Modified cone procedure along with bidirectional cavopulmonary shunt for Ebstein anomaly in adult patients
Keywords:Ebstein anomaly, Adult congenital heart disease, Modified cone reconstruction, Bidirectional cavopulmonary shunt, Tricuspid annuloplasty ring
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.
Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Children. Macmillan Company, New York, New York, 1958;34.
Ebstein W: Ueber einen sehr seltenen fall von isufficienz der valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol. 1866;238-254.
da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg. 2007;133(1):215-23.
Silva JP, Silva Lda F, Moreira LF, Lopez LM, Franchi SM, Lianza AC, et al. Cone reconstruction in Ebstein’s anomaly repair: early and long-term results [in Portuguese]. Arq Bras Cardiol 2011;97(3):199-208.
Dearani JA, Bacha E, da Silva JP. Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Oper Tech Thoracic Cardiovasc Surg. 2008;13:109-25.
Sata S, Murin P, Hraska V. Cone reconstruction of Ebstein’s anomaly in a neonate. Ann Thorac Surg. 2012;94(4):e99-100.
Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Meyer JE, et al. Ebstein’s malformation of the tricuspid valve: short-term outcomes of the “cone procedure” versus conventional surgery. Congenit Heart Dis. 2012;7(1):50-8.
Dearani JA, Said SM, Burkhart HM, Pike RB, O’Leary PW, Cetta F. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. Ann Thorac Surg. 2013;96(1):202-10.
Dearani JA, Said SM, O’Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein’s malformation: lessons learned with cone reconstruction. Ann Thorac Surg. 2013;95(1):220-8.
Dearani JA, Danielson GK. Congenital heart surgery nomenclature and database project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69(4):106-17.
Lev M, Liberthson RR, Joseph RH. The pathologic anatomy of Ebstein’s disease. Arch Pathol. 1970;90:334.
Celermajer DS, Bull C, Till JA. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23:170-6.
Dearani JA, Danielson GK. Surgical Management of Ebstein’s Anomaly in the Adult. Sem Thorac Cardiovasc Surg. 2005;17(2):148-54.
Anderson HN, Dearani JA, Said SM, Norris MD, Pundi KN, Miller AR, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014;9(3):266-71.