QRS Complex Enlargement as a Predictor of Ventricular Arrhythmias in Patients Affected by Surgically Treated Tetralogy of Fallot: A Comprehensive Literature Review and Historical Overview
Tetralogy of Fallot (TOF) is a congenital heart disease frequently treated by surgical repair to relieve symptoms and improve survival. However, despite the performing of an optimal surgical repair, TOF patients are at times characterized by a poor long-term survival rate, likely due to cardiac causes such as ventricular arrhythmias, with subsequent sudden death. In the 80s it was irrefutably demonstrated that QRS prolongation ≥180？msec at basal electrocardiogram is a strong predictor for refining risk stratification for ventricular tachycardia in these patients. The aim of this research was to undertake a review of all studies conducted to assess the impact of QRS duration on the development of life-threatening ventricular arrhythmias in repaired TOF subjects. 1. Introduction Tetralogy of Fallot (TOF) is a congenital heart disease characterized by a large ventricular septal defect, an aorta that overrides the left and right ventricles, obstruction of the right ventricular outflow tract, and right ventricle hypertrophy. Surgical repair of this pathology is recommended to relieve symptoms and to improve survival. However, despite the performing of an optimal surgical repair, TOF patients may feature a poor long-term survival rate, likely due to cardiac causes such as ventricular arrhythmias. The pathogenesis of this kind of tachycardia, thought to account for the majority of cases of sudden unexpected death in operated TOF patients, remains to be fully elucidated [1–3]. Consequently, life-threatening ventricular arrhythmia and sudden death continue to represent serious late complications following TOF repair. Several risk factors have been identified in predicting TOF patients at major risk of sudden death: severity of initial presentation of the disease, preoperative polycythemia, delay in surgical correction of the lesion, elevated left ventricular end-diastolic pressure, initial Blalock-Taussig shunt placement, ventriculotomy, and ventricular arrhythmias [4, 5]. The aim of this work was to undertake a paper of all studies conducted to assess the impact of QRS duration on the development of life-threatening ventricular arrhythmias in repaired TOF subjects. QRS complex corresponds to the depolarization of the right and left ventricles. Duration of the complex may be calculated simply by means of an electrocardiogram, an easy, economical, and noninvasive investigation that is usually readily available in an outpatient setting. This paper should provide help to primary care practitioners in identifying TOF patients at increased risk of developing
References
[1]
M. A. Gatzoulis, J. A. Till, J. Somerville, and A. N. Redington, “Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death,” Circulation, vol. 92, no. 2, pp. 231–237, 1995.
[2]
S. Cullen, D. S. Celermajer, R. C. G. Franklin, K. A. Hallidie-Smith, and J. E. Deanfield, “Prognostic significance of ventricular arrhythmia after repair of tetralogy of Fallot: a 12-year prospective study,” Journal of the American College of Cardiology, vol. 23, no. 5, pp. 1151–1155, 1994.
[3]
D. Giroud, M. Zimmerrnann, R. Adamec, I. Oberhansli, and B. Friedli, “Ventricular late potentials and spontaneous ventricular arrhythmias after surgical repair of tetralogy of Fallot: do they have prognostic value?” British Heart Journal, vol. 72, no. 6, pp. 580–583, 1994.
[4]
R. P. Steeds and D. Oakley, “Predicting late sudden death from ventricular arrythmia in adults following surgical repair of tetralogy of Fallot,” QJM, vol. 97, no. 1, pp. 7–13, 2004.
[5]
G. Nollert, T. Fischlein, S. Bouterwek, C. B？hmer, W. Klinner, and B. Reichart, “Long-term survival in patients with repair of tetralogy of Fallot: 36- year follow-up of 490 survivors of the first year after surgical repair,” Journal of the American College of Cardiology, vol. 30, no. 5, pp. 1374–1384, 1997.
[6]
A. Dunnigan, M. R. Pritzker, D. G. Benditt, and D. W. Benson, “Life threatening ventricular tachycardias in late survivors of surgically corrected tetralogy of Fallot,” British Heart Journal, vol. 52, no. 2, pp. 198–206, 1984.
[7]
M. A. Gatzoulis, S. Balaji, S. A. Webber et al., “Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study,” The Lancet, vol. 356, no. 9234, pp. 975–981, 2000.
[8]
K. Wall, H. Oddsson, B. M. Ternestedt, A. Jonzon, E. Nylander, and J. Schollin, “Thirty-year electrocardiographic follow-up after repair of tetralogy of Fallot or atrial septal defect,” Journal of Electrocardiology, vol. 40, no. 2, pp. 214–217, 2007.
[9]
B. Sarubbi, W. Li, and J. Somerville, “QRS width in right bundle branch block. Accuracy and reproducibility of manual measurement,” International Journal of Cardiology, vol. 75, no. 1, pp. 71–74, 2000.
[10]
M. Y. Abd 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, vol. 84, no. 4, pp. 416–420, 2000.
[11]
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, vol. 103, no. 12, pp. 1669–1673, 2001.
[12]
M. C. Yang, S. N. Chiu, J. K. Wang et al., “Natural and unnatural history of tetralogy of Fallot repaired during adolescence and adulthood,” Heart and Vessels, vol. 27, pp. 65–70, 2012.
