The Mid-Term Outcomes of Cone Repair or Replacement of Tricuspid Valve in Patients with Ebstein's Anomaly: Our Experience

Authors

  • Quamrul Islam Talukder, MBBS, FCPS, MD, FCVS Department of Cardiac Surgery, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • Saikat DasGupta, MBBS, MS Department of Cardiac Surgery, Square Hospitals Limited, West, Panthapath, Dhaka-1205, Bangladesh
  • Abdus Samad, MBBS, DA Department of Cardiac Anesthesia, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • Musfeq-Us-Saleheen Khan, MBBS, MS Department of Cardiac Surgery, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • Ziaur Rahman, MBBS, MS Department of Cardiac Surgery, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • Mohammad Kabiruzzaman, MD, FNIC, AFACC Department of Cardiology, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • ATM Khalilur Rahman, MBBS, DA, FCA Department of Cardiac Anesthesia, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh
  • Prof. Farooque Ahmed, MBBS, MS Department of Cardiac Surgery, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh

DOI:

https://doi.org/10.1532/hsf.4165

Keywords:

Ebstein's Anomaly, Cone Repair, Tricuspid Valve Repair / Replacement

Abstract

Background: Cone repair of the tricuspid valve (TV) is a contemporary reproducible technique for surgical reconstruction of Ebstein's anomaly. Different authorities have shown that this technique restores excellent tricuspid valve function. In Bangladesh, this technique still is unfamiliar to many. We hereby present a case series of cone repair and TV replacement with the mid-term outcome (one year to six years) at the National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh.

Methods: We prospectively studied 21 patients, who underwent surgical intervention (cone repair or tricuspid valve replacement) for Ebstein's anomaly of TV from March 2014 to June 2020. We divided the total patient population into the cone repair and TV replacement groups. Preoperative, postoperative, and follow-up data were collected from the hospital records, telephone conversations, and clinic visits. All collected data statistically were analyzed.

Results: Our patients showed there were statistically significant improvements after surgical intervention with regard to tricuspid regurgitation (TR) (P < 0.001), tricuspid annular plane systolic excursion (TAPSE) (P < 0.001), right ventricular (RV) function (P < 0.001), and New York Heart Association (NYHA) class (P < 0.001). These developments were sustained throughout the follow-up period.

Conclusion: Cone repair should be offered to the symptomatic patients of Ebstein's anomaly because symptoms relief, reduction of morbidity, and survival benefits are excellent. Above all, the cone reconstruction shows fantastic results and may well become the surgical technique for patients with Ebstein’s anomaly. We hope that new valve repair programs may provide extended longevity and restored quality of life to the patient of Ebstein's anomaly (EA) with the appropriate measures. In case of failed repair, valve replacement is an encouraging option.

References

Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Ammash NM, Dearani JA. 2014. Increased risk of possible paradoxical embolic events in adults with Ebstein anomaly and severe tricuspid regurgitation. Congenit Heart Dis. 9:30–7.

Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, Dearani JA. 2012. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol. 59:2101–6.

Attenhofer Jost CH, Edmister WD, Julsrud PR, et al. 2012. Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging. 28:1147-59.

Badiu CC, Schreiber C, Hörer J, et al. 2010. Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome. Eur J Cardiothorac Surg. 37(1):186-192.

Benson DW, Silberbach GM, Kavanaugh-McHugh A, et al. 1999. Mutations in the cardiac transcription factor NKX2.5 affect diverse cardiac developmental pathways. J Clin Invest. 104(11):1567-1573.

Beroukhim RS, Jing L, Harrild DM, Fornwalt BK, Mejia-Spiegeler A, Rhodes J, et al. 2018. Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson. 20(1):32.

Brown ML, Dearani JA, Danielson GK, et al. 2008. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. 135(5):1120-1136.e11367.

Chauvaud SM, Hernigou AC, Mousseaux ER, Sidi D, Hebert JL. 2006. Ventricular volumes in Ebstein’s anomaly: X-ray multislice computed tomography before and after repair. Ann Thorac Surg. 81:1443–9.

Das Gupta S, Uddin M, Howlader SS, Kumar Biswas P, Kabiruzzaman M, Islam Talukder MQ. 2020. Surgical management for an adult, female patient, with Ebstein Anomaly on Tricuspid Valve that has Subvalvular membrane with severe PS and multiple VSDs-A case report. Heart Surg Forum. 23(6):E781-E785.

