Abstract
Background: Right ventricular dysfunction occurs in many patients with significant pulmonary valve regurgitation late after initial total repair of tetralogy of Fallot. Methods to predict which of these patients are at increased risk of late morbidity and mortality are not yet known.
Hypothesis: This study evaluated electrocardiographic (ECG) predictors of severe right ventricular dilatation determined by magnetic resonance imaging (MRI) volumes in patients with tetralogy of Fallot late after initial corrective repair.
Method: We retrospectively reviewed the ECGs and MRI right ventricular volume measurements of 20 patients (age 4.4 to 19.3 years, mean 10.0 years) with significant pulmonary valve regurgitation late after repair of tetralogy of Fallot. All patients had enlarged, hypokinetic right ventricles by echocardiography. The patients were grouped based on an indexed right ventricular end‐diastolic volume (RVEDV/BSA) of <102 ml/m2 (Group 1) or ≥ 102 ml/m2 (Group 2). We determined the sensitivity, specificity, positive and negative predictive values of QRS duration, and mean frontal plane QRS axis for predicting right ventricular volumes.
Results: A maximal QRS duration of ≥ 150 ms or a northwest quadrant frontal plane QRS axis had 85% sensitivity, 86% specificity, 92% positive predictive value, and 75% negative predictive value for predicting an RVEDV/BSA of ≥ 102 ml/m2. The mean QRS duration was significantly longer in Group 2 than in Group 1 patients (156 ms vs. 123 ms, p=0.005).
Conclusions: In patients late after repair of tetralogy of Fallot with significant pulmonary valve regurgitation, a maximal manually measured QRS duration of ≥ 150 ms and/or a frontal plane QRS northwest quadrant axis can predict patients with marked right ventricular enlargement. The presence of either of these findings on the ECG signifies patients who require further evaluation and consideration for pulmonary valve replacement.
Keywords: congenital heart disease, right bundle‐branch block, right ventricle, ventricular dysfunction
Full Text
The Full Text of this article is available as a PDF (865.4 KB).
References
- 1. Lillehei CW, Morley C, Warden HE, Read RC, Aust JB, DeWall RA, Varco RL: Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects. Ann Surg 1955; 142 (3): 418–445 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2. Horneffer PJ, Zahka KG, Rowe SA, Manolio TA, Gott VL, Reitz BA, Gardner TJ: Long‐term results of total repair of tetralogy of Fallot in childhood. Ann Thorac Surg 1990; 50: 179–183 [DOI] [PubMed] [Google Scholar]
- 3. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK: Long‐term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993; 329: 593–599 [DOI] [PubMed] [Google Scholar]
- 4. Walsh EP, Rockenmacher S, Keane JF, Hougen TJ, Lock JE, Castaneda AR: Late results in patients with tetralogy of Fallot repaired during infancy. Circulation 1988; 77: 1062–1067 [DOI] [PubMed] [Google Scholar]
- 5. Fuster V, McGoon DC, Kennedy MA, Ritter DG, Kirklin JW: Long‐term evaluation (12 to 22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 1980; 46: 635–642 [DOI] [PubMed] [Google Scholar]
- 6. Waien SA, Lui PP, Ross BL, Williams WG, Webb GD, McLaughlin PR: Serial follow up of adults with repaired tetralogy of Fallot. J Am Coll Cardiol 1992; 20: 295–300 [DOI] [PubMed] [Google Scholar]
- 7. Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B: 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. J Am Coll Cardiol 1997; 30: 1374–1383 [DOI] [PubMed] [Google Scholar]
- 8. Gatzoulis MA, Till JA, Somerville J, Redington AN: Mechano‐electrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrythmias and sudden death. Circulation 1995; 92: 231–237 [DOI] [PubMed] [Google Scholar]
