Congenital: Atrioventricular Septal Defect| Volume 165, ISSUE 2, P424-433, February 2023

Atrioventricular septal defect in Fontan circulation: Right ventricular dominance, not valve surgery, adversely affects survival



      The effect of ventricular dominance and previous atrioventricular valve (AVV) surgery on patient outcomes after Fontan operation remains unclear. We sought to determine the effect of ventricular dominance and previous AVV surgery on transplantation-free survival and long-term AVV competency in patients with atrioventricular septal defect (AVSD) and Fontan circulation.


      We conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021.


      Of 174 patients with AVSD, 60% (105/174) had right ventricular (RV) dominance and 40% (69/174) had left ventricular (LV) dominance. The cumulative incidence of moderate or greater AVV regurgitation at 25 years after Fontan operation in patients with LV dominance was 56% (95% CI, 35%-72%), compared with 54% (95% CI, 40%-67%) in patients with RV dominance (P = .6). Nonetheless, transplantation-free survival at 25 years in patients with LV dominance was 94% (95% CI, 86%-100%), compared with 67% (95% CI, 52%-87%) in patients with RV dominance (hazard ratio, 5.9; 95% CI, 1.4-25.4; P < .01). Of note, transplantation-free survival was not different in patients who underwent AVV surgery before or at Fontan completion compared with those who did not (15 years: 81% [95% CI, 62%-100%] vs 88% [95% CI, 81%-95%]; P = .3).