Dearani JA, Mora BN, Nelson TJ, Haile DT, O'Leary PW. 2015. Ebstein anomaly review: what's now, what's next? Expert Rev Cardiovasc Ther. 13(10):1101-1109.

Erin Fender, Chad J. Zack, Alexander Egbe. 2018. National trends and outcomes for surgical treatment of Ebstein’s anomaly. Mayo Clinic J Am Coll Cardiol. Mar, 71 (11 Supplement) A618.

Ghio S, Recusani F, Klersy C, et al. 2000. Prognostic usefulness of the tricuspid annular plane systolic excursion in patients with congestive heart failure secondary to idiopathic or ischemic dilated cardiomyopathy. Am J Cardiol. 85:837-42.

Goleski PJ, Sheehan FH, Chen SS, Kilner PJ, Gatzoulis MA. 2014. The shape and function of the left ventricle in Ebstein's anomaly. Int J Cardiol. 171(3):404-12.

Holst KA, Dearani JA, Said S, Pike RB, Connolly HM, Cannon BC, et al. 2018. Improving results of surgery for Ebstein anomaly: where are we after 235 cone repairs? Ann Thorac Surg. 105(1):160-8.

Hunter SW, Lillehei CW. 1958. Ebstein malformation of the tricuspid valve. Study of a case together with suggestions of a new form of surgical therapy. Dis Chest. 33(3):297-304.

Kaul S, Tei C, Hopkins JM, et al. 1984. Assessment of right ventricular function using two-dimensional echocardiography. Am Heart J. 107:526-31.

Lang RM, Bierig M, Devereux RB, et al. 2005. Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr. 18:1440-63.

Lange R, Burri M, Eschenbach LK, Badiu CC, da Silva JP, Nagdyman N, et al. 2015. Da Silva’s cone repair for Ebstein’s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg. 48(2):316-21; discussion 320-1.

Li X, Wang SM, Schreiber C, Cheng W, Lin K, Sun JY, et al. 2016. More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol. 206:131-7.

Liu J, Qiu L, Zhu Z, Chen H, Hong H. 2011. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg. 141(5):1178-83.

Malhotra SP, Petrossian E, Reddy VM, Qiu M, Maeda K, Suleman S, et al. 2009. Selective right ventricular unloading and novel technical concepts in Ebstein’s anomaly. Ann Thorac Surg. 88:1975–81.

Mrad Agua K, Burri M, Cleuziou J, et al. 2019. Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. Eur J Cardiothorac Surg. 55(6):1187-1193.

Postma AV, van Engelen K, van de Meerakker J, et al. 2011. Mutations in the sarcomere gene MYH7 in Ebstein anomaly. Circ Cardiovasc Genet. 4(1):43-50.

Raju V, Dearani JA, Burkhart HM, et al. 2014. Right ventricular unloading for heart failure related to Ebstein malformation. Ann Thorac Surg. 98:167-73; discussion 173-4.

Said SM, Burkhart HM, Schaff HV, et al. 2014. When should a mechanical tricuspid valve replacement be considered? J Thorac Cardiovasc Surg. 148:603-8.

Saxena A, Relan J, Agarwal R, et al. 2019. Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases. Ann Pediatr Cardiol. 12(3):254-286.

Silva GVRD, Miana LA, Caneo LF, et al. 2019. Early and Long-Term Outcomes of Surgical Treatment of Ebstein's Anomaly. Braz J Cardiovasc Surg. 34(5):511-516. Published Dec 1.

Silva JP, Baumgratz JF, Fonseca Ld, et al. 2004. Anomalia de Ebstein: resultados com a reconstrução cônica da valva tricúspide [Ebstein's anomaly: results of the conic reconstruction of the tricuspid valve]. Arq Bras Cardiol. 82(3):212-216.

Taggart NW, Cabalka AK, Eicken A, et al. 2018. Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly. Am J Cardiol. 121:262-8.

Published

2022-01-17

How to Cite

Talukder, Q. I., DasGupta, S., Samad, A., Khan, M.-U.-S., Rahman, Z., Kabiruzzaman, M., Rahman, A. K., & Ahmed, F. (2022). The Mid-Term Outcomes of Cone Repair or Replacement of Tricuspid Valve in Patients with Ebstein’s Anomaly: Our Experience. The Heart Surgery Forum, 25(1), E030-E036. https://doi.org/10.1532/hsf.4165

Issue

Section

Article