Experience of medical treatment of neonates with Ebstein anomaly in the intensive care unit

Cover Page


Cite item

Abstract

Background: Neonates with Ebstein anomaly may remain a  challenge for neonatal intensive care units due to continuously changing clinical manifestations. Maintaining an adequate cardiac output in the presence of cardiomegaly, severe cyanosis, high pulmonary vascular resistance, and rhythm disorders is a difficult task.

Aim: To demonstrate the possibility of conservative management, as well as clinical and instrumental criteria of assessment of neonates with Ebstein anomaly and arterial hypoxemia and cardiomegaly.

Materials and methods: Four neonates aged from 3 to 8 days of life were admitted to the neonate intensive care unit due to persistent arterial hypoxemia (SaO2 ≤ 90%) and O2-dependence. Their cardiothoracic ratios ranged from 55 to 75% and were higher in the neonate with initial severe tricuspidal insufficiency (stage 3–4). Great Ormond Street Echocardiography (GOSE) score > 1 was found in 2 neonates with clinical manifestation of severe heart failure, arterial hypoxemia and cardiomegaly.

Results: During the following 3 to 4 days, two neonates (aged 5 and 6 days) were transferred to the 2nd stage of care due to the absence of clinical manifestations of heart failure and severe hypoxemia. In the other two cases, intensive management was necessary, with catecholamine support, oxygen therapy, infusion of prostaglandin E1, anti-arrhythmic agents, with no surgical intervention.

Conclusion: The majority of symptomatic neonates with Ebstein anomaly can be stabilized in the settings of adequate conservative management. However, some of them would require transition from conservative treatment to surgical intervention. Such clinical and instrumental criteria, as SaO2, cardiothoracic ratio, and GOSE are the main benchmarks to choose between conservative and surgical strategy.

About the authors

M. V. Tarayan

Moscow Regional Research and Clinical Institute (MONIKI)

Author for correspondence.
Email: tarayan@mail.ru

Marat V. Tarayan – MD, PhD, Cardiovascular Surgeon, Department of Pediatric Cardiac Surgery.

61/2–5 Shchepkina ul., Moscow, 129110, tel.: +7 (916) 693 81 35

Russian Federation

N. V. Shkarina

Moscow Regional Research and Clinical Institute (MONIKI)

Email: fake@neicon.ru

Nataliya V. Shkarina – MD, Pediatric Anesthesiologist, Department of Pediatric Intensive Care.

61/2 Shchepkina ul., Moscow, 129110

Russian Federation

E. S. Efremov

Moscow Regional Research and Clinical Institute (MONIKI)

Email: fake@neicon.ru

Evgeniy S. Efremov – MD, Cardiovascular Surgeon, Department of Cardiac Surgery.

61/2 Shchepkina ul., Moscow, 129110

Russian Federation

References

  1. Dearani JA, Danielson GK. Ebstein's anomaly of the tricuspid valve. In: Mavroudis C, Backer CL, editors. Pediatric Cardiac Surgery. 3rd edition. Philadelphia, Pa, USA: Mosby; 2003. p. 524–36.
  2. van Son JA, Konstantinov IE, Zimmermann V. Wilhelm Ebstein and Ebstein's malformation. Eur J Cardiothorac Surg. 2001;20(5): 1082–5. doi: 10.1016/S1010-7940(01)00913-7.
  3. Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69(3 Suppl 1):S106–17. doi: 10.1016/S0003-4975(99)01265-5.
  4. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT. Morphologic spectrum of Ebstein's anomaly of the heart: a review. Mayo Clin Proc. 1979;54(3): 174–80.
  5. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation. 2007;115(2): 277–85. doi: 10.1161/CIRCULATIONAHA.106.619338.
  6. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol. 1998;81(6): 749–54. doi: 10.1016/S0002-9149(97)01009-6.
  7. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE. Outcome in neonates with Ebstein's anomaly. J Am Coll Cardiol. 1992;19(5): 1041–6. doi: 10.1016/07351097(92)90291-T.
  8. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg. 1992;104(5): 1195–202.
  9. Bove EL, Hirsch JC, Ohye RG, Devaney EJ. How I manage neonatal Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009:63–5. doi: 10.1053/j.pcsu.2009.01.023.
  10. Goldberg SP, Jones RC, Boston US, Haddad LM, Wetzel GT, Chin TK, Knott-Craig CJ. Current trends in the management of neonates with Ebstein's anomaly. World J Pediatr Congenit Heart Surg. 2011;2(4): 554–7. doi: 10.1177/2150135111416016.
  11. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg. 1991;101(6): 1082–7.
  12. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg. 2006;132(6): 1285–90. doi: 10.1016/j.jtcvs.2006.08.044.
  13. van Son JA, Falk V, Black MD, Haas GS, Mohr FW. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiothorac Surg. 1998;13(3): 280–5. doi: 10.1016/S10107940(98)00009-8.
  14. Knott-Craig CJ, Goldberg SP. Management of neonatal Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007;10(1): 112–6. doi: 10.1053/j.pcsu.2007.01.008.
  15. 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(5): 1163–9. doi: 10.1016/j.jtcvs.2011.01.029.
  16. Dearani JA, Said SM, O'Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg. 2013;95(1): 220–6. doi: 10.1016/j.athoracsur.2012.04.146.
  17. Anderson HN, Dearani JA, Said SM, Norris MD, Pundi KN, Miller AR, Cetta ML, Eidem BW, O'Leary PW, Cetta F. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014;9(3): 266–71. doi: 10.1111/chd.12155.
  18. Krivoshchekov EV, Ackerman JP, Yanulevich OS, Sokolov AA, Ershova NV, Dearani JA, Cetta F. Modified cone reconstruction of the tricuspid valve for Ebstein anomaly as performed in Siberia. Tex Heart Inst J. 2017;44(1): 39–42. doi: 10.14503/THIJ-16-5832.
  19. Ibrahim M, Tsang VT, Caruana M, Hughes ML, Jenkyns S, Perdreau E, Giardini A, Marek J. Cone reconstruction for Ebstein's anomaly: Patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg. 2015;149(4): 1144–50. doi: 10.1016/j.jtcvs.2014.12.074.
  20. Wackel PL, Dearani JA, Cetta F. Neonatal Ebstein repair – where are we now? Ann Transl Med. 2017;5(5): 109. doi: 10.21037/atm.2017.01.19.
  21. Amplatz K, Lester RG, Schiebler GL, Adams P Jr, Anderson RC. The roentgenologic features of Ebstein's anomaly of the tricuspid valve. Am J Roentgenol Radium Ther Nucl Med. 1959;81(5): 788–94.
  22. Perloff JK. Ebstein's anomaly of the tricuspid valve. In: Perloff JK, editor. Clinical Recognition of Congenital Heart Disease. 5th edition. Philadephia, PA: Saunders; 2003. p. 194–215.
  23. Akdeniz C, Ergul Y, Kiplapinar N, Tuzcu V. Catheter ablation of drug resistant supraventricular tachycardia in neonates and infants. Cardiol J. 2013;20(3): 241–6. doi: 10.5603/CJ.2013.0068.

Supplementary files

There are no supplementary files to display.


Copyright (c) 2018 Tarayan M.V., Shkarina N.V., Efremov E.S.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies