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(Circulation. 1995;92:231-237.)
© 1995 American Heart Association, Inc.


Articles

Mechanoelectrical Interaction in Tetralogy of Fallot

QRS Prolongation Relates to Right Ventricular Size and Predicts Malignant Ventricular Arrhythmias and Sudden Death

Michael A. Gatzoulis, MD; Jan A. Till, MD; Jane Somerville, MD, FRCP; Andrew N. Redington, MD, FRCP

From the Department of Paediatric Cardiology (M.A.G., J.A.T., A.N.R.), Grown-up Congenital Heart Unit (J.S.), Royal Brompton Hospital and National Heart and Lung Institute, London, England.

Correspondence to Dr Andrew N. Redington, Department of Paediatric Cardiology, Royal Brompton Hospital/National Heart and Lung Institute, Sydney St, London, UK, SW3 6NP.

Background Life-threatening ventricular arrhythmia and sudden death remain serious late complications after tetralogy of Fallot repair. Nevertheless, there remains no clear way of predicting which patients are at risk.

Methods and Results The study population included a total of 178 adult survivors (mean follow-up, 21.4 years) of tetralogy of Fallot repair who were currently attending our clinic. Mechanoelectrical relations were sought in 41 of the patients (mean follow-up, 23.6 years) who were operated on by one surgeon and who were prospectively studied with a 12-lead ECG, chest radiography, and two-dimensional and Doppler echocardiography. Nine patients (mean follow-up, 17 years) from the total group of 178 were identified as having had sustained ventricular tachycardia (8 with near-miss sudden death), and their ECGs, Holter monitor readings, electrophysiological studies, and chest radiographs were reviewed. The case notes of an additional 4 patients with postoperative sudden cardiac death also were available for review. QRS duration in the 41 patients in whom mechanoelectrical interaction was sought ranged between 90 and 200 milliseconds and correlated with cardiothoracic ratio (CTR) on chest radiography (r=.64, P<.001) and with right ventricular size on echocardiography (r=.43, P<.02). Twenty of the 41 patients had restrictive right ventricular Doppler physiology (reduced ventricular compliance) with mean QRS duration of 129.3±20 milliseconds and mean CTR of 0.51±0.03. The remaining 21 patients with no evidence of right ventricular restriction had prolonged QRS duration of 157.5±13.2 milliseconds (P<.001) and CTR of 0.55±0.04 (P<.04) compared with the restrictive. In the 9 patients with ventricular tachycardia, the QRS duration ranged from 180 to 230 milliseconds (mean, 198.9±17.6 milliseconds), and the CTR ranged from 0.54 to 0.9 (mean, 0.67±0.12) (P<.0001 and P<.01, respectively, compared with patients without life-threatening arrhythmias). All patients with documented sustained ventricular tachycardia and the 4 patients with sudden death had a QRS duration of >=180 milliseconds (100% sensitivity).

Conclusions Chronic right ventricular volume overload after tetralogy of Fallot repair is related to diastolic function and correlates with QRS prolongation. The risk of symptomatic arrhythmia is high when marked right ventricular enlargement and QRS prolongation develop. A QRS duration on the resting ECG of >=180 milliseconds is the most sensitive predictor of life-threatening ventricular arrhythmias yet described.


Key Words: tetralogy of Fallot • arrhythmia • death • sudden • ventricles




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The Task Force on the Management of Grown Up Conge, Task Force members, J. Deanfield, E. Thaulow, C. Warnes, G. Webb, F. Kolbel, A. Hoffman, K. Sorenson, H. Kaemmerer, et al.
Management of Grown Up Congenital Heart Disease
Eur. Heart J., June 1, 2003; 24(11): 1035 - 1084.
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Ann. Thorac. Surg.Home page
F. J. Zimmerman, J. P. Starr, P. R. Koenig, P. Smith, Z. M. Hijazi, and E. A. Bacha
Acute hemodynamic benefit of multisite ventricular pacing after congenital heart surgery
Ann. Thorac. Surg., June 1, 2003; 75(6): 1775 - 1780.
[Abstract] [Full Text] [PDF]


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HeartHome page
W A Helbing, A A W Roest, R A Niezen, H W Vliegen, M G Hazekamp, J Ottenkamp, A de Roos, and E E van der Wall
ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation
Heart, December 1, 2002; 88(5): 515 - 519.
[Abstract] [Full Text] [PDF]


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J Am Coll CardiolHome page
A. Ghai, C. Silversides, L. Harris, G. D. Webb, S. C. Siu, and J. Therrien
Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of fallot
J. Am. Coll. Cardiol., November 6, 2002; 40(9): 1675 - 1680.
[Abstract] [Full Text] [PDF]


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CirculationHome page
H. W. Vliegen, A. van Straten, A. de Roos, A. A.W. Roest, P. H. Schoof, A. H. Zwinderman, J. Ottenkamp, E. E. van der Wall, and M. G. Hazekamp
Magnetic Resonance Imaging to Assess the Hemodynamic Effects of Pulmonary Valve Replacement in Adults Late After Repair of Tetralogy of Fallot
Circulation, September 24, 2002; 106(13): 1703 - 1707.
[Abstract] [Full Text] [PDF]


