Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy

Jonathan N. Johnson, Camilla Grifoni, J. Martijn Bos, Maha Saber-Ayad, Steve R. Ommen, Stefano Nistri, Franco Cecchi, Iacopo Olivotto, Michael John Ackerman

Research output: Contribution to journalArticle

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Abstract

Aims Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown. Methods and results After exclusion of patients on QT-prolonging drugs, a blinded, retrospective analysis of electrocardiograms, echocardiograms, and genotype status in 479 unrelated patients with HCM [201 females, age at diagnosis 41±18 years, maximal left ventricular wall thickness (MLVWT) 22±6 mm] from two independent centres was performed. The mean QTc was 440±28 ms. The QTc exceeded 480 ms in 13% of patients. Age, gender, family history of HCM or sudden cardiac arrest, and genotype status had no association with QTc. Patients with a QTc over 480 ms were more symptomatic at diagnosis (P < 0.001), had a higher MLVWT (P = 0.03), were more obstructive (P < 0.001), and were more likely to have undergone septal reduction therapy (P = 0.02). There was a weak but significant direct linear relationship between QTc and peak outflow gradient (r2 = 0.05, P < 0.0001). Conclusions Compared with ,1 in 200 otherwise healthy adults, QT prolongation (QTc > 480 ms) was present in 1 out of 8 patients with HCM. The QTc was partly reflective of the degree of cardiac hypertrophy and left ventricular outflow tract obstruction. Because of its pro-arrhythmic potential and its potential relevance to management and risk stratification, routine QTc assessment should be performed in patients with HCM, particularly when concomitant use of QT-prolonging medications is considered.

Original languageEnglish (US)
Pages (from-to)1114-1120
Number of pages7
JournalEuropean Heart Journal
Volume32
Issue number9
DOIs
StatePublished - May 2011

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Hypertrophic Cardiomyopathy
Genotype
Ventricular Outflow Obstruction
Sudden Cardiac Death
Risk Management
Cardiomegaly
Cardiac Arrhythmias
Electrocardiography
Pharmaceutical Preparations

Keywords

  • Hypertrophic cardiomyopathy
  • Long QT syndrome
  • QT interval
  • Sudden death
  • Ventricular arrhythmia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy. / Johnson, Jonathan N.; Grifoni, Camilla; Bos, J. Martijn; Saber-Ayad, Maha; Ommen, Steve R.; Nistri, Stefano; Cecchi, Franco; Olivotto, Iacopo; Ackerman, Michael John.

In: European Heart Journal, Vol. 32, No. 9, 05.2011, p. 1114-1120.

Research output: Contribution to journalArticle

Johnson, JN, Grifoni, C, Bos, JM, Saber-Ayad, M, Ommen, SR, Nistri, S, Cecchi, F, Olivotto, I & Ackerman, MJ 2011, 'Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy', European Heart Journal, vol. 32, no. 9, pp. 1114-1120. https://doi.org/10.1093/eurheartj/ehr021
Johnson, Jonathan N. ; Grifoni, Camilla ; Bos, J. Martijn ; Saber-Ayad, Maha ; Ommen, Steve R. ; Nistri, Stefano ; Cecchi, Franco ; Olivotto, Iacopo ; Ackerman, Michael John. / Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy. In: European Heart Journal. 2011 ; Vol. 32, No. 9. pp. 1114-1120.
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abstract = "Aims Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown. Methods and results After exclusion of patients on QT-prolonging drugs, a blinded, retrospective analysis of electrocardiograms, echocardiograms, and genotype status in 479 unrelated patients with HCM [201 females, age at diagnosis 41±18 years, maximal left ventricular wall thickness (MLVWT) 22±6 mm] from two independent centres was performed. The mean QTc was 440±28 ms. The QTc exceeded 480 ms in 13{\%} of patients. Age, gender, family history of HCM or sudden cardiac arrest, and genotype status had no association with QTc. Patients with a QTc over 480 ms were more symptomatic at diagnosis (P < 0.001), had a higher MLVWT (P = 0.03), were more obstructive (P < 0.001), and were more likely to have undergone septal reduction therapy (P = 0.02). There was a weak but significant direct linear relationship between QTc and peak outflow gradient (r2 = 0.05, P < 0.0001). Conclusions Compared with ,1 in 200 otherwise healthy adults, QT prolongation (QTc > 480 ms) was present in 1 out of 8 patients with HCM. The QTc was partly reflective of the degree of cardiac hypertrophy and left ventricular outflow tract obstruction. Because of its pro-arrhythmic potential and its potential relevance to management and risk stratification, routine QTc assessment should be performed in patients with HCM, particularly when concomitant use of QT-prolonging medications is considered.",
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AU - Johnson, Jonathan N.

AU - Grifoni, Camilla

AU - Bos, J. Martijn

AU - Saber-Ayad, Maha

AU - Ommen, Steve R.

AU - Nistri, Stefano

AU - Cecchi, Franco

AU - Olivotto, Iacopo

AU - Ackerman, Michael John

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N2 - Aims Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown. Methods and results After exclusion of patients on QT-prolonging drugs, a blinded, retrospective analysis of electrocardiograms, echocardiograms, and genotype status in 479 unrelated patients with HCM [201 females, age at diagnosis 41±18 years, maximal left ventricular wall thickness (MLVWT) 22±6 mm] from two independent centres was performed. The mean QTc was 440±28 ms. The QTc exceeded 480 ms in 13% of patients. Age, gender, family history of HCM or sudden cardiac arrest, and genotype status had no association with QTc. Patients with a QTc over 480 ms were more symptomatic at diagnosis (P < 0.001), had a higher MLVWT (P = 0.03), were more obstructive (P < 0.001), and were more likely to have undergone septal reduction therapy (P = 0.02). There was a weak but significant direct linear relationship between QTc and peak outflow gradient (r2 = 0.05, P < 0.0001). Conclusions Compared with ,1 in 200 otherwise healthy adults, QT prolongation (QTc > 480 ms) was present in 1 out of 8 patients with HCM. The QTc was partly reflective of the degree of cardiac hypertrophy and left ventricular outflow tract obstruction. Because of its pro-arrhythmic potential and its potential relevance to management and risk stratification, routine QTc assessment should be performed in patients with HCM, particularly when concomitant use of QT-prolonging medications is considered.

AB - Aims Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown. Methods and results After exclusion of patients on QT-prolonging drugs, a blinded, retrospective analysis of electrocardiograms, echocardiograms, and genotype status in 479 unrelated patients with HCM [201 females, age at diagnosis 41±18 years, maximal left ventricular wall thickness (MLVWT) 22±6 mm] from two independent centres was performed. The mean QTc was 440±28 ms. The QTc exceeded 480 ms in 13% of patients. Age, gender, family history of HCM or sudden cardiac arrest, and genotype status had no association with QTc. Patients with a QTc over 480 ms were more symptomatic at diagnosis (P < 0.001), had a higher MLVWT (P = 0.03), were more obstructive (P < 0.001), and were more likely to have undergone septal reduction therapy (P = 0.02). There was a weak but significant direct linear relationship between QTc and peak outflow gradient (r2 = 0.05, P < 0.0001). Conclusions Compared with ,1 in 200 otherwise healthy adults, QT prolongation (QTc > 480 ms) was present in 1 out of 8 patients with HCM. The QTc was partly reflective of the degree of cardiac hypertrophy and left ventricular outflow tract obstruction. Because of its pro-arrhythmic potential and its potential relevance to management and risk stratification, routine QTc assessment should be performed in patients with HCM, particularly when concomitant use of QT-prolonging medications is considered.

KW - Hypertrophic cardiomyopathy

KW - Long QT syndrome

KW - QT interval

KW - Sudden death

KW - Ventricular arrhythmia

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