Journal of Health Sciences & Surveillance System

How to manage Ebstein’s Anomaly in Pregnancy: A Literature Review with Case Study

Document Type : Case Study

Authors

Alireza Nematollahi 1
Shadi Zamansaraei 2
Farima Safari 3
Parvin Bahrami 4

1 Department of Internal Medicine, School of Medicine Al-Zahra Hospital Isfahan University of Medical Sciences, Isfahan, Iran

2 Department of Obstetrics and Gynecology, Isfahan University of Medical Sciences, Isfahan, Iran

3 Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran

4 Department of Cardiology, Alzahra University Hospital, Isfahan University of Medical Science, Isfahan, Iran

https://doi.org/10.30476/jhsss.2021.92186.1318
Abstract
Background: Ebstein’s anomaly (EA) is a congenital heart defect that causes cyanosis and arrhythmia. The treating physician has difficulty when it comes to pregnant women since patients frequently reach reproductive age. How to manage these patients during pregnancy or approach patients who are planning to become pregnant is an important issue. The aim of this study was to evaluate previous and current literature reviews, as well as case studies, to better understand how to treat Ebstein’s abnormality in pregnancy. Methods: This study is a literature review with case report. Here, we review the literature on this subject to discuss how to manage Ebstein’s anomaly in pregnancy. We analyzed the literature from different perspectives. We also focused on three of Ebstein’s anomaly-affected women’s pregnancies. Results: Three women had four pregnancies, all of which were delivered vaginally or through cesarean section. There were no preterm births. 2.540.88 kg was the average birth weight. There were no cardiac abnormalities in any of the three infants. One patient had Ebstein’s abnormality and congenitally corrected transposition of the great arteries (ccTGA). Conclusion: In Ebstein’s abnormality, pregnancy is often well tolerated. However, the maternal risks of pregnancy correlated with the severity of anatomical malformations and the presence of cyanosis or simultaneous other cardiac anomalies.