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CirculationHome page
C. H. Davos, P. A. Davlouros, R. Wensel, D. Francis, L. C. Davies, P. J. Kilner, A. J. S. Coats, M. Piepoli, and M. A. Gatzoulis
Global Impairment of Cardiac Autonomic Nervous Activity Late After Repair of Tetralogy of Fallot
Circulation, September 24, 2002; 106(12_suppl_1): I-69 - I-75.
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HeartHome page
J G J Neffke, I I Tulevski, E E van der Wall, A A M Wilde, D J van Veldhuisen, A Dodge-Khatami, and B J M Mulder
ECG determinants in adult patients with chronic right ventricular pressure overload caused by congenital heart disease: relation with plasma neurohormones and MRI parameters
Heart, September 1, 2002; 88(3): 266 - 270.
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Eur. J. Cardiothorac. Surg.Home page
C. Alexiou, Q. Chen, M. Galogavrou, J. Gnanapragasam, A. P. Salmon, B. R. Keeton, M. P. Haw, and J. L. Monro
Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach
Eur. J. Cardiothorac. Surg., August 1, 2002; 22(2): 174 - 183.
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Ann. Thorac. Surg.Home page
F. T.H. de Ruijter, I. Weenink, F. J. Hitchcock, E. J. Meijboom, and G. B.W.E. Bennink
Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of fallot
Ann. Thorac. Surg., June 1, 2002; 73(6): 1794 - 1800.
[Abstract] [Full Text] [PDF]


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J. Thorac. Cardiovasc. Surg.Home page
A. Dodge-Khatami, O. I. Miller, R. H. Anderson, A. P. Goldman, J. M. Gil-Jaurena, M. J. Elliott, V. T. Tsang, and M. R. de Leval
Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects
J. Thorac. Cardiovasc. Surg., April 1, 2002; 123(4): 624 - 630.
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CirculationHome page
P. Turrini, D. Corrado, C. Basso, A. Nava, B. Bauce, and G. Thiene
Dispersion of Ventricular Depolarization-Repolarization : A Noninvasive Marker for Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy
Circulation, June 26, 2001; 103(25): 3075 - 3080.
[Abstract] [Full Text] [PDF]


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J. Thorac. Cardiovasc. Surg.Home page
A. R. Hosseinpour, R. H. Anderson, and S. Y. Ho
The anatomy of the septal perforating arteries in normal and congenitally malformed hearts
J. Thorac. Cardiovasc. Surg., June 1, 2001; 121(6): 1046 - 1052.
[Abstract] [Full Text] [PDF]


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CirculationHome page
J. Therrien, S. C. Siu, L. Harris, A. Dore, K. Niwa, J. Janousek, W. G. Williams, G. Webb, and M. A. Gatzoulis
Impact of Pulmonary Valve Replacement on Arrhythmia Propensity Late After Repair of Tetralogy of Fallot
Circulation, May 22, 2001; 103(20): 2489 - 2494.
[Abstract] [Full Text] [PDF]


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Eur. J. Cardiothorac. Surg.Home page
M.G. Hazekamp, M.M.J. Kurvers, P.H. Schoof, H.W. Vliegen, B.M. Mulder, A.A.W. Roest, J. Ottenkamp, and R.A.E. Dion
Pulmonary valve insertion late after repair of Fallot's tetralogy
Eur. J. Cardiothorac. Surg., May 1, 2001; 19(5): 667 - 670.
[Abstract] [Full Text] [PDF]


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Cardiovasc ResHome page
D. Babuty and M. J Lab
Mechanoelectric contributions to sudden cardiac death
Cardiovasc Res, May 1, 2001; 50(2): 270 - 279.
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CirculationHome page
M. Vogel, J. Sponring, S. Cullen, J. E. Deanfield, and A. N. Redington
Regional Wall Motion and Abnormalities of Electrical Depolarization and Repolarization in Patients After Surgical Repair of Tetralogy of Fallot
Circulation, March 27, 2001; 103(12): 1669 - 1673.
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Ann. Thorac. Surg.Home page
C. Alexiou, H. Mahmoud, A. Al-Khaddour, J. Gnanapragasam, A. P. Salmon, B. R. Keeton, and J. L. Monro
Outcome after repair of tetralogy of Fallot in the first year of life
Ann. Thorac. Surg., February 1, 2001; 71(2): 494 - 500.
[Abstract] [Full Text] [PDF]


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J. Thorac. Cardiovasc. Surg.Home page
B. Discigil, J. A. Dearani, F. J. Puga, H. V. Schaff, D. J. Hagler, C. A. Warnes, and G. K. Danielson
Late pulmonary valve replacement after repair of tetralogy of Fallot
J. Thorac. Cardiovasc. Surg., February 1, 2001; 121(2): 0344 - 351.
[Abstract] [Full Text] [PDF]


