Predictors for Dilated Aorta in Repaired and Unrepaired Tetralogy of Fallot

Abstract

Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients at the level of annulus, STJ & sinus of Valsalva was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). Conclusions: The first important finding of this study is the occurrence of significant aortic root dilatation in 22% of patients after intra-cardiac repair of TOF. Older age at repair, long shunt to repair interval and residual ventricular septal defect are the most common variables associated with aortopathy and aortic regurgitation in such group of patients. The second important finding is the occurrence of aortic root dilatation in 70% of patients before surgical repair of TOF; whereas male sex and TOF with pulmonary atresia appeared to be the most common variables associated with aortopathy and aortic regurgitation in this group of patients.

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El Meguid, K. , Mahmoud, H. and Mohammad, M. (2015) Predictors for Dilated Aorta in Repaired and Unrepaired Tetralogy of Fallot. World Journal of Cardiovascular Diseases, 5, 233-253. doi: 10.4236/wjcd.2015.58027.

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1] Therrien, J. and Webb, G. (2003) Clinical Update on Adults with Congenital Heart Disease. The Lancet, 362, 1305-1313.
http://dx.doi.org/10.1016/S0140-6736(03)14574-6
[2] Niwa, K., Siu, S.C., Webb, G.D. and Gatzoulis, M.A. (2002) Progressive Aortic Root Dilatation in Adults Later after Repair of Tetralogy of Fallot. Circulation, 106, 1374-1378.
http://dx.doi.org/10.1161/01.CIR.0000028462.88597.AD
[3] McMahon, C.J., Ravekes, W.J. and Smith, E.O. (2004) Risk Factors for Neo-Aortic Root Enlargement and Aortic Regurgitation Following Arterial Switch Operation. Pediatric Cardiology, 25, 329-335.
http://dx.doi.org/10.1007/s00246-003-0483-6
[4] Tzemos, N., Lyseggen, E., Silversides, C., Jamorski, M., Tong, J.H., Harvey, P., et al. (2010) Endothelial Function, Carotid-Femoral Stiffness, and Plasma Matrix Metalloproteinase-2 in Men with Bicuspid Aortic Valve and Dilated Aorta. Journal of the American College of Cardiology, 55, 660–668.
http://dx.doi.org/10.1016/j.jacc.2009.08.080
[5] Oliver, J.M., Gallego, P., Gonzalez, A., Aroca, A., Bret, M. and Mesa, J.M. (2004) Risk Factors for Aortic Complications in Adults with Coarctation of the Aorta. Journal of the American College of Cardiology, 44, 1641-1647.
http://dx.doi.org/10.1016/j.jacc.2004.07.037
[6] Ono, M., Goerler, H., Boethig, D., Westhorr-Bleck, M., Hartung, D. and Breymann, T. (2007) Valve-Sparing Operation for Aortic Root Aneurysm Late after Mustard Procedure. The Annals of Thoracic Surgery, 83, 2224-2226.
http://dx.doi.org/10.1016/j.athoracsur.2006.12.043
[7] Lillehei, C.W., Varco, R.L., Cohen, M., Warden, H.E., Gott, V.L., DeWall, R.A., Patton, C. and Moller, J.H. (1986) The First Open Heart Corrections of Tetralogy of Fallot: A 26-31 Year Follow-Up of 106 Patients. Annals of Surgery, 204, 490-502.
http://dx.doi.org/10.1097/00000658-198610000-00017
[8] Capelli, H., Ross, D. and Somerville, J. (1982) Aortic Regurgitation in Tetrad of Fallot and Pulmonary Atresia. American Journal of Cardiology, 49, 1979-1983.
http://dx.doi.org/10.1016/0002-9149(82)90218-1
[9] Dodds, G.A., Warnes, C.A. and Danielson, G.K. (1997) Aortic Valve Replacement after Repair of Pulmonary Atresia and Ventricular Septal Defect or Tetralogy of Fallot. The Journal of Thoracic and Cardiovascular Surgery, 113, 736-741.
http://dx.doi.org/10.1016/S0022-5223(97)70232-0
[10] Marelli, A.J., Perloff, J.K., Child, J.S. and Laks, H. (1994) Pulmonary Atresia with Ventricular Septal Defect in Adults. Circulation, 89, 243-251.
http://dx.doi.org/10.1161/01.CIR.89.1.243
[11] Anitha, S.J., Rychik, J., Yang, W., et al. (2009) 22q11.2 Deletion Status and the Risk of Aortic Root Dilation in Pediatric Patients with Tetralogy of Fallot. Circulation, 120, S585-S586.
