Advertisement

A new surgical challenge: Reconstruction of aortic arch aneurysm late after the Norwood procedure in an adult with Fontan circulation

  • Mimi X. Deng
    Affiliations
    Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, Toronto, Ontario, Canada
    Search for articles by this author
  • David Barron
    Affiliations
    Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, Toronto, Ontario, Canada

    Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada

    Department of Surgery, University of Toronto, Toronto, Ontario, Canada
    Search for articles by this author
  • Kevin R. An
    Affiliations
    Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, Toronto, Ontario, Canada
    Search for articles by this author
  • Marjan Jariani
    Affiliations
    Department of Cardiovascular Anesthesia, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada
    Search for articles by this author
  • Osami Honjo
    Correspondence
    Address for reprints: Osami Honjo, MD, PhD, Division of Cardiovascular Surgery, Labatt Family Heart Center, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada
    Affiliations
    Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, Toronto, Ontario, Canada

    Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada

    Department of Surgery, University of Toronto, Toronto, Ontario, Canada
    Search for articles by this author
Open AccessPublished:July 07, 2022DOI:https://doi.org/10.1016/j.xjtc.2022.06.019

      Graphical abstract

      Figure thumbnail fx2
      Mechanical Bentall and aortic arch aneurysm repair with end-to-side graft anastomosis.
      Reoperation for aortic arch aneurysm after Fontan completion is challenged by the patient's unique physiology and recreation of the proximal arch without kinking.
      With increasing survival into adulthood after Norwood-type reconstruction, there is a growing population of patients with aneurysmal degeneration of the reconstructed ascending aorta and aortic arch.
      • Cohen M.S.
      • Marino B.S.
      • McElhinney D.B.
      • Robbers-Visser D.
      • van der Woerd W.
      • Gaynor J.W.
      • et al.
      Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome.
      A redo aortic arch reconstruction poses significant risk in patients with Fontan circulation.

      Clinical Summary

      A 27-year-old patient, with original diagnosis of hypoplastic left heart syndrome variant and tricuspid atresia with malposed great vessels, underwent Norwood and subsequent Fontan operation in in the neonatal period, and presented with an asymptomatic aortic arch aneurysm, dilated neoaortic root, severe neoaortic valve regurgitation, and mildly reduced ventricular function. Chest computed tomography imaging showed an aneurysmal ascending aorta measuring 63 × 58 × 57-mm abutting the sternum (Figure 1). The surgical challenges include high-risk reentry and arch aneurysm dissection while avoiding left recurrent nerve and left pulmonary artery injury, myocardial and cerebral protection, and recreation of the aortic arch without kinking. The patient's informed consent was obtained.
      Figure thumbnail gr1
      Figure 1Preoperative cardiac computed tomography showing 63 × 58 × 57 mm ascending aortic and aortic arch aneurysm in a 27-year-old patient with Fontan circulation shown in (A) coronal and (B) axial views.
      Valve-sparing root replacement was not feasible in this case because of severe aortic insufficiency in the setting of a heavily dysplastic neoaortic valve. At our institution, there is a low threshold to offer the Bentall procedure. Rather than risking an unsuccessful valve repair, the reliability of valve replacement minimizes repeat sternotomies in Fontan patients, of whom many eventually require a heart transplant, for which there is compounding risk of allosensitization and adhesions associated with multiple operations.

