Successful surgery of Ebstein’s anomaly of 11-year-old patient after having had two cardiac arrests

Abstract

Ebstein’s anomaly is a rarely seen congenital heart disease with high morbidity and mortality rates. Impaired function of tricuspid valve and partially atrialized right ventricle are the main characteristics of the disease. Patients may have symptom-free survival for long terms whereas intra uterine death may also be encountered due to wide range of the symptoms. Regurgitation of the tricuspid valve and right-sided heart failure are the foremost symptoms. Surgery must be performed in symptomatic patients without delay. Carpentier’s approach to the surgical treatment of Ebstein’s anomaly is one of the methods that can be applicable. In this study it is reported that successful surgical treatment of 11-year-old child with Ebstein’s anomaly who had two cardiac arrests before operation, by using Carpentier’s technique.

Keywords

• Carpentier's techniuqe
• Ebstein's anomaly
• Surgery

Ebstein anomalisi olan ve iki kez kardiyak arrest geçiren 11 yaşındaki hastanın başarılı cerrahisi

Abstract

Ebstein anomalisi, yüksek morbidite ve mortalite oranlarına sahip, nadir görülen bir doğuştan kalp hastalığıdır. Triküspit kapağın işlev bozukluğu ve kısmen atriyalize olmuş sağ ventrikül, hastalığın temel özellikleridir. Hastalar uzun süreli semptomsuz sağkalıma sahip olabilirken, semptomların geniş bir yelpazesi nedeniyle rahim içi ölümle de karşılaşılabilir. Triküspit kapağın yetersizliği ve sağ kalp yetmezliği en başta gelen semptomlardır. Semptomatik hastalarda ameliyat gecikmeden yapılmalıdır. Carpentier’in Ebstein anomalisinin cerrahi tedavisine yaklaşımı, uygulanabilecek yöntemlerden biridir. Bu çalışmada, ameliyattan önce iki kez kardiyak arrest geçiren Ebstein anomalili 11 yaşındaki bir çocuğun Carpentier tekniği ile başarılı cerrahi tedavisi bildirildi.

Keywords

• Carpentier Tekniği
• Ebstein Anomalisi
• Cerrahi

References

1. Nguyen HS, Vu TD, Nguyen TQ. A Modified Carpenter’s Technique for Ebstein’s Anomaly Repair. J Card Surg. 2014; 29:554- 560. https://doi.org/10.1111/jocs.12364.
2. Shinkawa T, Polimenakos AC, Gomez-Fifer CA, Charpie JR, Hirsc JC, Devaney EJ. Management and Long-term Outcome of Neonatal Ebstein Anomaly. J Thorac Card Surg. 2010; 39(2):354- 358. https://doi.org/10.1016/j.jtcvs.2009.07.062.
3. Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s Anomaly. Circulation.2007;115:277-285. https://doi. org/10.1161/CIRCULATIONAHA.106.619338.
4. Holst KA, Connolly HM, Dearani JA. Ebstein’s Anomaly. Methodist Debakey Cardiovasc J. 2019; 15(2): 138-144. https://doi.org/10.14797/mdjc-15-2-138.
5. Jain N, Saran DP, Yadav SC, Dwivedi S. Ebstein’s anomaly with recurrent massive pericardial effusion: a rare presentation. BMJ Case Rep. 2016; 2016: bcr2016216176. https://doi. org/10.1136//bcr-2016-216176
6. Yuan SM. Ebstein’s Anomaly: Genetics, Clinical Manifestations, and Management. Pediatrics and Neonatology.2017; 58: 211-215. https://doi.org/10.1016/j.pedneo.2016.08.004.
7. Burri M, Lange R. Surgical Treatment of Ebstein’s Anomaly. Thorac Cardiovasc Surg. 2017; 65:639-648. https://doi. org/10.1055/s-0037-1604469.
8. Hunter SW, Lillehei CW. Ebstein malformation of the tricuspid valve.Study of a case together with suggestions of a new form of surgicaltherapy. Dis Chest. 1958;33(3):297-304. https://doi. org/10.1378/chest.33.3.297.

9. Carpentier A, Chauvaud S, Macé L, Relland J, Mihaileanu S, Marino JP, etal. A new reconstructive operation for Ebstein’s anomaly of the tricuspidvalve. J Thorac Cardiovasc Surg. 1988;96(1):92-101.
10. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr.Operative treatment of Ebstein anomaly. J Thorac Cardiovasc Surg.1992;104(5):1195-202.
11. Da Silva JP, Baumgratz JF, Fonseca L, Afiune JY, Franchi SM, Lopes LM,et al. Ebstein’s anomaly. Results of the conical reconstruction of thetricuspid valve. Arq Bras Cardiol. 2004;82(3):217-20. https://doi.org/10.1590/S0066-782X2004000300002.
12. Da Silva GVR,Miana LA, Caneo LF, Turquetto AL, Tanamati C, Penha JG, et al. Early and Long-Term Outcomes of Surgical Treatment of Ebstein’s Anomaly. Braz J Cardiovasc Surg. 2019; 34(5): 511-516. https://doi.org/10.21470/1678-9741-2018-0333.
13. Arnaz A, Doğan A, Oktay A, Yalçınbaş Y, Türköz R, Saygılı A, ve ark. Ebstein Anomalisi; Cerrahi Yaklaşım ve Orta Dönem Sonuçları. GMJ. 2018;29:323-326. https://doi.org/10.12996/gmj.2018.87.
14. Nagdyman N, Ewert P, Komoda T, Meskisvilli VA, Weng Y, Berger F, et al. Modified Repair in Patient’s with Ebstein’s Anomaly. J Heart Valve Dis. 2010;19(3): 364-370.
15. İpek G, Kırali K, Rabuş MB, Ömeroğlu SN, Erdoğan HB, Göksedef D, ve ark. Turk Kardiol Dern Ars. 2001;29(7):420-425.
16. Werf C, Zwinderman AH, Wilde AA. Therapeutic approach for patients with catecholaminergic polymorphic ventricular tachycardia: state of the art and future developments. Europace 2012;14:175-183.