[13]
A. L. Knauth, K. Gauvreau, A. J. Powell et al., “Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair,” Heart, vol. 94, no. 2, pp. 211–216, 2008.
[14]
W. A. Helbing, A. A. W. Roest, R. A. Niezen et al., “ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation,” Heart, vol. 88, no. 5, pp. 515–520, 2002.
[15]
N. Tzemos, L. Harris, S. Carasso et al., “Adverse left ventricular mechanics in adults with repaired tetralogy of fallot,” American Journal of Cardiology, vol. 103, no. 3, pp. 420–425, 2009.
[16]
R. W. C. Scherptong, M. G. Hazekamp, B. J. M. Mulder et al., “Follow-up after pulmonary valve replacement in adults with tetralogy of Fallot: association between QRS duration and outcome,” Journal of the American College of Cardiology, vol. 56, no. 18, pp. 1486–1492, 2010.
[17]
E. W. Y. Cheung, W. H. S. Wong, and Y. F. Cheung, “Meta-analysis of pulmonary valve replacement after operative repair of tetralogy of fallot,” American Journal of Cardiology, vol. 106, no. 4, pp. 552–557, 2010.
[18]
D. M. Harrild, C. I. Berul, F. Cecchin et al., “Pulmonary valve replacement in tetralogy of Fallot. Impact on survival and ventricular tachycardia,” Circulation, vol. 119, no. 3, pp. 445–451, 2009.
[19]
B. Hooft van Huysduynen, I. R. Henkens, C. A. Swenne et al., “Pulmonary valve replacement in tetralogy of Fallot improves the repolarization,” International Journal of Cardiology, vol. 124, no. 3, pp. 301–306, 2008.
[20]
B. H. Van Huysduynen, A. Van Straten, C. A. Swenne et al., “Reduction of QRS duration after pulmonary valve replacement in adult Fallot patients is related to reduction of right ventricular volume,” European Heart Journal, vol. 26, no. 9, pp. 928–932, 2005.
[21]
J. Therrien, S. C. Siu, L. Harris et al., “Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot,” Circulation, vol. 103, no. 20, pp. 2489–2494, 2001.
[22]
T. Oosterhof, H. W. Vliegen, F. J. Meijboom, A. H. Zwinderman, B. Bouma, and B. J. M. Mulder, “Long-term effect of pulmonary valve replacement on QRS duration in patients with corrected tetralogy of Fallot,” Heart, vol. 93, no. 4, pp. 506–509, 2007.
[23]
S. V. Babu-Narayan, P. J. Kilner, W. Li et al., “Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome,” Circulation, vol. 113, no. 3, pp. 405–413, 2006.
[24]
J. Therrien, G. R. Marx, and M. A. Gatzoulis, “Late problems in tetralogy of Fallot—recognition, management, and prevention,” Cardiology Clinics, vol. 20, no. 3, pp. 395–404, 2002.
[25]
N. Galea, O. Aquilina, and V. Grech, “Risk factors for QRS prolongation after repaired tetralogy of Fallot,” Hellenic Journal of Cardiology, vol. 47, no. 2, pp. 66–71, 2006.
[26]
K. G. Zahka, P. J. Horneffer, S. A. Rowe et al., “Long-term valvular function after total repair of tetralogy of Fallot. Relation to ventricular arrhythmias,” Circulation, vol. 78, no. 5, pp. III14–III19, 1988.
[27]
A. Uebing, D. G. Gibson, S. V. Babu-Narayan et al., “Right ventricular mechanics and QRS duration in patients with repaired tetralogy of Fallot: implications of infundibular disease,” Circulation, vol. 116, no. 14, pp. 1532–1539, 2007.
[28]
E. A. Stephenson, F. Cecchin, M. E. Alexander, J. K. Triedman, E. P. Walsh, and C. I. Berul, “Relation of right ventricular pacing in tetralogy of Fallot to electrical resynchronization,” American Journal of Cardiology, vol. 93, no. 11, pp. 1449–1452, 2004.
[29]
R. E. Omeroglu, S. Olgar, and K. Nisli, “Signal-averaged electrocardiogram may be a beneficial prognostic procedure in the postoperative follow-up tetralogy of fallot patients to determine the risk of ventricular arrhythmias,” Pediatric Cardiology, vol. 28, no. 3, pp. 208–212, 2007.
[30]
J. K. Perloff, H. R. Middlekauf, J. S. Child, W. G. Stevenson, P. D. Miner, and G. D. Goldberg, “Usefulness of post-ventriculotomy signal averaged electrocardiograms in congenital heart disease,” American Journal of Cardiology, vol. 98, no. 12, pp. 1646–1651, 2006.
[31]
A. F. Folino and L. Daliento, “Arrhythmias after tetralogy of Fallot repair,” Indian Pacing and Electrophysiology Journal, vol. 5, no. 4, pp. 312–324, 2005.