      Surgical Techniques

      Axillary artery cannulation using a Dacron side graft was performed before redo sternotomy, reducing the risk of sternal reentry, and providing selective cerebral perfusion during arch reconstruction. The chest was entered uneventfully, and cardiopulmonary bypass was initiated with axillary arterial and bicaval cannulation. The next challenge was navigating the large aneurysmal aortic arch while dissecting between the aortic arch and the left pulmonary artery. Placement of the crossclamp was managed after careful dissection, and antegrade cardioplegia was delivered to achieve cardiac arrest. The ascending aorta was quickly opened near the Damus–Kaye–Stansel anastomosis and additional direct coronary cardioplegia was given. The aneurysmal portion was resected while maintaining continuity between the descending aorta and arch vessels on the posterior aspect. When the patient was cooled to 20 °C for deep hypothermic circulatory arrest. A 26-mm Dacron graft was beveled and anastomosed to the descending aorta and the aortic arch with the patient under full circulatory arrest. The remaining aortic arch reconstruction was performed with the patient under selective cerebral perfusion. To address the acute angle formed by the Dacron graft at the transition from the ascending aorta to the aortic arch, the proximal end of the graft was oversewn in anticipation for the end-to-side anastomosis to the distal Bentall graft. The native pulmonary valve (ie, the neoaortic valve) was excised and a 29-mm Bentall composite mechanical valve was implanted. The distal end of the Bentall graft was then anastomosed to the inferior aspect of the proximal aortic arch graft in an end-to-side fashion. Finally, a rectangular opening was created on the anterior aspect of the ascending aortic graft and the native aorta was reanastomosed to the graft (Figure 2). Cardiopulmonary bypass was terminated without difficulty. The postoperative course was complicated by an acute right middle cerebral artery infarct, acute respiratory distress syndrome, and left vocal cord paralysis. The patient was discharged to stroke rehabilitation on postoperative day 22. At 3 months postrepair, his neurological deficits and vocal cord function significantly improved, and he has returned to work.
      Figure thumbnail gr2
      Figure 2Surgical illustration of complete ascending and arch aneurysm repair in Fontan circulation, involving end-to-side aortic graft anastomosis and mechanical Bentall.

      Discussion

      With more single-ventricle palliation patients surviving into adulthood, neoaortic dilatation after Fontan reconstruction is becoming increasing recognized. In one study it was reported that 98% of post-Fontan patients develop an aortic root z-score of >2 at a median follow-up of 9.2 years.
      • Cohen M.S.
      • Marino B.S.
      • McElhinney D.B.
      • Robbers-Visser D.
      • van der Woerd W.
      • Gaynor J.W.
      • et al.
      Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome.
      The 2 long-term concerns of Norwood reconstruction are dilatation of the pulmonary homograft from high-pressure circulation, and cystic medial degeneration of the aortic arch in the absence of connective tissue disease as a result of myxoid matrix proliferation.
      • Shuhaiber J.H.
      • Patel V.
      • Husayni T.
      • El-Zein C.
      • Barth M.J.
      • Ilbawi M.N.
      Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome.
      ,
      • Ehsan A.
      • Singh H.
      • Vargas S.O.
      • Sachweh J.
      • Jonas R.A.
      Neoaortic aneurysm after stage I Norwood reconstruction.
      Seven cases of neoaortic aneurysms after Fontan reconstructions have been previously reported, with ages at the time of repair ranging from 4.5 to 24 years and aneurysm size ranging from 5.4 to 17 cm.
      • Shuhaiber J.H.
      • Patel V.
      • Husayni T.
      • El-Zein C.
      • Barth M.J.
      • Ilbawi M.N.
      Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome.
      • Ehsan A.
      • Singh H.
      • Vargas S.O.
      • Sachweh J.
      • Jonas R.A.
      Neoaortic aneurysm after stage I Norwood reconstruction.
      • Hebson C.L.
      • Kanter K.R.
      • Maher K.O.
      • Slesnick T.C.
      Late development of a gigantic aneurysm of the neoaorta after Norwood palliation.
      • Contreras J.
      • Bannan B.
      • Chaturvedi R.
      • Barron D.J.
      Bentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection.
      • Herrmann J.L.
      • Lewis M.J.
      • Fuller S.
      • Mascio C.E.
      Aneurysm formation after the Norwood procedure: case report and review of the literature.
      • Pizarro C.
      • Baffa J.M.
      • Derby C.D.
      • Krieger P.A.
      Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome.
      • Pizarro C.
      Valve-sparing neoaortic root replacement post Norwood.
      Repair techniques involved valve-sparing root replacement,
      • Pizarro C.
      • Baffa J.M.
      • Derby C.D.
      • Krieger P.A.
      Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome.
      ,
      • Pizarro C.
      Valve-sparing neoaortic root replacement post Norwood.
      ascending aortic replacement with
      • Shuhaiber J.H.
      • Patel V.
      • Husayni T.
      • El-Zein C.
      • Barth M.J.
      • Ilbawi M.N.
      Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome.
      or without
      • Ehsan A.
      • Singh H.
      • Vargas S.O.
      • Sachweh J.
      • Jonas R.A.
      Neoaortic aneurysm after stage I Norwood reconstruction.
      external polytetrafluoroethylene strip reinforcement for neoaortic root downsizing, bio-
      • Hebson C.L.
      • Kanter K.R.
      • Maher K.O.
      • Slesnick T.C.
      Late development of a gigantic aneurysm of the neoaorta after Norwood palliation.
      or mechanical
      • Contreras J.
      • Bannan B.
      • Chaturvedi R.
      • Barron D.J.
      Bentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection.
      Bentall, and 1 case of aortic arch aneurysm repair using a 2-graft technique with end-to-end anastomosis.
      • Herrmann J.L.
      • Lewis M.J.
      • Fuller S.
      • Mascio C.E.
      Aneurysm formation after the Norwood procedure: case report and review of the literature.
      To our knowledge, our 27-year-old male patient is the oldest of reported cases and our report is the first to describe end-to-side graft anastomosis to facilitate transition between the arch and ascending neoaorta. This technique navigates the curvature of the aortic arch while minimizing graft kinking associated with single-graft arch reconstruction or inadequate bevelling of an end-to-end graft anastomosis (Figure 3).
      Figure thumbnail gr3
      Figure 3End-to-side aortic graft anastomosis to navigate arch transition in ascending and arch aneurysm repair after Fontan completion.
      The case also highlights the need for lifetime imaging surveillance in late survivors of Norwood reconstructions, as well as the need for expert consensus on timing of imaging surveillance and reintervention thresholds. The indications for surgical intervention include symptomatic presentation, significant neoaortic regurgitation, compromise of the Fontan circulation because of pulmonary artery obstruction secondary to extrinsic aneurysmal compression, and progression of neoaorta dilatation.
      • Shuhaiber J.H.
      • Patel V.
      • Husayni T.
      • El-Zein C.
      • Barth M.J.
      • Ilbawi M.N.
      Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome.
      In asymptomatic and hemodynamically stable patients, reoperation is commonly deferred until implantation of an adult-size graft is feasible. When surgery is decided, careful preoperative planning is paramount for reoperations after Fontan completion because of dense adhesions and tenuous Fontan physiology.

