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The Journal of Thoracic and Cardiovascular Surgery, Vol 101, 1082-1087, Copyright © 1991 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
VA Starnes, PT Pitlick, D Bernstein, ML Griffin, M Choy and NE Shumway
Ebstein's anomaly appearing during the neonatal period carries a high
mortality rate. These infants exhibit cyanosis, acidosis, and congestive
heart failure. The pathophysiologic characteristics consist of severe
tricuspid regurgitation and functional pulmonary atresia. As a result of
the inability of the right ventricle to generate forward flow through the
pulmonary arteries, these infants remain dependent on ductal patency. Since
May 1988, five newborn infants with severe Ebstein's anomaly have been
admitted for treatment at our institution. At initial examination, they
weighed 3.6 +/- 1.8 kg and had a mean oxygen tension of 29.6 +/- 2.3 mm Hg
and a mean pH of 7.20 +/- 0.05. Chest roentgenography demonstrated a mean
cardiothoracic ratio of 0.81 +/- 0.02. As determined by echocardiography,
the right atria were massively enlarged, severe tricuspid regurgitation was
present in all patients, and the pulmonary valves were not opening. All
infants were dependent on prostaglandin E1 and attempts to wean them from
this drug were unsuccessful. Palliative treatment consisted of tricuspid
closure with autologous pericardium and an aortopulmonary shunt of 4 mm
polytetrafluoroethylene tubing. There were no operative or late deaths. At
discharge, mean oxygen tension was 42.2 +/- 0.85 mm Hg and mean systemic
oxygen saturation was 83.2% +/- 1.94%. Infants have grown satisfactorily
during the follow-up period. Three infants have since returned for further
surgical intervention. One infant, at 11 months of age, underwent a Glenn
anastomosis for progressive oxygen desaturation. Two infants have returned,
at ages 23 and 22 months, for Fontan procedures, which represent their
definitive operative management. We believe this new procedure offers
excellent palliative treatment for Ebstein's anomaly in critically ill
neonates. Feasibility of later definitive correction is demonstrated by the
good results obtained with the Fontan procedure in two infants.
ARTICLES
Ebstein's anomaly appearing in the neonate. A new surgical approach
Department of Cardiovascular Surgery, Stanford University School of Medicine, Calif.
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 L. Kapusta, R. M. Eveleigh, S. E. Poulino, M. E. Rijlaarsdam, G. J. du Marchie Sarvaas, J. L. Strengers, T. Delhaas, C. L. de Korte, T. Feuth, and W. A. Helbing Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study Eur. Heart J., November 1, 2007; 28(21): 2661 - 2666. [Abstract] [Full Text] [PDF]
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N. T. Ogus, C. Indelen, T. Yildirim, O. Selimoglu, and M. Basaran Pericardial Patch Augmentation of Both Anterior and Septal Leaflets in Ebstein's Anomaly Ann. Thorac. Surg., February 1, 2007; 83(2): 676 - 678. [Abstract] [Full Text] [PDF]
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D. P.V. Bichell, B. N. Mora, J. W. Mathewson, S. K. Kirkpatrick, J. J. Tyner, and T. McLees-Palinkas Modified Technique for the Surgical Treatment of Severe Tricuspid Valve Deformity in Ebstein's Anomaly Ann. Thorac. Surg., February 1, 2007; 83(2): 678 - 680. [Abstract] [Full Text] [PDF]
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C.-H. Lai, J.-M. Wu, and Y.-J. Yang Ebstein's Anomaly of the Tricuspid Valve in Combination of Tetralogy of Fallot: Total Correction in Infancy Ann. Thorac. Surg., January 1, 2007; 83(1): 304 - 306. [Abstract] [Full Text] [PDF]
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 B. L. Reemtsen, B. T. Fagan, W. J. Wells, and V. A. Starnes Current surgical therapy for Ebstein anomaly in neonates J. Thorac. Cardiovasc. Surg., December 1, 2006; 132(6): 1285 - 1289. [Abstract] [Full Text] [PDF]
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G. E. Sarris, N. M. Giannopoulos, A. J. Tsoutsinos, A. K. Chatzis, G. Kirvassilis, W. J. Brawn, J. V. Comas, A. F. Corno, D. Di Carlo, J. Fragata, et al. Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association J. Thorac. Cardiovasc. Surg., July 1, 2006; 132(1): 50 - 57. [Abstract] [Full Text] [PDF]
