What prognosis for Ebstein's anomaly without surgery? Hassan II teaching hospital experience.
Keywords:
Key words: Ebstein anomaly, prognosis, managementAbstract
Abstract:
Background: From the first description in 1886, significant progress was made on the treatment of Ebstein disease by mono and bi-ventricular surgery.
Aim: To highlight the prognosis of Ebstein's anomaly in the pediatric department of Hassan II hospital in Fez.
Methods: This is a descriptive and retrospective study of 4 years.
Results: We collected nine patients (seven boys and two girls) with a mean age of 5.3 years. Cyanosis was the main mode of revelation of the disease. In three patients Ebstein's anomaly was part of a malformative syndrome. All patients had only symptomatic medical treatment. Five patients are still alive at a follow up of one to six years.
Conclusion: Symptomatic medical treatment of Ebstein malformation may improve the prognosis of the disease.
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References
Geerdink LM, Kapusta L. Dealing with Ebstein anomaly. Cardiol Young 2014 Jul; 24:191-200
Kapusta L, Eveleigh RM, Poulino SE. Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre,long-term study. Eur Heart J 2007; 28:2661-2666
Pflaumer A, Eicken A, Augustin N, Hess J. Symptomatic neonates with Ebstein anomaly. J Thorac Cardiovasc Surg 2004; 127:1208-1209.
Brown ML, Dearani JA. Ebstein Malformation of the Tricuspid Valve: Current Concepts in Management and Outcomes. Curr Treat Options Cardiovasc Med 2009; 11:396-402
Mann RJ, Lie JT. The life story of Wilhelm Ebstein (1836-1912) and his almost over looked description of a congenital heart disease. Mayo Clin Proc 1979; 54:197-204
Sulafa A, Nuha N. Clinical and echocardiographic features of Ebstein's malformation in Sudanese patients. Cardiol Young 2006; 16:147-151
Oxenius A, Jost CH, Pretre R et al. Management and outcome of Ebstein's anomaly in children, Cardiol Young 2013; 23:27-34
Negoi RI, Ispas AT, Ghiorghiu I et al. Complex Ebstein's Malformation: Defining Preoperative Cardiac Anatomy and Function. J Card Surg 2013; 28:70-81
Arizmendi AF, Pineda LF, Jimenez CQ. The clinical profile of Ebstein's malformation as seen from the fetus to the adult in 52 patients. Cardiol Young 2004; 14:55-63
Shinkawa T, Polimenakos AC, Gomez-Fifer CA et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2010; 139:354-358
Del Pasqua A, De Zorzi A, Sanders SP. Severe Ebstein's Anomaly Can Benefit From a Small Ventricular Septal Defect: Two Cases. Pediatr Cardiol 2008; 29:217-219
Bettinelli AL, Mulder TJ, Funke BH, Lafferty KA, Longo SA, Niyazov DM. Familial Ebstein anomaly, left ventricular hypertrabeculation, and ventricular septal defect associated with a MYH7 mutation. Am J Med Genet A Semin Med Genet 2013; 161:3187-3190
Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170-6
Roberson DA, Silverman NH. Ebstein's malformation: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 1989; 14:1300-1307
Celermajer DS, Dodd SM. Morbid Anatomy in Neonates with Ebstein's Anomaly of the Tricuspid Valve: Pathophysiologic and Clinical Implications. Pediatr Cardiol. 1992;19(5):101-105
Andrew HS, Moore IE, Fagg N. Histological Changes in the Left and Right Ventricle in Hearts with Ebstein's Malformation and Tricuspid Valvar Dysplasia: A Morphometric Study of Patients Dying in the Fetal and Perinatal Periods. Cardiovasc pathol 1995 Mar; 4(1):19-24
Bove EL, Hirsch JC, Ohye RG, Devaney EJ. How I Manage Neonatal Ebstein's Anomaly. Pediatr Cardiol 2012; 30:63-65
Jin Yu J, Yun TJ, Won HS, Im YM, Lee BS et al. Outcome of Neonates with Ebstein's Anomaly in the Current Era. Pediatr Cardiol 2013; 34:1590- 1596
Boston US, Goldberg SP, Ward KE, OverholT ED, Spentzas T, Chin TK, Knott-Craig CJ. Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up. J Thorac Cardiovasc Surg 2011; 141:1163-1169
Geerdink LM, Kapusta L. Dealing with Ebstein's anomaly. Cardiol Young 2014; 24:191-200
Younoszai AK, Brook MM, Silverman NH. Ebstein's Malformation. Pediatr Cardiol 1999; 1:363-372
Paranon S, Acar P. Ebstein's anomaly of the tricuspid valve: from fetus to adult. Heart 2008; 94:237-243
Sano S, Fujii Y, Kasahara S, Kuroko Y, Tateishi A, Yoshizumi K, Arai S. Repair of Ebstein's anomaly in neonates and small infants: impact of right ventricular exclusion and its indications. Eur J Cardiothor Surg 2014; 45:549-555
