little is known about the spectrum and prevalence of ECG features beyond the length and morphology of repolarization in patients with congenital long QT syndrome (LQTS). Objective: The purpose of this study was to characterize the full ECG phenotype of LQTS patients and evaluate differences by age and LQTS genotype. Methods: Retrospective review of 943 patients with LQTS (57% female; median age 25 years; interquartile range 9–34 years) was performed. Comprehensive analysis of their initial evaluation ECG was performed using definitions outlined in professional guidelines. Results: Bradycardia was common (n = 320 [34%]), regardless of beta-blocker use. Left-axis deviation (n = 33 [3.5%]) and bundle branch block (n = 5 [0.5%]) were uncommon. T-wave inversion (TWI) involving leads V1 and V3 was more common in LQTS type 2 compared to LQTS type 1 or type 3 (odds ratio [OR] for V1: 2.67, 95% confidence interval [CI] 1.8–3.9; OR for V3: 1.76, 95% CI 1.2–2.6), whereas TWI in leads III and aVF was most common in LQTS type 3 (OR for III: 2.38, 95% CI 1.4–4.2; OR for aVF: 3.14, 95% CI 1.6–6.4). Notched T waves were most apparent at younger ages (48% in patients age 4–10 compared to 12% in patients age >40: P <.0001). Conclusion: Beyond the QT interval and bradycardia, ECG abnormalities are uncommon in LQTS patients, and patients almost never have concomitant bundle branch block. Notably, 19% of LQTS patients overall and 27% of LQTS type 2 patients exhibit anterior TWI that would satisfy a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy, thus creating the potential for diagnostic miscues.

AB - Background: Little is known about the spectrum and prevalence of ECG features beyond the length and morphology of repolarization in patients with congenital long QT syndrome (LQTS). Objective: The purpose of this study was to characterize the full ECG phenotype of LQTS patients and evaluate differences by age and LQTS genotype. Methods: Retrospective review of 943 patients with LQTS (57% female; median age 25 years; interquartile range 9–34 years) was performed. Comprehensive analysis of their initial evaluation ECG was performed using definitions outlined in professional guidelines. Results: Bradycardia was common (n = 320 [34%]), regardless of beta-blocker use. Left-axis deviation (n = 33 [3.5%]) and bundle branch block (n = 5 [0.5%]) were uncommon. T-wave inversion (TWI) involving leads V1 and V3 was more common in LQTS type 2 compared to LQTS type 1 or type 3 (odds ratio [OR] for V1: 2.67, 95% confidence interval [CI] 1.8–3.9; OR for V3: 1.76, 95% CI 1.2–2.6), whereas TWI in leads III and aVF was most common in LQTS type 3 (OR for III: 2.38, 95% CI 1.4–4.2; OR for aVF: 3.14, 95% CI 1.6–6.4). Notched T waves were most apparent at younger ages (48% in patients age 4–10 compared to 12% in patients age >40: P <.0001). Conclusion: Beyond the QT interval and bradycardia, ECG abnormalities are uncommon in LQTS patients, and patients almost never have concomitant bundle branch block. Notably, 19% of LQTS patients overall and 27% of LQTS type 2 patients exhibit anterior TWI that would satisfy a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy, thus creating the potential for diagnostic miscues.

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KW - Sudden cardiac death

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