- 9. Gatzoulis MA, Till JA, Redington AN: Depolarization‐repolarization inhomogeneity after repair of tetralogy of Fallot: A substrate for malignant ventricular tachycardia? Circulation 1997; 95: 401–404 [DOI] [PubMed] [Google Scholar]
- 10. Berul CI, Hill SL, Geggel RL, Hijazi ZM, Marx GR, Rhodes J, Walsh KA, Fulton DR: Electrocardiographic markers of late sudden death risk in postoperative tetralogy of Fallot children. J Cardiovasc Electrophysiol 1997; 8: 1349–1356 [DOI] [PubMed] [Google Scholar]
- 11. Stelling JA, Danford DA, Kugler JD, Windle JR, Cheatham JP, Gumbiner CH, Latson LA, Hofschire PJ: Late potentials and inducible ventricular tachycardia in surgically repaired congenital heart disease. Circulation 1990; 82: 1690–1696 [DOI] [PubMed] [Google Scholar]
- 12. Balaji S, Lau YR, Case CL, Gillette PC: QRS prolongation is associated with inducible ventricular tachycardia after repair of tetralogy of Fallot. Am J Cardiol 1997; 80: 160–163 [DOI] [PubMed] [Google Scholar]
- 13. Cullen S, Cellarmajer DS, Franklin RCG, Hallidie‐Smith KA, Deanfield JE: Prognostic significance of ventricular arrhythmia after repair of tetralogy of Fallot: A 12‐year prospective study. J Am Coll Cardiol 1994; 23: 1151–1155 [DOI] [PubMed] [Google Scholar]
- 14. Lillehei CW, Varco RL, Cohen M, Warden HE, Gott VL, DeWall RA, Patton C, Moller JH: The first open heart corrections of tetralogy of Fallot. A 26–31 year follow‐up of 106 patients. Ann Surg 1986; 204: 490–502 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 15. Shimazaki Y, Blackstone EH, Kirklin JW: The natural history of isolated pulmonary valve incompetence: Surgical implications. Thorac Cardiovasc Surg 1984; 32: 257–259 [DOI] [PubMed] [Google Scholar]
- 16. Rowe SA, Zahka KG, Manolio TA, Horneffer PJ, Kidd L: Lung function and pulmonary regurgitation limit exercise capacity in post‐operative tetralogy of Fallot. J Am Coll Cardiol 1991; 17: 461–466 [DOI] [PubMed] [Google Scholar]
- 17. Norgård G, Bjørkhaug A, Vik‐Mo H: Effects of impaired lung function and pulmonary regurgitation on maximal exercise capacity in repaired tetralogy of Fallot. Eur Heart J 1992; 13: 1380–1386 [DOI] [PubMed] [Google Scholar]
- 18. Norgård G, Gatzoulis MA, Moraes F, Lincoln C, Shore DF, Shinebourne EA, Redington AN: Relationship between type of outflow tract repair and postoperative right ventricular diastolic physiology in tetralogy of Fallot. Circulation 1196; 94: 3276–3280 [DOI] [PubMed] [Google Scholar]
- 19. Wessel HU, Cunningham WJ, Paul MH, Bastanier CK, Muster AJ, Idriss FS: Exercise performance in tetralogy of Fallot after intracardiac repair. J Thorac Cardiovasc Surg 1980; 80: 582–593 [PubMed] [Google Scholar]
- 20. Carvalho JS, Shinebourne EA, Busst C, Redington AN: Exercise capacity after complete repair of tetralogy of Fallot: Deleterious effects of residual pulmonary regurgitation. Br Heart J 1992; 67: 470–473 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 21. Redington AN, Oldershaw PJ, Shinebourne EA, Rigby ML: A new technique for the assessment of pulmonary regurgitation and its application to the assessment of right ventricular function before and after repair of tetralogy of Fallot. Br Heart J 1988; 60: 57–65 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 22. Marie PY, Macron F, Brunotte F, Briancon S, Danchin N, Worns AM, Rober J, Pernot C: Right ventricular overload and induced sustained ventricular tachycardia in operatively “repaired” tetralogy of Fallot. Am J Cardiol 1992; 69: 785–789 [DOI] [PubMed] [Google Scholar]
- 23. Quattlebaum TG, Varghese J, Neill CA, Donahoo JS: Sudden death among postoperative patients with tetralogy of Fallot: A follow up study of 243 patients for an average of twelve years. Circulation 1976; 54: 289–293 [DOI] [PubMed] [Google Scholar]
