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Severe Ebstein’s Anomaly Can Benefit From a Small Ventricular Septal Defect: Two Cases

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Abstract

Ebstein’s anomaly is a rare congenital heart defect. Associated lesions are uncommon, and the mortality rates can be as high as 54% during the first month of life. Two cases of severe Ebstein’s anomaly with ventricular septal defect are described. It is speculated that this rare association, allowing adequate forward pulmonary blood flow in the neonate, permitted the reported patients to survive the neonatal period, which is the most life-threatening time. The authors propose that the presence of a small ventricular septal defect can be beneficial for such patients, averting the need for surgery during early infancy when the risk is highest.

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References

  1. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK (2005) Ebstein’s anomaly: review of a multifaceted congenital cardiac condition. Swiss Med Wkly 135:269–281

    PubMed  CAS  Google Scholar 

  2. Attenhofer Jost CH, Connolly HM, O’Leary PW, Warnes CA, Tajik AJ, Seward JB (2005) Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 80:361–368

    Article  PubMed  Google Scholar 

  3. Carpentier A, Chauvaud S, Mace L, Relland J, Mihaileanu S, Marino JP, Abry B, Guibourt (1988) A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 96:92–101

  4. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE (1994) Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 23:170–176

    PubMed  CAS  Google Scholar 

  5. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE (1992) Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 19:1041–1046

    Article  PubMed  CAS  Google Scholar 

  6. Ebaid M, Vila JH, Pedroso CO, Stolf NA, Verginelli G, Macruz R (1980) Ebstein-type anomaly associated with intraventricular communication: report of 4 cases. Arq Bras Cardiol 34:129–133

    PubMed  CAS  Google Scholar 

  7. Gaussin V, Morley GE, Cox L, Zwijsen A, Vance KM, Emile L, Tian Y, Liu J, Hong C, Myers D, Conway SJ, Depre C, Mishina Y, Behringer RR, Hanks MC, Schneider MD, Huylebroeck D, Fishman GI, Burch JB, Vatner SF (2005) Alk3/Bmpr1a receptor is required for development of the atrioventricular canal into valves and annulus fibrosus. Circ Res 97:219–226

    Article  PubMed  CAS  Google Scholar 

  8. Knott-Craig CJ, Overholt ED, Ward KE, Razook JD (2000) Neonatal repair of Ebstein’s anomaly: indications, surgical technique, and medium-term follow-up. Ann Thorac Surg 69:1505–1510

    Article  PubMed  CAS  Google Scholar 

  9. Kumar AE, Fyler DC, Miettinen OS, Nadas AS (1971) Ebstein’s anomaly. Clinical profile and natural history. Am J Cardiol 28:84–95

    Article  PubMed  CAS  Google Scholar 

  10. Miller MS, Rao PN, Dudovitz RN, Falk RE (2005) Ebstein anomaly and duplication of the distal arm of chromosome 15: report of two patients. Am J Med Genet A 139:141–145

    PubMed  Google Scholar 

  11. Santoro G, Pisacane C, Bigazzi MC, Russo MG, Calabrò R (2000) Ebstein’s anomaly associated with ventricular septal defect and pulmonary stenosis. Ital Heart J 1:705

    PubMed  CAS  Google Scholar 

  12. Sarris GE, Giannopoulos NM, Tsoutsinos AJ, Chatzis AK, Kirvassilis G, Brawn WJ, Comas JV, Corno AF, Di Carlo D, Fragata J, Hraska V, Jacobs JP, Krupianko S, Sairanen H, Stellin G, Urban A, Ziemer G, European Congenital Heart Surgeons Association (2006) Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg 132:50–57

    Article  PubMed  Google Scholar 

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Correspondence to G. Rinelli.

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Del Pasqua, A., de Zorzi, A., Sanders, S.P. et al. Severe Ebstein’s Anomaly Can Benefit From a Small Ventricular Septal Defect: Two Cases. Pediatr Cardiol 29, 217–219 (2008). https://doi.org/10.1007/s00246-007-9097-8

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  • DOI: https://doi.org/10.1007/s00246-007-9097-8

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