International Surgery Journal | August 2022 | Vol 9 | Issue 8 Page 1 International Surgery Journal Sharma R et al. Int Surg J. 2022 Aug;9(8):xxx-xxx http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 Case Series Modified cone procedure along with bidirectional cavopulmonary shunt for Ebstein anomaly in adult patients Rakesh Sharma*, Satyajit Samal, Rahul Banik, Saket Aggarwal, Sayyed Ehtesham Hussain Naqvi, Mohammed Abid Geelani INTRODUCTION Ebstein anomaly is a rare congenital malformation affecting the tricuspid valve and right ventricle and accounts for less than 1% of all congenital heart diseases. 1 Wilhelm Ebstein, a German pathologist, first described this anomaly in 1866. 2 Asymptomatic patients with stable hemodynamic pass on to adulthood and are diagnosed later as disease progresses and require surgical treatment. After many unsuccessful attempts at repair, da Silva described the cone reconstruction technique. 3,4 Modifications of this technique have been used by Dearani and others and it has become the preferred repair method for patients with Ebstein anomaly. 5-9 Here we described the surgical technique and the results of modified cone reconstruction along with bidirectional Glenn shunt used for the treatment of Ebstein anomaly in 5 adult patients at a tertiary care referral centre in North India. CASE SERIES All adult patients aged more than 18 years referred to us with Ebstein anomaly from 2019 to 2021 were included in the study. A total of 5 patients, all females underwent the corrective surgery after a detailed clinical examination, laboratory and echocardiographic evaluation. They presented with symptoms of dyspnoea ABSTRACT Ebstein anomaly is an uncommon congenital cardiac disease affecting the tricuspid valve and right ventricle. Da silva pioneered the cone reconstruction of tricuspid valve for surgical repair of this anomaly in 2007. We describe our experience of 5 adult patients who underwent cone repair along with a bidirectional cavopulmonary shunt and tricuspid annuloplasty ring placement at our institute. Five adult patients with Ebstein anomaly with progressive disease and symptoms were taken up cone reconstruction from 2019 to 2021. Along with cone reconstruction of tricuspid valve, we routinely added an end to side superior vena cava to right pulmonary artery shunt and a tricuspid annuloplasty ring in the newly formed tricuspid annulus in all cases. All patients tolerated the procedure well and easily weaned off cardiopulmonary bypass. There were no complications regarding bidirectional cavopulmonary shunt and only one patient had a temporary heart block which was managed conservatively. This series shows routine addition of a cavopulmonary shunt and an annuloplasty ring improves outcomes and can be performed at other experienced cardiac surgery centres. Keywords: Ebstein anomaly, Adult congenital heart disease, Modified cone reconstruction, Bidirectional cavopulmonary shunt, Tricuspid annuloplasty ring Department of Cardiothoracic Surgery, G. B. Pant Institute of Postgraduate Medical Education and Research (GIPMER), New Delhi, India Received: 20 June 2022 Revised: 02 July 2022 Accepted: 05 July 2022 *Correspondence: Dr. Rakesh Sharma, E-mail: raksy_222@yahoo.co.in Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: https://dx.doi.org/10.18203/2349-2902.isj20221825