Abstract
The paper describes two cases of successful surgical correction of the extreme forms of Ebstein anomaly in combination with pulmonary
atresia in newborns. In both cases, the diagnosis of Ebstein anomaly and pulmonary atresia was made prenatally, which
made it possible to start therapy and plan transportation to the intensive care unit of a specialized hospital immediately after birth.
The condition of both newborns at the time of admission was assessed as extremely severe. A cone reconstruction technique with
simultaneous prosthetic repair of the pulmonary valve by a venous valve-containing homograft was used, which made it possible to
perform a radical correction of the defect while maintaining biventricular hemodynamics. In the presence of concomitant pulmonary
atresia using valve-containing conduit excludes the right ventricular volume overload and contributes to the restoration of good contractile
function of the right ventricular myocardium in the early postoperative period.
References
- Attenhofer Jost C.H., Connolly H.M., Dearani J.A., Edwards W.D.,
Danielson G.K. Ebstein's anomaly. Circulation. 2007; 115 (2):
277–85. DOI: 10.1161/circulationaha.106.619338
- Epstein M.L. Congenital stenosis and insufficiency of the tricuspid
valve. In: Allen H.D., Clark E.B., Gutgesell H.P., Driscoll
D.J. (Eds.) Moss and Adams' heart disease in infants,
children, and adolescents, including the fetus and young adult.
Children’s Heart and Vascular Diseases. 2019; 16 (2)
DOI: 10.24022/1810-0686-2019-16-2-134-138
6th ed. Vol 2. Philadelphia: Lippincott Williams and Wilkins;
2001: 810–9.
- Dearani J.A., Danielson G.K. Ebstein's Anomaly. In: Sellke F.W.,
del Nido P.J., Swanson S.J. (Eds.) Sabiston and Spencer's
surgery of the chest. 7th ed. Vol 2. Philadelphia: Elsevier
Saunders; 2005: 2219–31.
- Boston S.U., Goldberg S.P., Ward K.E., Overholt E.D., Spentzas
T., Chin T.K., Knott-Craig C.J. 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
- Andrews R.E., Tibby S.M., Sharland G.K., Simpson J.M. Prediction
of outcome of tricuspid valve malformations diagnosed
during fetal life. Am. J. Cardiol. 2008; 101 (7): 1046–50.
DOI: 10.1016/j.amjcard.2007.11.049
- Kapusta L., Eveleigh P.M., Poulino S., Rijlaarsam M.E.,
du Marchie Sarvass G.J., Strengers J.L. et al. Ebstein's anomaly:
factors associated with death in childhood and adolescence:
a multi-centre, long-term study. Eur. Heart J. 2007; 28
(21): 2661–6. DOI: 10.1093/eurheartj/ehm398
- Celermajer D.S., Dodd S.M., Greenwald S.E., Wyse R.K.,
Deanfield J.E. Morbid anatomy in neonates with Ebstein's
anomaly of the tricuspid valve: pathophysiologic and clinical
implications. J. Am. Coll. Cardiol. 1992; 19 (5): 1049–53.
DOI: 10.1016/0735-1097(92)90293-v
- Starnes V.A., Pitlick P.T., Bernstein D., Griffin M.L., Choy M.,
Shumway N.E. Ebstein's anomaly appearing in the neonate.
A new surgical approach. J. Thorac. Cardiovasc. Surg. 1991;
101 (6): 1082–7.
- Knott-Craig C.J., Overholt E.D., Ward K.E., Razook J.D. Neonatal
repair of Ebstein's anomaly: indications, surgical technique,
and medium-term follow-up. Ann. Thorac. Surg. 2000;
69 (5): 1505–10. DOI: 10.1016/s0003-4975(00)01138-3
- Knott-Craig C.J., Kumar T.K., Arevalo A.R., Joshi V.M. Surgical
management of symptomatic neonates with Ebstein's
anomaly: choice of operation. Cardiol. Young. 2015; 25 (6):
1119–23. DOI: 10.1017/s1047951114001747
- Congenital Heart Surgery Database. Available at:
https://www.sts.org/registries-research-center/sts-nationaldatabase/
congenital-heart-surgery-database/data-collection
(accessed 11.06.2019).
- Holst K.A., Dearani J.A., Said S.M., Davies R.R., Pizarro C.,
Knott-Craig C. et al. Surgical management and outcomes of
Ebstein anomaly in neonates and infants: a Society of
Thoracic Surgeons Congenital Heart Surgery Database analysis.
Ann. Thorac. Surg. 2018; 106 (3): 785–91. DOI: 10.1016/j.athoracsur.2018.04.049
- Luxford J.C., Arora N., Ayer J.G., Verrall C.E., Cole A.D.,
Orr Y. et al. Neonatal Ebstein anomaly: a 30-year institutional
review. Semin. Thorac. Cardiovasc. Surg. 2017; 29 (2):
206–12. DOI: 10.1053/j.semtcvs.2017.01.012
About the authors
- Nikolay V. Antsygin, Surgeon, orcid.org/0000-0001-9206-8050;
-
Ruben R. Movsesyan, Dr. Med. Sc., Correspondent Member of RAS, Head of Department, Professor, orcid.org/0000-0003-3089-3113;
-
Vladimir A. Bolsunovsky, Cand. Med. Sc., Surgeon, Assistant Professor, orcid.org/0000-0002-1380-9680;
-
Aleksey A. Shikhranov, Cand. Med. Sc., Surgeon, orcid.org/0000-0001-9971-3390;
-
Sergey P. Shefer, Resuscitator, orcid.org/0000-0002-1814-7778;
-
Aleksandr E. Shagal, Resuscitator, orcid.org/0000-0002-0206-4310
