CLINICAL STUDY
Differential effects of beta-blockade on dispersion of repolarization in the absence and presence of sympathetic stimulation between the lqt1 and lqt2 forms of congenital long qt syndrome
Wataru Shimizu, MD, PhD*,*,
Yasuko Tanabe, MD*,
Takeshi Aiba, MD, PhD*,
Masashi Inagaki, MD, PhD ,
Takashi Kurita, MD, PhD*,
Kazuhiro Suyama, MD, PhD*,
Noritoshi Nagaya, MD, PhD*,
Atsushi Taguchi, MD*,
Naohiko Aihara, MD*,
Kenji Sunagawa, MD, PhD ,
Kazufumi Nakamura, MD, PhD ,
Tohru Ohe, MD, PhD, FACC ,
Jeffrey A. Towbin, MD ,
Silvia G. Priori, MD, PhD|| and
Shiro Kamakura, MD, PhD*
* Division of Cardiology, Department of Internal MedicineSuita, Osaka, Japan
Department of Cardiovascular Dynamics, National Cardiovascular Center, Suita, Japan
Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan
Department of Pediatrics (Cardiology), Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA
|| Molecular Cardiology, Salvatore Maugeri Foundation, Pavia, Italy
Manuscript received December 6, 2001;
revised manuscript received March 7, 2002,
accepted March 27, 2002.
* Reprint requests and correspondence: Dr. Wataru Shimizu, Division of Cardiology, Department of Internal Medicine, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565 Japan. wshimizu{at}hsp.ncvc.go.jp
OBJECTIVES: This study compared the effects of beta-blockade on transmural and spatial dispersion of repolarization (TDR and SDR, respectively) between the LQT1 and LQT2 forms of congenital long QT syndrome (LQTS).
BACKGROUND: The LQT1 form is more sensitive to sympathetic stimulation and more responsive to beta-blockers than either the LQT2 or LQT3 forms.
METHODS: Eighty-seven-lead, body-surface electrocardiograms (ECGs) were recorded before and after epinephrine infusion (0.1 µg/kg body weight per min) in the absence and presence of oral propranolol (0.52.0 mg/kg per day) in 11 LQT1 patients and 11 LQT2 patients. The Q-Tend interval, the Q-Tpeak interval and the interval between Tpeak and Tend (Tp-e), representing TDR, were measured and averaged from 87-lead ECGs and corrected by Bazetts method (corrected Q-Tend interval [cQTe], corrected Q-Tpeak interval [cQTp] and corrected interval between Tpeak and Tend [cTp-e]). The dispersion of cQTe (cQTe-D) was obtained among 87 leads and was defined as the interval between the maximum and minimum values of cQTe.
RESULTS: Propranolol in the absence of epinephrine significantly prolonged the mean cQTp value but not the mean cQTe value, thus decreasing the mean cTp-e value in both LQT1 and LQT2 patients; the differences with propranolol were significantly larger in LQT1 than in LQT2 (p < 0.05). The maximum cQTe, minimum cQTe and cQTe-D were not changed with propranolol. Propranolol completely suppressed the influence of epinephrine in prolonging the mean cQTe, maximum cQTe and minimum cQTe values, as well as increasing the mean cTp-e and cQTe-D values in both groups.
CONCLUSIONS: Beta-blockade under normal sympathetic tone produces a greater decrease in TDR in the LQT1 form than in the LQT2 form, explaining the superior effectiveness of beta-blockers in LQT1 versus LQT2. Beta-blockers also suppress the influence of sympathetic stimulation in increasing TDR and SDR equally in LQT1 and LQT2 syndrome.
|
Abbreviations and Acronyms
| | APD | | action potential duration | | ECG | | electrocardiogram | | LQTS | | long QT syndrome | | cQTe | | (corrected) Q-Tend interval | | cQTp | | (corrected) Q-Tpeak interval | | cQTe-D | | (corrected) dispersion of QTe | | SDR | | spatial dispersion of repolarization | | cTp-e | | (corrected) interval between Tpeak and Tend | | TDR | | transmural dispersion of repolarization |
|
This article has been cited by other articles:

|
 |

|
 |
 
K. P. Letsas, R. Weber, K. Astheimer, D. Kalusche, and T. Arentz
Tpeak-Tend interval and Tpeak-Tend/QT ratio as markers of ventricular tachycardia inducibility in subjects with Brugada ECG phenotype
Europace,
November 6, 2009;
(2009)
eup357v1.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
H. V. Hume-Smith, S. Sanatani, J. Lim, A. Chau, and S. D. Whyte
The Effect of Propofol Concentration on Dispersion of Myocardial Repolarization in Children
Anesth. Analg.,
September 1, 2008;
107(3):
806 - 810.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. D. Whyte, S. Sanatani, J. Lim, and P. D. Booker
A Comparison of the Effect on Dispersion of Repolarization of Age-Adjusted MAC Values of Sevoflurane in Children
Anesth. Analg.,
February 1, 2007;
104(2):
277 - 282.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. Viitasalo, L. Oikarinen, H. Swan, H. Vaananen, J. Jarvenpaa, H. Hietanen, J. Karjalainen, and L. Toivonen
Effects of Beta-Blocker Therapy on Ventricular Repolarization Documented by 24-h Electrocardiography in Patients With Type 1 Long-QT Syndrome
J. Am. Coll. Cardiol.,
August 15, 2006;
48(4):
747 - 753.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. M. Fish, J. Brugada, and C. Antzelevitch
Potential Proarrhythmic Effects of Biventricular Pacing
J. Am. Coll. Cardiol.,
December 20, 2005;
46(12):
2340 - 2347.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
W. Shimizu
The long QT syndrome: Therapeutic implications of a genetic diagnosis
Cardiovasc Res,
August 15, 2005;
67(3):
347 - 356.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. E. Clancy and R. S. Kass
Inherited and Acquired Vulnerability to Ventricular Arrhythmias: Cardiac Na+ and K+ Channels
Physiol Rev,
January 1, 2005;
85(1):
33 - 47.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. D. Whyte, P. D. Booker, and D. G. Buckley
The Effects of Propofol and Sevoflurane on the QT Interval and Transmural Dispersion of Repolarization in Children
Anesth. Analg.,
January 1, 2005;
100(1):
71 - 77.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
W. Shimizu, M. Horie, S. Ohno, K. Takenaka, M. Yamaguchi, M. Shimizu, T. Washizuka, Y. Aizawa, K. Nakamura, T. Ohe, et al.
Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congenital long QT syndrome: Multicenter study in Japan
J. Am. Coll. Cardiol.,
July 7, 2004;
44(1):
117 - 125.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
W. Shimizu, T. Noda, H. Takaki, T. Kurita, N. Nagaya, K. Satomi, K. Suyama, N. Aihara, S. Kamakura, K. Sunagawa, et al.
Epinephrine unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome
J. Am. Coll. Cardiol.,
February 19, 2003;
41(4):
633 - 642.
[Abstract]
[Full Text]
[PDF]
|
 |
|
|