Commentary: Never let the truth get in the way of a good story

Central MessageManagement of neonatal Ebstein's anomaly remains elusive and varies based on presentation, morphology, and experience. A staged Starnes procedure followed by cone repair is a valid strategy in selected patients.See Article page 281. Management of neonatal Ebstein's anomaly remains elusive and varies based on presentation, morphology, and experience. A staged Starnes procedure followed by cone repair is a valid strategy in selected patients. See Article page 281. In this issue of the Journal, Da Silva and colleagues1da Silva J.P. Viegas M. Castro-Medina M. da Silva L.D.F. The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: new surgical strategy and initial results.J Thorac Cardiovasc Surg Tech. 2020; 3: 281-283Google Scholar describe 2 children with Ebstein's anomaly who had initially undergone a neonatal Starnes procedure (right ventricular exclusion with fenestrated tricuspid valve patch and modified Blalock–Taussig shunt) and subsequently underwent takedown of the patch and cone repair of the tricuspid valve plus right ventricular outflow tract obstruction relief, thus converting them to biventricular repair (1 patient at age 5 months) and one-and-a-half ventricular repair (1 patient at age 17 months after an intermediary Glenn shunt). The authors concluded that this sequence of initial single ventricle palliation and subsequent tricuspid valve repair is a viable strategy for treating neonates with Ebstein's anomaly and severe heart failure. Ebstein's anomaly is a heterogeneous complex congenital heart disease that involves the tricuspid valve and the right ventricle, with the presentation varying based on the severity of the disease. Dr José Pedro Da Silva is credited for introducing the cone repair, with reports of excellent early and late outcomes.2da Silva J.P. Baumgratz J.F. da Fonseca L. Franchi S.M. Lopes L.M. Tavares G.M.P. 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: 215-223Abstract Full Text Full Text PDF PubMed Scopus (175) Google Scholar Subsequently, many surgeons adopted cone repair as the procedure of choice for Ebstein's anomaly. Nonetheless, the majority of patients who underwent cone repair in the early experience of Da Silva and others were older children and young adults, whereas cone repair in very small children has been rarely described.2da Silva J.P. Baumgratz J.F. da Fonseca L. Franchi S.M. Lopes L.M. Tavares G.M.P. 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: 215-223Abstract Full Text Full Text PDF PubMed Scopus (175) Google Scholar,3Holst K.A. Dearani J.A. Said S. Pike R.B. Connolly H.M. Cannon B.C. et al.Improving results of surgery for Ebstein anomaly: where are we after 235 cone repairs?.Ann Thorac Surg. 2018; 105: 160-168Abstract Full Text Full Text PDF PubMed Scopus (36) Google Scholar The current contribution from Da Silva and colleagues demonstrates the following: (1) cone repair is effective in neonates and small infants with Ebstein's anomaly, analogous to some reports from Asia4Mizuno M. Hoashi T. Sakaguchi H. Kagisaki K. Kitano K. Kurosaki K. et al.Application of cone reconstruction for neonatal Ebstein anomaly or tricuspid valve dysplasia.Ann Thorac Surg. 2016; 101: 1811-1817Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar,5Huang S.C. Wu E.T. Chen S.J. Huang C.-H. Shih J.C. Chou H.-W. et al.Surgical strategy toward biventricular repair for severe Ebstein anomaly in neonates and infancy.Ann Thorac Surg. 2017; 104: 917-925Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar; and (2) cone repair can be performed following the Starnes procedure. Although tricuspid valve repair has been previously described following the Starnes procedure, this current report might be the first describing cone repair in that setting.6Kumar T.K.S. Boston U.S. Knott-Craig C.J. Neonatal Ebstein anomaly.Semin Thorac Cardiovasc Surg. 2017; 29: 331-337Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar This is valuable information, given the evidence that right ventricular exclusion leads to ventricular regression.7Reemtsen B.L. Polimenakos A.C. Fagan B.T. Wells W.J. Starnes V.A. Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly.J Thorac Cardiovasc Surg. 2007; 134: 1406-1410Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar The window for performing biventricular repair after right ventricular exclusion remains unclear. The issue of whether or not to proceed with a primary neonatal cone repair versus initial single ventricle Starnes procedure followed by cone repair is also unresolved. Although neonatal cone repair might be technically feasible, Ebstein's anomaly involves the right ventricle as well, and the dysfunctional right ventricle might not be able to generate sufficient pressure to open the pulmonary valve in neonates with severe Ebstein's anomaly, favoring initial single ventricle palliation. The criteria for selecting candidates for primary neonatal repair or for eventual biventricular repair remain to be determined and are likely linked to clinical presentation, heart morphology, and institutional experience and philosophy towards this complex disease. This report of a staged approach offers a valid strategy in neonates with significant heart failure as a result of severe Ebstein's anomaly.1da Silva J.P. Viegas M. Castro-Medina M. da Silva L.D.F. The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: new surgical strategy and initial results.J Thorac Cardiovasc Surg Tech. 2020; 3: 281-283Google Scholar Although adding another treatment strategy for a difficult problem to the surgeon's armamentarium is a cause for celebration, unfortunately, the reality is less appealing. First, there are substantial variances in the management strategies of neonates with severe Ebstein's anomaly: some centers (eg, Toronto, Melbourne) have reported successful conservative medical management in the majority of patients.8Wald R.M. Adatia I. Van Arsdell G.S. Hornberger L.K. Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly.Am J Cardiol. 2005; 96: 851-856Abstract Full Text Full Text PDF PubMed Scopus (56) Google Scholar,9Luxford J.C. Arora N. Ayer J.G. Verrall C.E. Cole A.D. Orr Y. et al.Neonatal Ebstein anomaly: a 30-year institutional review.Semin Thorac Cardiovasc Surg. 2017; 29: 206-212Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar When intervention is necessary, some centers are more likely to implement a single ventricle palliation strategy (eg, Los Angeles, Michigan), whereas a few applied primary biventricular repair in the majority of patients (eg, Memphis, Osaka, Taipei).4Mizuno M. Hoashi T. Sakaguchi H. Kagisaki K. Kitano K. Kurosaki K. et al.Application of cone reconstruction for neonatal Ebstein anomaly or tricuspid valve dysplasia.Ann Thorac Surg. 2016; 101: 1811-1817Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar, 5Huang S.C. Wu E.T. Chen S.J. Huang C.-H. Shih J.C. Chou H.-W. et al.Surgical strategy toward biventricular repair for severe Ebstein anomaly in neonates and infancy.Ann Thorac Surg. 2017; 104: 917-925Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar, 6Kumar T.K.S. Boston U.S. Knott-Craig C.J. Neonatal Ebstein anomaly.Semin Thorac Cardiovasc Surg. 2017; 29: 331-337Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar,10Kumar S.R. Kung G. Noh N. Castillo N. Fagan B. Wells W.J. et al.Single-ventricle outcomes after neonatal palliation of severe Ebstein anomaly with modified Starnes procedure.Circulation. 2016; 134: 1257-1264Crossref PubMed Scopus (10) Google Scholar,11Shinkawa T. Polimenakos A.C. Gomez-Fifer C.A. Charpie J.R. Hirsch J.C. Devaney E.J. et al.Management and long-term outcome of neonatal Ebstein anomaly.J Thorac Cardiovasc Surg. 2010; 139: 354-358Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar These variations again highlight the differences in experience and approach to this complex disease and the knowledge gap that exists, prohibiting a consensus uniform tactic for managing these complicated neonates. Second, operative mortality following the diverse treatment strategies of neonatal Ebstein's anomaly continues to be higher than desired. One of the many contributions of Dr Vaughn Starnes to our field include the single ventricle right ventricular exclusion principle that provided improved survival in these patients; however, mortality continues to be among the highest in our specialty: 5 of 27 (19%) in the experience from Los Angeles (only 13% with patch fenestration).10Kumar S.R. Kung G. Noh N. Castillo N. Fagan B. Wells W.J. et al.Single-ventricle outcomes after neonatal palliation of severe Ebstein anomaly with modified Starnes procedure.Circulation. 2016; 134: 1257-1264Crossref PubMed Scopus (10) Google Scholar Similarly, reported mortality following neonatal tricuspid valve repair for Ebstein's anomaly is high, up to 75%; in the experience of Dr Knott-Craig from Memphis, mortality was 28%, although it was lower in those without pulmonary atresia and those with pulmonary atresia who underwent a right ventricle–to–pulmonary artery conduit.6Kumar T.K.S. Boston U.S. Knott-Craig C.J. Neonatal Ebstein anomaly.Semin Thorac Cardiovasc Surg. 2017; 29: 331-337Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar In a recent review of the Society of Thoracic Surgeons database, tricuspid valve repair was the most common neonatal intervention (40%; mortality, 34%), followed by aortopulmonary shunt (20%; mortality, 17%) and the Starnes procedure (9%; mortality, 25%); again highlighting variations in strategies and continuous high mortality.12Holst K.A. Dearani J.A. Said S.M. Davies R.R. Pizarro C. Knott-Craig C. et al.Surgical management and outcomes of Ebstein anomaly in neonates and infants: a Society of Thoracic Surgeons congenital heart surgery database analysis.Ann Thorac Surg. 2018; 106: 785-791Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar In summary, neonatal management of Ebstein's anomaly remains elusive, and proper candidates for conservative medical management and single or biventricular repair are not well identified. Management varies based on morphology, clinical presentation, and institutional experience; however, overall early mortality remains high for all treatment strategies. A staged approach that includes an initial single ventricle Starnes procedure followed by conversion to biventricular repair is feasible and might be considered in selected neonates with intractable heart failure that is not responsive to conservative management. The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: New surgical strategy and initial resultsJTCVS TechniquesVol. 3PreviewNeonates with severe Ebstein's anomaly (EA) represent a surgical challenge. The Starnes procedure has been used as effective surgical palliation for these critically ill patients.1 This strategy decompresses the right ventricle, allowing the left ventricle to assume a globular shape, improving function.2 Kumar and associates3 reported mid-term survival of 81% at 10 years by committing patients to the single-ventricle pathway after the Starnes procedure.3 However, this strategy exposes these patients to long-term complications associated with Fontan palliation. Full-Text PDF Open Access

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