Abstract
The combination of double outlet right ventricle and complete atrioventricular septal defect (DORV-AVSD) is a rare congenital heart malformation. Double outlet right ventricle with complete atrioventricular septal defect is commonly associated with heterotaxy but is distinct from DORV as associated with tetralogy of Fallot. Biventricular repair of DORV-AVSD is challenging because it requires not only correction of the complete AVSD and DORV, but often also involves resection of the outlet portion of the ventricular septum to create an unobstructed left ventricular outflow tract as well as repair of the frequently associated systemic and pulmonary venous connection anomalies and right ventricular outflow tract obstruction. Because of this high degree of complexity, functional single ventricle palliation has been recommended as a treatment option for DORV-AVSD by some groups. However, biventricular repair in these patients, despite its complexity, can be accomplished with excellent results. Reoperation is common in this difficult group of patients, most commonly for conduit replacement. Reintervention for recurrent left ventricular outflow tract obstruction or atrioventricular valve stenosis or regurgitation is uncommon. We feel that biventricular repair of DORV-AVSD is the preferred treatment option, although further studies are needed to define long-term outcomes.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Zhao QM, Ma XJ, Jia B, Huang GY. Prevalence of congenital heart disease at live birth: an accurate assessment by echocardiographic screening. Acta Paediatr. 2013;102:397–402.
Suzuki T, Bove EL, Devaney EJ, et al. Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 2008;86:596–602.
Bradley TJ, Karamlou T, Kulik A, et al. Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle. J Thorac Cardiovasc Surg. 2007;134:967–73 e6.
Bakhtiary F, Takacs J, Cho MY, et al. Long-term results after repair of complete atrioventricular septal defect with two-patch technique. Ann Thorac Surg. 2010;89:1239–43.
Bogers AJ, Akkersdijk GP, de Jong PL, et al. Results of primary two-patch repair of complete atrioventricular septal defect. Eur J Cardiothorac Surg. 2000;18:473–9.
Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. 2006;29:545–50.
Bharati S, Kirklin JW, McAllister Jr HA, Lev M. The surgical anatomy of common atrioventricular orifice associated with tetralogy of Fallot, double outlet right ventricle and complete regular transposition. Circulation. 1980;61:1142–9.
Sridaromont S, Feldt RH, Ritter DG, Davis GD, Mcgoon DC, Edwards JE. Double-outlet right ventricle associated with persistent common atrioventricular-canal. Circulation. 1975;52:933–42.
Pacifico AD, Kirklin JW, Bargeron Jr LM. Repair of complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle: report of 10 patients. Ann Thorac Surg. 1980;29:351–6.
He GW, Mee RB. Complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: a report of successful surgical treatment. Ann Thorac Surg. 1986;41:612–5.
Raju V, Burkhart HM, Rigelman Hedberg N, et al. Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle. Ann Thorac Surg. 2013;95:2079–84; discussion 84–5.
Devaney EJ, Lee T, Gelehrter S, et al. Biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle. Ann Thorac Surg. 2010;89:537–42; discussion 42–3.
Anderson RH, Wessels A, Vettukattil JJ. Morphology and morphogenesis of atrioventricular septal defect with common atrioventricular junction. World J Pediatr Congenit Heart Surg. 2010;1:59–67.
Anderson RH, Ho SY, Falcao S, Daliento L, Rigby ML. The diagnostic features of atrioventricular septal defect with common atrioventricular junction. Cardiol Young. 1998;8:33–49.
Mahle WT, Martinez R, Silverman N, Cohen MS, Anderson RH. Anatomy, echocardiography, and surgical approach to double outlet right ventricle. Cardiol Young. 2008;18 Suppl 3:39–51.
Anderson RH, Ho SY, Wilcox BR. The surgical anatomy of ventricular septal defect part IV: double outlet ventricle. J Card Surg. 1996;11:2–11.
Anderson RH, Becker AE, Wilcox BR, Macartney FJ, Wilkinson JL. Surgical anatomy of double-outlet right ventricle – a reappraisal. Am J Cardiol. 1983;52:555–9.
Ong J, Brizard CP, d’Udekem Y, et al. Repair of atrioventricular septal defect associated with tetralogy of Fallot or double-outlet right ventricle: 30 years of experience. Ann Thorac Surg. 2012;94:172–8.
Jacobs JP, Anderson RH, Weinberg PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young. 2007;17 Suppl 2:1–28.
Anderson RH, Webb S, Brown NA. Defective lateralisation in children with congenitally malformed hearts. Cardiol Young. 1998;8:512–31.
Karl TR. Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations. Semin Thorac Cardiovasc Surg. 1997;9:26–34.
Tchervenkov CI, Hill S, Del Duca D, Korkola S. Surgical repair of atrioventricular septal defect with common atrioventricular junction when associated with tetralogy of Fallot or double outlet right ventricle. Cardiol Young. 2006;16 Suppl 3:59–64.
Caffarena JM, Gomez-Ullate JM. Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure. Interact Cardiovasc Thorac Surg. 2005;4:200–2.
Ruzmetov M, Rodefeld MD, Turrentine MW, Brown JW. Rational approach to surgical management of complex forms of double outlet right ventricle with modified Fontan operation. Congenit Heart Dis. 2008;3:397–403.
Russo P, Danielson GK, Puga FJ, McGoon DC, Humes R. Modified Fontan procedure for biventricular hearts with complex forms of double-outlet right ventricle. Circulation. 1988;78:III20–5.
Takeuchi K, McGowan Jr FX, Bacha EA, et al. Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. Ann Thorac Surg. 2006;82:146–52.
Aoki M, Forbess JM, Jonas RA, Mayer Jr JE, Castaneda AR. Result of biventricular repair for double-outlet right ventricle. J Thorac Cardiovasc Surg. 1994;107:338–49; discussion 49–50.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Si, MS., Ohye, R.G., Hirsch-Romano, J.C., Bove, E.L. (2016). Biventricular Repair of Double Outlet Right Ventricle with Complete Atrioventricular Septal Defect. In: Lacour-Gayet, F., Bove, E., Hraška, V., Morell, V., Spray, T. (eds) Surgery of Conotruncal Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-23057-3_24
Download citation
DOI: https://doi.org/10.1007/978-3-319-23057-3_24
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-23056-6
Online ISBN: 978-3-319-23057-3
eBook Packages: MedicineMedicine (R0)