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T.-J. Yun, S.-H. Lee, and J.-K. Ko Neonatal stenotic Ebstein's anomaly: A novel technique of right ventricular exclusion J. Thorac. Cardiovasc. Surg., February 1, 2006; 131(2): 469 - 471. [Full Text] [PDF]
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P. E.F. Daubeney, D. Wang, D.J. Delany, B.R. Keeton, R.H. Anderson, Z. Slavik, M. Flather, S.A. Webber, and for the UK and Ireland Collaborative Study of Pulm Pulmonary atresia with intact ventricular septum: Predictors of early and medium-term outcome in a population-based study J. Thorac. Cardiovasc. Surg., October 1, 2005; 130(4): 1071 - 1071. [Abstract] [Full Text] [PDF]
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A. Murakami, T. Takaoka, J. Kobayashi, K. Maeda, T. Yamamoto, N. Motomura, S. Takamoto, H. Sugimura, K. Shibuya, H. Kato, et al. In situ implantation of a valved femoral vein allograft in a neonate with pulmonary atresia with intact ventricular septum and severe tricuspid regurgitation J. Thorac. Cardiovasc. Surg., April 1, 2004; 127(4): 1198 - 1199. [Full Text] [PDF]
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A. Pflaumer, A. Eicken, N. Augustin, and J. Hess Symptomatic neonates with Ebstein anomaly J. Thorac. Cardiovasc. Surg., April 1, 2004; 127(4): 1208 - 1209. [Full Text] [PDF]
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M. Watanabe, Y. Harada, T. Takeuchi, G. Satomi, and S. Yasukouchi Modified Starnes operation for neonatal Ebstein's anomaly Ann. Thorac. Surg., September 1, 2002; 74(3): 916 - 917. [Abstract] [Full Text] [PDF]
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C. J. Knott-Craig, E. D. Overholt, K. E. Ward, J. M. Ringewald, S. S. Baker, and J. D. Razook Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up Ann. Thorac. Surg., June 1, 2002; 73(6): 1786 - 1793. [Abstract] [Full Text] [PDF]
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S. Sano, K. Ishino, M. Kawada, S. Kasahara, T. Kohmoto, M. Takeuchi, and S.-i. Ohtsuki Total right ventricular exclusion procedure: An operation for isolated congestive right ventricular failure J. Thorac. Cardiovasc. Surg., April 1, 2002; 123(4): 640 - 647. [Abstract] [Full Text] [PDF]
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C. J. Knott-Craig, E. D. Overholt, K. E. Ward, and J. D. Razook Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up Ann. Thorac. Surg., May 1, 2000; 69(5): 1505 - 1510. [Abstract] [Full Text] [PDF]
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J. A. Dearani and G. K. Danielson Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease Ann. Thorac. Surg., April 1, 2000; 69(4): S106 - 117. [Abstract] [Full Text] [PDF]
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S. John and C. Rao Prosthetic valve replacement in ebstein’s malformation: 30-year follow-up Ann. Thorac. Surg., August 1, 1999; 68(2): 556 - 557. [Abstract] [Full Text] [PDF]
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J. A.M. van Son, P. Kinzel, and F. W. Mohr Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein's anomaly Ann. Thorac. Surg., November 1, 1998; 66(5): 1831 - 1832. [Abstract] [Full Text] [PDF]
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 J.A.M. van Son, V. Falk, M.D Black, G.S. Haas, and F.W. Mohr Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia Eur. J. Cardiothorac. Surg., March 1, 1998; 13(3): 280 - 285. [Abstract] [Full Text] [PDF]
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M. Endo, M. Ohmi, K. Sato, T. Tanaka, H. Kakizawa, and K. Tabayashi Tricuspid Valve Closure for Neonatal Ebstein's Anomaly Ann. Thorac. Surg., February 1, 1998; 65(2): 540 - 540. [Abstract] [Full Text] [PDF]
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N. Augustin, P. Schmidt-Habelmann, M. Wottke, H. Meisner, and F. Sebening Results After Surgical Repair of Ebstein's Anomaly Ann. Thorac. Surg., June 1, 1997; 63(6): 1650 - 1656. [Abstract] [Full Text]
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