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HeartHome page
S Brili, C Aggeli, K Gatzoulis, A Tzonou, C Hatzos, C Pitsavos, C Stefanadis, and P Toutouzas
Echocardiographic and signal averaged ECG indices associated with non-sustained ventricular tachycardia after repair of tetralogy of Fallot
Heart, January 1, 2001; 85(1): 57 - 60.
[Abstract] [Full Text]


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J Am Coll CardiolHome page
J. Therrien, S. C. Siu, P. R. McLaughlin, P. P. Liu, W. G. Williams, and G. D. Webb
Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late?
J. Am. Coll. Cardiol., November 1, 2000; 36(5): 1670 - 1675.
[Abstract] [Full Text] [PDF]


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HeartHome page
M A Gatzoulis, J Walters, P R McLaughlin, N Merchant, G D Webb, and P Liu
Late arrhythmia in adults with the Mustard procedure for transposition of great arteries: a surrogate marker for right ventricular dysfunction?
Heart, October 1, 2000; 84(4): 409 - 415.
[Abstract] [Full Text]


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HeartHome page
M Y A. El Rahman, H Abdul-Khaliq, M Vogel, V Alexi-Meskishvili, M Gutberlet, and P E Lange
Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair
Heart, October 1, 2000; 84(4): 416 - 420.
[Abstract] [Full Text]


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NEJMHome page
M. E. Brickner, L. D. Hillis, and R. A. Lange
Congenital Heart Disease in Adults- Second of Two Parts
N. Engl. J. Med., February 3, 2000; 342(5): 334 - 342.
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Ann. Thorac. Surg.Home page
V. Rao, M. Kadletz, L. K. Hornberger, R. M. Freedom, and M. D. Black
Preservation of the pulmonary valve complex in tetralogy of Fallot: how small is too small?
Ann. Thorac. Surg., January 1, 2000; 69(1): 176 - 179.
[Abstract] [Full Text] [PDF]


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CirculationHome page
R. R. Chaturvedi, D. F. Shore, C. Lincoln, S. Mumby, M. Kemp, J. Brierly, A. Petros, J. M.G. Gutteridge, J. Hooper, and A. N. Redington
Acute Right Ventricular Restrictive Physiology After Repair of Tetralogy of Fallot : Association With Myocardial Injury and Oxidative Stress
Circulation, October 5, 1999; 100(14): 1540 - 1547.
[Abstract] [Full Text] [PDF]


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Eur. J. Cardiothorac. Surg.Home page
M. A. Norgaard, P. Lauridsen, M. Helvind, and G. Pettersson
Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot
Eur. J. Cardiothorac. Surg., August 1, 1999; 16(2): 125 - 130.
[Abstract] [Full Text] [PDF]


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J. Thorac. Cardiovasc. Surg.Home page
E. N. Oechslin, D. A. Harrison, L. Harris, E. Downar, G. D. Webb, S. S. Siu, and W. G. Williams
REOPERATION IN ADULTS WITH REPAIR OF TETRALOGY OF FALLOT: INDICATIONS AND OUTCOMES
J. Thorac. Cardiovasc. Surg., August 1, 1999; 118(2): 245 - 251.
[Abstract] [Full Text] [PDF]


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Cardiovasc ResHome page
P. Schaffer, B. Pelzmann, E. Bernhart, P. Lang, H. Machler, B. Rigler, and B. Koidl
Repolarizing currents in ventricular myocytes from young patients with tetralogy of Fallot
Cardiovasc Res, August 1, 1999; 43(2): 332 - 343.
[Abstract] [Full Text] [PDF]


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HeartHome page
L Daliento, G Rizzoli, L Menti, M C Baratella, P Turrini, A Nava, and S Dalla Volta
Accuracy of electrocardiographic and echocardiographic indices in predicting life threatening ventricular arrhythmias in patients operated for tetralogy of Fallot
Heart, June 1, 1999; 81(6): 650 - 655.
[Abstract] [Full Text]


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HeartHome page
K A McLeod, W S Hillis, A B Houston, N Wilson, A Trainer, J Neilson, and W B Doig
Reduced heart rate variability following repair of tetralogy of Fallot
Heart, June 1, 1999; 81(6): 656 - 660.
[Abstract] [Full Text]


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Eur. J. Cardiothorac. Surg.Home page
S. Conte, R. Jashari, B. Eyskens, M. Gewillig, M. Dumoulin, and W. Daenen
Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction
Eur. J. Cardiothorac. Surg., February 1, 1999; 15(2): 143 - 149.
[Abstract] [Full Text] [PDF]


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Ann. Thorac. Surg.Home page
S. B. O'Blenes, D. B. Ross, M. A. Nanton, and D. A. Murphy
Atrioventricular septal defect with tetralogy of fallot: results of surgical correction
Ann. Thorac. Surg., December 1, 1998; 66(6): 2078 - 2082.
[Abstract] [Full Text] [PDF]


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CirculationHome page
D. P. Zipes and H. J. J. Wellens
Sudden Cardiac Death
Circulation, November 24, 1998; 98(21): 2334 - 2351.
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