- 24. Bove EL, Byrum CJ, Deaver T, Kavey RW, Sondheimer HM, Blackman MS, Parker FB: The influence of pulmonary insufficiency on ventricular function following repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1983; 85: 691–696 [PubMed] [Google Scholar]
- 25. Warner KG, Anderson JE, Fulton DR, Payne DD, Geggel RL, Marx GR: Restoration of the pulmonary valve reduces right ventricular volume overload after previous repair of tetralogy of Fallot. Circulation 1993; 88 (2): 189–197 [PubMed] [Google Scholar]
- 26. Finck SJ, Puga FJ, Danielson GK: Pulmonary valve insertion during reoperation for tetralogy of Fallot. Ann Thorac Surg 1988; 45: 610–613 [DOI] [PubMed] [Google Scholar]
- 27. Bove EL, Kavey RW, Byrum CJ, Sondheimer HM, Blackman MS, Thomas FD: Improved right ventricular function following late pulmonary valve replacement for residual pulmonary insufficiency or stenosis. J Thorac Cardiovasc Surg 1985; 90: 50–55 [PubMed] [Google Scholar]
- 28. Ilbawi MN, Idriss FS, DeLeon SY, Muster AJ, Berry TE, Paul MH: Long‐term results of porcine valve insertion for pulmonary regurgitation following repair of tetralogy of Fallot. Ann Thorac Surg 1986; 41: 478–482 [DOI] [PubMed] [Google Scholar]
- 29. Yemets IM, Williams WG, Webb GD, Harrison DA, McLaughlin PR, Trusler GA, Coles JG, Rebeyka IM, Freedom RM: Pulmonary valve replacement late after repair of tetralogy of Fallot. Ann Thorac Surg 1997; 64: 526–530 [DOI] [PubMed] [Google Scholar]
- 30. Davis CP, McKinnon GC, Debatin JF, Wetter D, Eichenberger AC, Duewell S, von Schulthess GK: Normal heart: Evaluation with echo‐planar MR imaging. Radiology 1994; 191: 691–696 [DOI] [PubMed] [Google Scholar]
- 31. Seelos KC, von Smekal A, Vahlensieck M, Gieseke J, Reiser M: Cardiac abnormalities: Assessment with T2‐weighted turbo spin‐echo MR imaging with electrocardiogram gating at 0.5 T. Radiology 1993; 189: 517–522 [DOI] [PubMed] [Google Scholar]
- 32. Hernandez RJ, Aisen AM, Foo TKF, Beekman RH: Thoracic cardiovascular anomalies in children: Evaluation with a fast gradient‐recalled‐echo sequence with cardiac‐triggered segmented acquisition. Radiology 1993; 188: 775–780 [DOI] [PubMed] [Google Scholar]
- 33. Hirsch R, Kilner PJ, Connelly MS, Redington AN, St. John Sutton MG, Somerville J: Diagnosis in adolescents and adults with congenital heart disease: Prospective assessment of individual and combined roles of magnetic resonance imaging and transesophageal echocardiography. Circulation 1994; 90: 2937–2951 [DOI] [PubMed] [Google Scholar]
- 34. Rebergen SA, Chin JA, Ottenkamp J, van der Wall EE, de Roos A: Pulmonary regurgitation in the late postoperative follow‐up of tetralogy of Fallot: Volumetric quantitation by nuclear magnetic resonance velocity mapping. Circulation 1993; 88 (part 1): 2257–2266 [DOI] [PubMed] [Google Scholar]
- 35. Helbing WA, Niezen RA, Le Cessie S, van der Geest RJ, Ottenkamp J, de Roos A: Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: Volumetric evaluation by magnetic resonance velocity mapping. J Am Coll Cardiol 1996; 28: 1827–1835 [DOI] [PubMed] [Google Scholar]
- 36. Niezen RA, Helbing WA, van der Wall EE, van der Geest RJ, Rebergen SA, de Roos A: Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair of tetralogy of Fallot. Radiology 1996; 201: 135–140 [DOI] [PubMed] [Google Scholar]
- 37. Grant RP, Estes EH Jr: Spatial Vectorcardiography: Clinical Electrocardiographic Interpretation, p. 14 Philadelphia: The Blakiston Company, 1951. [Google Scholar]
- 38. Park MK, Guntheroth WG: How to Read ECGs, ed. 3 St Louis: Mosby, 1992. [Google Scholar]
- 39. Gahlinger PM, Abramson JH: Computer Programs for Epidemiological Analysis (PEPI), Version 2. Stone Mountain, Georgia: USD, Inc., 1995. [Google Scholar]