[12] Sahn, D.J., Demaria, A., Kisslo, J., et al. (1978) Recommendations Regarding Quantitation in M-Mode Echocardiography: Results of a Survey of Echocardiographic Measurements. Circulation, 58, 1072-1083.
http://dx.doi.org/10.1161/01.CIR.58.6.1072
[13] Broberg, C.S., Aboulhosn, J., Mongeon, F.P., Kay, J., Valente, A.M., Khairy, P., Earing, M.G., Opotowsky, A.R., Lui, G., Gersony, D.R., Cook, S., Ting, J.G., Webb, G. and Gurvitz, M.Z. (2011) Alliance for Adult Research in Congenital Cardiology (AARCC). Prevalence of Left Ventricular Systolic Dysfunction in Adults with Repaired Tetralogy of Fallot. American Journal of Cardiology, 107, 1215-1220.
http://dx.doi.org/10.1016/j.amjcard.2010.12.026
[14] Zoghbi, W.A., Enriquez-Sarano, M., Foster, E., Grayburn, P.A., Kraft, C.D., Levine, R.A., Nihoyannopoulos, P., Otto, C.M., Quinones, M.A., Rakowski, H., Stewart, W.J., Waggoner, A., Weissman, N.J. (2003) American Society of Echocardiography. Recommendations for Evaluation of the Severity of Native Valvular Regurgitation with Two-Dimensional and Doppler Echocardiography. Journal of the American Society of Echocardiography, 16, 777-802.
http://dx.doi.org/10.1016/S0894-7317(03)00335-3
[15] Suzuki, Y., Kambara, H., Kadota, K., et al. (1986) Detection and Evaluation of Tricuspid Regurgitation Using a Real-Time, Two-Dimensional, Color-Coded, Doppler Flow Imaging System: Comparison with Contrast Two-Dimensional Echocardiography and Right Ventriculography. American Journal of Cardiology, 57, 811-815.
http://dx.doi.org/10.1016/0002-9149(86)90619-3
[16] Williams, R.V., Minich, L.L., Shaddy, R.E., et al. (2002) Comparison of Doppler Echocardiography with Angiography for Determining the Severity of Pulmonary Regurgitation. American Journal of Cardiology, 89, 1438-1441.
http://dx.doi.org/10.1016/S0002-9149(02)02365-2
[17] Sahn, D.J., DeMaria, A., Kisslo, J., et al. (1978) Recommendations Regarding Quantitation in M-Mode Echocardiograpy: Results of a Survey of Echocardiographic Measurements. Circulation, 58, 1072-1083.
http://dx.doi.org/10.1161/01.CIR.58.6.1072
[18] Yang, M., Mo, X.M., Jin, J.Y., Wu, M., Liu, B., Liu, Z.Y., Gao, X.C., Tang, W.W. and Teng, G.J. (2010) Diagnostic Value of 64 Multislice CT in Typing of Congenital Aortic Anomaly in Neonates and Infants. National Medical Journal of China, 90, 2167-2171.
[19] Bailliard, F., Hughes, M.L. and Taylor, A.M. (2008) Introduction to Cardiac Imaging in Infants and Children: Techniques, Potential, and Role in the Imaging Workup of Various Cardiac Malformations and Other Pediatric Heart Conditions. European Journal of Radiology, 68, 191-198.
http://dx.doi.org/10.1016/j.ejrad.2008.05.016
[20] Tops, L.F., Wood, D.A., Delgado, V., et al. (2008) Noninvasive Evaluation of the Aortic Root with Multislice Computed Tomography. JACC: Cardiovascular Imaging, 1, 321-330.
http://dx.doi.org/10.1016/j.jcmg.2007.12.006
[21] Loren, F., George, L., Joshua, A., et al. (2010) Recommendations for Aortic Imaging Techniques to Determine the Presence and Progression of Thoracic Aortic Disease, Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease: Executive Summary. Circulation, 3, 14-17.
[22] Pettersen, M.D., Du, W., Skeens, M.E. and Humes, R.A. (2008) Regression Equations for Calculation of Z Scores of Cardiac Structures in a Large Cohort of Healthy Infants, Children, and Adolescents: An Echocardiographic Study. Journal of the American Society of Echocardiography, 8, 922-934.
http://dx.doi.org/10.1016/j.echo.2008.02.006
[23] Francois, K., Zaqout, M., Bové, T., et al. (2010) The Fate of the Aortic Root after Early Repair of Tetralogy of Fallot. European Journal Cardio-Thoracic Surgery, 37, 1254-1258.