Keywords

Ebstein’s anomaly
Pregnancy
Congenital heart defect
Cardiovascular abnormality
Cardiovascular disease
  1. Sherwin ED, Abrams DJ. Ebstein Anomaly. Cardiac electrophysiology clinics. 2017 Jun 1;9(2):245-54.
  2. Freud LR, Escobar-Diaz MC, Kalish BT et al Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation 2015;132:481–489.
  3. Safi LM, Liberthson RR, Bhatt A. Current Management of Ebstein's Anomaly in the Adult. Curr Treat Options Cardiovasc Med 2016 ;18-56.
  4. Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly. Acta obstetricia et gynecologica Scandinavica. 2018 Aug;97(8):1025-31.
  5. Rydzewska K, Sylwestrzak O, Krekora M, Słodki M, Respondek-Liberska M. Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management. The Journal of Maternal-Fetal & Neonatal Medicine. 2020 Sep 15:1-8.
  6. Possner M, Gensini FJ, Mauchley DC, Krieger EV, Steinberg ZL. Ebstein’s Anomaly of the Tricuspid Valve: An Overview of Pathology and Management. Current Cardiology Reports. 2020 Dec;22(12):1-7.
  7. Gite JV, Gangakhedkar GR, Nadkarni M. Anaesthetic management of emergency lower segment caesarean section in a patient with Ebstein's anomaly. Indian journal of anaesthesia. 2018 Nov;62(11):915.
  8. Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly. Acta obstetricia et gynecologica Scandinavica. 2018 Aug;97(8):1025-31.
  9. Torigoe F, Ishida H, Ishii Y, Ishii R, Narita J, Kawazu Y, Kayatani F, Inamura N. Fetal echocardiographic prediction score for perinatal mortality in tricuspid valve dysplasia and Ebstein's anomaly. Ultrasound in Obstetrics & Gynecology. 2020 Feb;55(2):226-32.
  10. Masoller N, Del Rincón OG, Herraiz I, Gómez-Montes E, Soveral I, Pérez-Cruz M, Martínez-Biosques C, Granados MA, Bennasar M, Escobar-Diaz MC, Martínez JM. Prediction of Perinatal Mortality in Ebstein’s Anomaly Diagnosed in the Second Trimester of Pregnancy. Fetal diagnosis and therapy. 2020;47(8):604-14.
  11. Wu, Yuduo, Xiaoyan Gu, Yihua He, and Hongjia Zhang. "Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy." (2020).
  12. Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly. Acta obstetricia et gynecologica Scandinavica. 2018 Aug;97(8):1025-31.
  13. Regitz-Zagrosek, V., et al., 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy: The Task Force for the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). European Heart Journal 2018 ;39: 3165-3241.
  14. Siu SC, Sermer M, Colman JM, Alvarez AN, Mercier LA, Morton BC, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation. 2001;104:515–21.
  15. Drenthen W, Boersma E, Balci A, Moons P, RoosHesselink JW, Mulder BJ, et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J. 2010;31:2124–32.
  16. Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol. 1994 Apr; 23:1194-8. doi: 10.1016/0735-1097(94)90610-6. PMID: 8144788.
  17. Kanoh, M, Inai, K, Shinohara, T, Shimada, E, Shimizu, M, Tomimatsu, H, et al. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly. Acta Obstet Gynecol Scand 2018; 97: 1025– 1031.
  18. Possner M, Gensini FJ, Mauchley DC, Krieger EV, Steinberg ZL. Ebstein’s Anomaly of the Tricuspid Valve: An Overview of Pathology and Management. Current Cardiology Reports. 2020 Dec;22(12):1-7.
  19. Rydzewska K, Murlewska J, Krekora M, Maroszyńska I, Moll M, Respondek-Liberska M. The attempt of pregnancy modification in a case of fetal Ebstein’s anomaly. Prenatal Cardiology. 2020;2019(1):56-60.
  20. Sharma T, Habash F, Mounsey J, Baker C, Candales AL. Ebstein’s Anomaly in Disguise: Follow the Cues and the Diagnosis Can Be Made. Cureus. 2020 Oct;12(10).
  21. Drenthen W, Pieper PG, Roos-Hesselink JW et al Outcome of pregnancy in women with congenital heart disease. J Am Coll Cardiol 2007;49:2303–2311.
  22. Ruys TPE, Roos-Hesselink JW, Hall R et al Heart failure in pregnant women with cardiac disease: data from the ROPAC. Heart 2014; 100:231–238.
  23. Kampman MM, Balci A, van Veldhuisen DJ et al N-terminal pro-B-type natriuretic peptide predicts cardiovascular complications in pregnant women with congenital heart disease. Eur Heart J 2014; 35:708–715 33.
  24. Tanous D, Siu SC, Mason J et al B-type natriuretic peptide in pregnant women with heart disease. J Am Coll Cardiol 2010; 56:1247–125.
  25. Susilowati E. Management of Pregnancy in 31-Year-Old Woman with Persistent Atrial Flutter After Cone Reconstruction for Ebstein’s Anomaly. Cardiovascular and Cardiometabolic Journal (CCJ). 2020 Mar 20;1(1):17-25.
  26. Holst KA, Connolly HM, Dearani JA. Ebstein's anomaly. Methodist DeBakey cardiovascular journal. 2019 Apr;15(2):138.
  27. Kochav J. Ebstein’s Anomaly of the Tricuspid Valve. InAdult Congenital Heart Disease in Clinical Practice 2018 (pp. 371-390). Springer, Cham.
  28. JCS Joint Working Group. Guidelines for indication and management of pregnancy and delivery in women with heart disease (JCS 2010): digest version. Circ J. 2012; 76: 240– 60.
  29. Holst KA, Dearani JA, Qureshi MY, Wackel P, Cannon BC, O’Leary PW, Olson TM, Seisler DK, Nelson TJ, Cavanaugh K, Wobig J. From safety to benefit in cell delivery during surgical repair of Ebstein anomaly: initial results. The Annals of Thoracic Surgery. 2021 Feb 1.
  30. Elzein C, Subramanian S, Ilbawi M. Surgical management of neonatal Ebstein’s anomaly associated with circular shunt. World Journal for Pediatric and Congenital Heart Surgery. 2019 Jan;10(1):116-20.
  31. Katamreddy A, Thachil R. Ebstein's anomaly in a tachycardic postpartum woman. Journal of the American College of Cardiology. 2019 Mar 12;73(9S1):2518.
  32. Evans E, McDonald M, Adamson DL, Khan JN. Cardiomyopathy in the peripartum period due to left ventricular non-compaction and association with Ebstein’s anomaly: a case report. European Heart Journal-Case Reports. 2021 Mar;5(3): ytab088.
  33. D duraisamy ka. Ebstein's anomaly in congenital rubella syndrome-a rare association-a case report. university journal of medicine and medical specialities. 2019 feb 9;5(1).
  34. Phi K, Martin DP, Beebe A, Klamar J, Tobias JD. Anesthetic Care During Posterior Spinal Fusion in an Adolescent with Ebstein’s Anomaly. Journal of Medical Cases. 2020 Mar 26;11(3):68-72.
Journal of Health Sciences & Surveillance System
Volume 10, Issue 3
July 2022
Pages 380-387

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