      In patients with AVSD and Fontan circulation the rate of moderate or greater common AVV regurgitation is similar in those with LV and RV dominance. RV dominance, rather than previous AVV surgery, is a risk factor for death or transplantation.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      AVSD (atrioventricular septal defect), AVV (atrioventricular valve), HR (hazard ratio), LV (left ventricular), RV (right ventricular)
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        • Buratto E.
        • Ye X.T.
        • King G.
        • Shi W.Y.
        • Weintraub R.G.
        • d'Udekem Y.
        • et al.
        Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought.
        J Thorac Cardiovasc Surg. 2017; 153: 430-438
        • Arrigoni S.C.
        • Ijsselhof R.
        • Postmus D.
        • Vonk Y.M.
        • François K.
        • Bové T.
        • et al.
        Long-term outcomes of atrioventricular septal defect and single ventricle: a multicenter study.
        J Thorac Cardiovasc Surg. 2022; 163: 1166-1175
        • King G.
        • Gentles T.L.
        • Winlaw D.S.
        • Cordina R.
        • Bullock A.
        • Grigg L.E.
        • et al.
        Common atrioventricular valve failure during single ventricle palliation.
        Eur J Cardiothorac Surg. 2017; 51: 1037-1043
        • King G.
        • Ayer J.
        • Celermajer D.
        • Zentner D.
        • Justo R.
        • Disney P.
        • et al.
        Atrioventricular valve failure in Fontan palliation.
        J Am Coll Cardiol. 2019; 73: 810-822
        • Moon J.
        • Shen L.
        • Likosky D.S.
        • Sood V.
        • Hobbs R.D.
        • Sassalos P.
        • et al.
        Relationship of ventricular morphology and atrioventricular valve function to long-term outcomes following Fontan procedures.
        J Am Coll Cardiol. 2020; 76: 419-431
        • Oster M.E.
        • Knight J.H.
        • Suthar D.
        • Amin O.
        • Kochilas L.K.
        Long-term outcomes in single-ventricle congenital heart disease.
        Circulation. 2018; 138: 2718-2720
        • Buratto E.
        • Ye X.T.
        • Brizard C.P.
        • Brink J.
        • d’Udekem Y.
        • Konstantinov I.E.
        Successful atrioventricular valve repair improves long-term outcomes in children with unbalanced atrioventricular septal defect.
        J Thorac Cardiovasc Surg. 2017; 154: 2019-2027
        • Iyengar A.J.
        • Winlaw D.S.
        • Galati J.C.
        • Gentles T.L.
        • Weintraub R.G.
        • Justo R.N.
        • et al.
        The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry.
        Intern Med J. 2014; 44: 148-155
        • Kawahira Y.
        • Kishimoto H.
        • Kawata H.
        • Ikawa S.
        • Ueda H.
        • Nakajima T.
        • et al.
        Morphologic analysis of common atrioventricular valves in patients with right atrial isomerism.
        Pediatr Cardiol. 1997; 18: 107-111
        • Stein J.I.
        • Smallhorn J.F.
        • Coles J.G.
        • Williams W.G.
        • Trusler G.A.
        • Freedom R.M.
        Common atrioventricular valve guarding double inlet atrioventricular connexion: natural history and surgical results in 76 cases.
        Int J Cardiol. 1990; 28: 7-17
        • Buratto E.
        • Konstantinov I.E.
        Atrioventricular valve surgery: restoration of the fibrous skeleton of the heart.
        J Thorac Cardiovasc Surg. 2021; 162: 360-365
        • Buratto E.
        • Konstantinov I.E.
        Commentary: skeleton in the closet: toward durable repair of atrioventricular valve in univentricular circulation.
        J Thorac Cardiovasc Surg. 2022; 163: 1176-1177
        • Saremi F.
        • Sánchez-Quintana D.
        • Mori S.
        • Muresian H.
        • Spicer D.E.
        • Hassani C.
        • et al.
        Fibrous skeleton of the heart: anatomic overview and evaluation of pathologic conditions with CT and MR imaging.
        Radiographics. 2017; 37: 1330-1351
        • Konstantinov I.E.
        • Sughimoto K.
        • Brizard C.P.
        • d’Udekem Y.
        Single ventricle: repair of atrioventricular valve using the bridging technique.
        Multimed Man Cardiothorac Surg. 2015; 2015: mmv027
        • Takayama T.
        • Nagata N.
        • Miyairi T.
        • Abe M.
        • Koseni K.
        • Yoshimura Y.
        Bridging annuloplasty for common atrioventricular valve regurgitation.
        Ann Thorac Surg. 1995; 59: 1003-1005
        • Sughimoto K.
        • Konstantinov I.E.
        • Brizard C.P.
        • d’Udekem Y.
        Polytetrafluoroethylene bridge for atrioventricular valve repair in single-ventricle palliation.
        J Thorac Cardiovasc Surg. 2015; 149: 641-643
        • He F.
        • Jiao Y.
        • Ma K.
        • Hua Z.
        • Zhang H.
        • Yan J.
        • et al.
        Outcomes of common atrioventricular valve repair in patients with single-ventricle physiology - indication, timing and repair techniques.
        Circ J. 2019; 83: 647-653
        • Misumi Y.
        • Hoashi T.
        • Kagisaki K.
        • Kitano M.
        • Kurosaki K.
        • Shiraishi I.
        • et al.
        Long-term outcomes of common atrioventricular valve plasty in patients with functional single ventricle.
        Interact Cardiovasc Thorac Surg. 2013; 18: 259-265
        • Sughimoto K.
        • Hirata Y.
        • Hirahara N.
        • Miyata H.
        • Suzuki T.
        • Murakami A.
        • et al.
        Mid-term result of atrioventricular valve replacement in patients with a single ventricle.
        Interact Cardiovasc Thorac Surg. 2018; 27: 895-900
        • Perrier S.L.
        • Zhu M.Z.L.
        • Weintraub R.G.
        • Konstantinov I.E.
        Tricuspid valve replacement in failing Fontan circulation with severe ventricular dysfunction: the road not taken?.
        J Thorac Cardiovasc Surg. 2018; 156: e141-e143
        • King G.
        • d’Udekem Y.
        Atrioventricular valve replacement in single-ventricle circulation: a viable option?.
        Interact Cardiovasc Thorac Surg. 2019; 27: 900-901
        • Stephens E.
        • Dearani J.
        Management of the bad atrioventricular valve in Fontan… Time for a change.
        J Thorac Cardiovasc Surg. 2019; 158: 1643-1648
        • Honjo O.
        • Atlin C.R.
        • Mertens L.
        • Al-Radi O.O.
        • Redington A.N.
        • Caldaron C.A.
        • et al.
        Atrioventricular valve repair in patients with functional single-ventricle physiology: impact of ventricular and valve function and morphology on survival and reintervention.
        J Thorac Cardiovasc Surg. 2011; 142: 326-335
        • Menon S.C.
        • Dearani J.A.
        • Cetta F.
        Long-term outcome after atrioventricular valve surgery following modified Fontan operation.
        Cardiol Young. 2011; 21: 83-88
        • Stephens E.H.
        • Dearani J.A.
        • Niaz T.
        • Arghami A.
        • Phillips S.D.
        • Cetta F.
        Effect of earlier atrioventricular valve intervention on survival after the Fontan operation.
        Am J Cardiol. 2020; 137: 103-110

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      • Commentary: The hit parade
        The Journal of Thoracic and Cardiovascular SurgeryVol. 165Issue 2
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          In the Venn diagram overlap of characteristics for single-ventricle candidacy, those with both unbalanced atrioventricular (AV) septal defects and significant AV valve regurgitation are among the greatest risk for palliation failure. In this issue of the Journal, King and colleagues1 present an analysis of the Australia and New Zealand Fontan Registry that attempts to tease apart the contributions of ventricular dominance (right ventricle [RV] vs left) and AV-valve surgery in this population to better profile those at greatest risk for death or transplantation.
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