[24] Stulak, J.M., Dearani, J.A., Burkhart, H.M., Sundt, T.M., Connolly, H.M., Schaff, H.V. (2010) Does the Dilated Ascending Aorta in an Adult with Congenital Heart Disease Require Intervention? The Journal of Thoracic and Cardiovascular Surgery, 140, S52-S57.
http://dx.doi.org/10.1016/j.jtcvs.2010.08.052
[25] Matsuoka, R., Takao, A., Kimura, M., et al. (1994) Confirmation that the Conotruncal Anomaly Face Syndrome Is Associated with Deletion within 22q11.2. American Journal of Medical Genetics, 53 285-289.
http://dx.doi.org/10.1002/ajmg.1320530314
[26] Tan, J.L., Davlouros, P.A., McCarthy, K.P., Gatzoulis, M.A. and Ho, S.Y. (2005) Intrinsic Histological Abnormalities of Aortic Root and Ascending Aorta in Tetralogy of Fallot: Evidence of Causative Mechanism for Aortic Dilatation and Aortopathy. Circulation, 112, 961-968.
http://dx.doi.org/10.1161/CIRCULATIONAHA.105.537928
[27] Hiratzka, L.F., Bakris, G.L., Beckman, J.A., Bersin, R.M., Carr, V.F., Casey Jr., D.E., Eagle, K.A., Hermann, L.K., Isselbacher, E.M., Kazerooni, E.A., Kouchoukos, N.T., Lytle, B.W., Milewicz, D.M., Reich, D.L., Sen, S., Shinn, J.A., Svensson, L.G. and Williams, D.M. (2010) Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease: A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation, 121, e266-e369.
[28] Bhat, A.H., Smith, C.J. and Hawker, R.E. (2004) Late Aortic Root Dilatation in Tetralogy of Fallot May Be Prevented by Early Repair in Infancy. Pediatric Cardiology, 25, 654-659.
http://dx.doi.org/10.1007/s00246-003-0665-2
[29] Rao, B.N.S., Anderson, R.C. and Edwards, J.E. (1971) Anatomic Variations in the Tetralogy of Fallot. American Heart Journal, 81, 361-371.
http://dx.doi.org/10.1016/0002-8703(71)90106-2
[30] Roman, M.J., Devereux, R.B., Kramer-Fox, R., et al. (1989) Two-Dimensional Echocardiographic Aortic Root Dimensions in Normal Children and Adults. American Journal of Cardiology, 64, 507-512.
http://dx.doi.org/10.1016/0002-9149(89)90430-X
[31] Niwa, K. (2005) Aortic Root Dilatation in Tetralogy of Fallot Long-Term after Repairhistology of the Aorta in Tetralogy of Fallot: Evidence of Intrinsic Aortopathy. International Journal of Cardiology, 103, 117-119.
http://dx.doi.org/10.1016/j.ijcard.2004.07.002
[32] Nagy, C.D., Alejo, D.E., Corretti, M.C., et al. (2013) Tetralogy of Fallot and Aortic Root Dilation: A Long-Term Outlook. Pediatric Cardiology, 34, 809-816.
[33] Hennein, H.A., Mosca, R.S., Urcelay, G., et al. (1995) Intermediate Results after Complete Repair of Tetralogy of Fallot in Neonates. The Journal of Thoracic and Cardiovascular Surgery, 109, 332-344.
http://dx.doi.org/10.1016/S0022-5223(95)70395-0
[34] Niwa, K., Perloff, J.K., Bhuta, S.M., et al. (2001) Structural Abnormalities of Great Arterial Walls in Congenital Heart Disease: Light and Electron Microscopic Analyses. Circulation, 103, 393-400.
http://dx.doi.org/10.1161/01.CIR.103.3.393
[35] Lee, L.C., Torres, M.C., Khoo, S.M., et al. (2010) The Relative Impact of Obstructive Sleep Apnea and Hypertension on the Structural and Functional Changes of the Thoracic Aorta. Sleep, 33, 1173-1176.
[36] Chong, W.Y., Wong, W.H., Chiu, C.S. and Cheung, Y.F. (2006) Aortic Root Dilation and Aortic Elastic Properties in Children after Repair of Tetralogy of Fallot. American Journal of Cardiology, 97, 905-909.
http://dx.doi.org/10.1016/j.amjcard.2005.09.141
[37] Peters, R.M. (1971) Total Repair of Tetralogy of Fallot and Replacement of Aortic Valve with Starr-Edwards Prosthesis. Case Report. The Journal of Thoracic and Cardiovascular Surgery, 61, 599-601.