[32]
G. Russo, A. F. Folino, E. Mazzotti, L. Rebellato, and L. Daliento, “Comparison between QRS duration at standard ECG and signal-averaging ECG for arrhythmic risk stratification after surgical repair of tetralogy of Fallot,” Journal of Cardiovascular Electrophysiology, vol. 16, no. 3, pp. 288–292, 2005.
[33]
S. N. Chiu, H. H. Chiu, J. K. Wang, et al., “Increased microvolt T-wave alternans in patients with repaired tetralogy of Fallot,” International Journal of Cardiology, vol. 159, pp. 220–224, 2012.
[34]
M. A. Gatzoulis, J. A. Till, and A. N. Redington, “Depolarization-repolarization inhomogeneity after repair of tetralogy of Fallot: the substrate for malignant ventricular tachycardia?” Circulation, vol. 95, no. 2, pp. 401–404, 1997.
[35]
C. I. Berul, S. L. Hill, R. L. Geggel et al., “Electrocardiographic markers of late sudden death risk in postoperative tetralogy of fallot children,” Journal of Cardiovascular Electrophysiology, vol. 8, no. 12, pp. 1349–1356, 1997.
[36]
J. van den Berg, S. de Bie, F. J. Meijboom et al., “Changes during exercise of ECG intervals related to increased risk for ventricular arrhythmia in repaired tetralogy of Fallot and their relationship to right ventricular size and function,” International Journal of Cardiology, vol. 124, no. 3, pp. 332–338, 2008.
[37]
B. Sarubbi, G. Pacileo, V. Ducceschi et al., “Arrhythmogenic substrate in young patients with repaired tetralogy of Fallot: role of an abnormal ventricular repolarization,” International Journal of Cardiology, vol. 72, no. 1, pp. 73–82, 1999.
[38]
S. J. Park, Y. K. On, J. S. Kim, et al., “Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of fallot,” The American Journal of Cardiology, vol. 109, pp. 110–115, 2012.
[39]
W. Budts, J. Defoor, A. Stevens, M. V. Wyngaerd, P. Moons, and L. Vanhees, “Changes in QRS duration are associated with maximal exercise capacity in adult patients with repaired tetralogy of Fallot,” International Journal of Cardiology, vol. 104, no. 1, pp. 46–51, 2005.
[40]
S. L. Roche, L. Grosse-Wortmann, A. N. Redington et al., “Exercise induces biventricular mechanical dyssynchrony in children with repaired tetralogy of Fallot,” Heart, vol. 96, no. 24, pp. 2010–2015, 2010.
[41]
S. Balaji, Y. R. Lau, C. L. Case, and P. C. Gillette, “QRS prolongation is associated with inducible ventricular tachycardia after repair of tetralogy of Fallot,” American Journal of Cardiology, vol. 80, no. 2, pp. 160–163, 1997.
[42]
L. Daliento, A. F. Folino, L. Menti, P. Zanco, M. C. Baratella, and S. Dalla Volta, “Adrenergic nervous activity in patients after surgical correction of tetralogy of fallot,” Journal of the American College of Cardiology, vol. 38, no. 7, pp. 2043–2047, 2001.
[43]
C. H. Davos, P. A. Davlouros, R. Wensel et al., “Global impairment of cardiac autonomic nervous activity late after repair of Tetralogy of Fallot,” Circulation, vol. 106, no. 13, pp. I69–I75, 2002.
[44]
M. Nakazawa, T. Shinohara, A. Sasaki et al., “Arrhythmias late after repair of tetralogy of Fallot: a Japanese multicenter study,” Circulation Journal, vol. 68, no. 2, pp. 126–130, 2004.
[45]
J. K. Papagiannis, “Postoperative arrhythmias in tetralogy of Fallot,” Hellenic Journal of Cardiology, vol. 46, no. 6, pp. 402–407, 2005.
[46]
A. Dore, P. Santagata, M. Dubuc, and L. A. Mercier, “Implantable cardioverter defibrillators in adults with congenital heart disease: a single center experience,” Pacing and Clinical Electrophysiology, vol. 27, no. 1, pp. 47–51, 2004.
[47]
L. Le Gloan and P. Khairy, “Management of arrhythmias in patients with tetralogy of Fallot,” Current Opinion in Cardiology, vol. 26, no. 1, pp. 60–65, 2011.
[48]
V. L. Vettel, “Postoperative arrhythmias after surgery for congenital heart defects,” Cardiology in Review, vol. 2, pp. 83–97, 1994.
[49]
M. M. Massin, S. G. Malekzadeh-Milani, S. Schifflers, et al., “Long-term electrocardiographic follow-up after repair of tetralogy of Fallot,” Annals of Noninvasive Electrocardiology, vol. 16, pp. 336–343, 2011.
[50]
S. C. Menon, A. K. Kaza, and M. D. Puchalski, “Effect of ventricular size and function on exercise performance and the electrocardiogram in repaired tetralogy of Fallot with pure pulmonary regurgitation,” Annals of Pediatric Cardiology, vol. 5, pp. 151–155, 2012.
[51]
P. P. Bassareo, “Sports practice in congenital heart diseases,” in Sports Cardiology, M. Fioranelli and G. Frajese, Eds., pp. 273–289, Springer, Milan, Italy, 2012.