      References

        • Cohen M.S.
        • Marino B.S.
        • McElhinney D.B.
        • Robbers-Visser D.
        • van der Woerd W.
        • Gaynor J.W.
        • et al.
        Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome.
        J Am Coll Cardiol. 2003; 42: 533-540https://doi.org/10.1016/S0735-1097(03)00715-0
        • Shuhaiber J.H.
        • Patel V.
        • Husayni T.
        • El-Zein C.
        • Barth M.J.
        • Ilbawi M.N.
        Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 2006; 131: 478-479https://doi.org/10.1016/j.jtcvs.2005.09.047
        • Ehsan A.
        • Singh H.
        • Vargas S.O.
        • Sachweh J.
        • Jonas R.A.
        Neoaortic aneurysm after stage I Norwood reconstruction.
        Ann Thorac Surg. 2005; 79: e23-e25https://doi.org/10.1016/j.athoracsur.2004.10.056
        • Hebson C.L.
        • Kanter K.R.
        • Maher K.O.
        • Slesnick T.C.
        Late development of a gigantic aneurysm of the neoaorta after Norwood palliation.
        Ann Thorac Surg. 2013; 95: 1457https://doi.org/10.1016/j.athoracsur.2012.08.064
        • Contreras J.
        • Bannan B.
        • Chaturvedi R.
        • Barron D.J.
        Bentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection.
        Interact Cardiovasc Thorac Surg. 2020; 31: 578-579https://doi.org/10.1093/icvts/ivaa139
        • Herrmann J.L.
        • Lewis M.J.
        • Fuller S.
        • Mascio C.E.
        Aneurysm formation after the Norwood procedure: case report and review of the literature.
        J Thorac Cardiovasc Surg. 2014; 147: e55-e56https://doi.org/10.1016/j.jtcvs.2013.12.056
        • Pizarro C.
        • Baffa J.M.
        • Derby C.D.
        • Krieger P.A.
        Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 2011; 141: 1083-1084https://doi.org/10.1016/j.jtcvs.2010.07.076
        • Pizarro C.
        Valve-sparing neoaortic root replacement post Norwood.
        Oper Tech Thorac Cardiovasc Surg. 2020; 25: 2-12https://doi.org/10.1053/j.optechstcvs.2019.12.001