[38] Chowdhury, U.K., Mishra, A.K., Ray, R., et al. (2008) Histopathological Changes in Ascending Aorta and the Risk Factors Related to Histopathological Conditions and Aortic Dilatation in Patients with Tetralogy of Fallot. The Journal of Thoracic and Cardiovascular Surgery, 135, 69-77.
http://dx.doi.org/10.1016/j.jtcvs.2007.06.011
[39] Kim, W.H., Seo, J.W., Kim, S.J., et al. (2005) Aortic Dissection Late after Repair of Tetralogy of Fallot. International Journal of Cardiology, 101, 515-516.
http://dx.doi.org/10.1016/j.ijcard.2004.03.026
[40] Rathi, V.K., Doyle, M., Williams, R.B., et al. (2005) Massive Aortic Aneurysm and Dissection in Repaired Tetralogy of Fallot; Diagnosis by Cardiovascular Magnetic Resonance Imaging. International Journal of Cardiology, 101, 169-170.
http://dx.doi.org/10.1016/j.ijcard.2004.05.037
[41] Sonesson, B., Lanne, T., Vesnersson, E., et al. (1994) Sex Difference in the Mechanical Properties of the Abdominal Aorta in Human Being. Journal of Vascular Surgery, 20, 959-965.
http://dx.doi.org/10.1016/0741-5214(94)90234-8
[42] Nollen, G.J., Groenink, M., Tijssen, J.G., Van Der Wall, E.E. and Mulder, B.J. (2004) Aortic Stiffness and Diameter Predict Progressive Aortic Dilatation in Patients with Marfan Syndrome. European Heart Journal, 25, 1146-1152.
http://dx.doi.org/10.1016/j.ehj.2004.04.033
[43] Nistri, S., Grande-Allen, J., Noale, M., Basso, C., Siviero, P., Maggi, S., Crepaldi, G. and Thiene, G. (2008) Aortic Elasticity and Size in Bicuspid Aortic Valve Syndrome. European Heart Journal, 29, 472-479.
http://dx.doi.org/10.1093/eurheartj/ehm528
[44] Faller, D.V. (1999) Endothelial Cell Re-sponses to Hypoxic Stress. Clinical and Experimental Pharmacology and Physiology, 26, 74-84.
http://dx.doi.org/10.1046/j.1440-1681.1999.02992.x
[45] Rao, B.N.S., Anderson, R.C. and Edwards, J.E. (1971) Anatomic Variations in the Tetralogy of Fallot. American Heart Journal, 81, 361-371.
http://dx.doi.org/10.1016/0002-8703(71)90106-2
[46] Johnson, M.C., Strauss, A.W., Dawton, B., et al. (1995) Deletion within Chromosome 22 Is Common in Patients with Absent Pulmonary Valve Syndrome. American Journal of Cardiology, 76, 66-69.
http://dx.doi.org/10.1016/S0002-9149(99)80803-0
[47] Marelli, A.J., Perloff, J.K., Child, J.S., et al. (1994) Pulmonary Atresia with Ventricular Septal Defect in Adults. Circulation, 89, 243-251.
http://dx.doi.org/10.1161/01.CIR.89.1.243
[48] Bull, K., Somerville, J., Ty, E., et al. (1995) Presentation and Attrition in Complex Pulmonary Atresia. Journal of the American College of Cardiology, 25, 491-499.
http://dx.doi.org/10.1016/0735-1097(94)00364-V
[49] Matsuda, H., Ihara, K., Mori, T., et al. (1980) Tetralogy of Fallot Associated with Aortic Insufficiency. The Annals of Thoracic Surgery, 29, 529-533.
http://dx.doi.org/10.1016/S0003-4975(10)61699-2
[50] Geva, T., Sandweiss, B.M., Gauvreau, K., et al. (2004) Factors Associated with Impaired Clinical Status in Long-Term Survivors of Tetralogy of Fallot Repair Evaluated by Magnetic Resonance Imaging. Journal of the American College of Cardiology, 43, 1068-1074.
http://dx.doi.org/10.1016/j.jacc.2003.10.045
[51] Anderson, R.H., Cook, A., Brown, N.A., Henderson, D.J., Chaudhry, B. and Mohun, T. (2010) Development of the Outflow Tracts with Reference to Aortopulmonary Windows and Aortoventricular Tunnels. Cardiology in the Young, 20, 92-99.
http://dx.doi.org/10.1017/S1047951110001137

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