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Meta-Analysis of the Effectiveness of Heart Transplantation in Patients With a Failing Fontan

Published:January 25, 2017DOI:https://doi.org/10.1016/j.amjcard.2017.01.001
      The Fontan procedure is increasingly being used to palliate univentricular physiology. It is a complex anatomic and physiologic repair that can fail at any age, often leaving heart transplantation as the only remaining solution. A meta-analysis was performed to achieve the aim of systematically evaluating the existing evidence for survival after heart transplantation in patients who have undergone a Fontan palliation. MEDLINE, Embase, PubMed, and Web of Science were searched for original research studies. The primary outcome was mortality at 1 and 5 years after transplantation. Five hundred eighty-two records were screened, after the removal of duplicates, 12 retrospective observational studies were selected for inclusion in our meta-analysis. This encompassed a total of 351 Fontan patients undergoing heart transplantation. Mean age was 14 years (range 7 to 24 years) and 65% were men. One- and 5-year survival rates after heart transplantation were found to be 80.3% (95% CI 75.9% to 84.2%) and 71.2% (95% CI 66.3% to 75.7%), respectively. No significant association was found between age, gender, and pulmonary pressures and 1-year mortality. In conclusion, in the largest analysis to date, we found that heart transplantation in younger patients after Fontan procedure has an acceptable early and mid-term mortality. It is comparable to published mortality data of heart transplantation for other forms of congenital heart disease. Heart transplantation in the younger failing Fontan population appears to be a reasonable option when all other avenues have been exhausted and appropriate screening has taken place.
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      References

        • Fontan F.
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. 1971; 26: 240-248
        • Gewillig M.
        The Fontan circulation.
        Heart. 2005; 91: 839-846
        • Davies R.R.
        • Chen J.M.
        • Quaegebeur J.M.
        • Mosca R.S.
        Transplantation for the “failed” Fontan.
        Prog Pediatr Cardiol. 2009; 26: 21-29
        • Huddleston C.B.
        The failing Fontan: options for surgical therapy.
        Pediatr Cardiol. 2007; 28: 472-476
        • Jayakumar K.A.
        • Addonizio L.J.
        • Kichuk-Chrisant M.R.
        • Galantowicz M.E.
        • Lamour J.M.
        • Quaegebeur J.M.
        • Hsu D.T.
        Cardiac transplantation after the Fontan or Glenn procedure.
        J Am Coll Cardiol. 2004; 44: 2065-2072
        • Kanter K.R.
        • Mahle W.T.
        • Vincent R.N.
        • Berg A.M.
        • Kogon B.E.
        • Kirshbom P.M.
        Heart transplantation in children with a Fontan procedure.
        Ann Thorac Surg. 2011; 91: 823-830
        • Hsu D.T.
        • Quaegebeur J.M.
        • Michler R.E.
        • Smith C.R.
        • Rose E.A.
        • Kichuk M.R.
        • Gersony W.M.
        • Douglas J.F.
        • Addonizio L.J.
        Heart-transplantation in children with congenital heart disease.
        J Am Coll Cardiol. 1995; 26: 743-749
        • Gamba A.
        • Merlo M.
        • Fiocchi R.
        • Terzi A.
        • Mammana C.
        • Sebastiani R.
        • Ferrazzi P.
        Heart transplantation in patients with previous Fontan operations.
        J Thorac Cardiovasc Surg. 2004; 127: 555-562
        • Chaudhari M.
        • Sturman J.
        • O'Sullivan J.
        • Smith J.
        • Wrightson N.
        • Parry G.
        • Bolton D.
        • Haynes S.
        • Hamilton L.
        • Hasan A.
        Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.
        J Thorac Cardiovasc Surg. 2005; 129: 416-422
        • Simmonds J.
        • Burch M.
        • Dawkins H.
        • Tsang V.
        Heart transplantation after congenital heart surgery: improving results and future goals.
        Eur J Cardiothorac Surg. 2008; 34: 313-317
        • Irving C.
        • Parry G.
        • O'Sullivan J.
        • Dark J.K.
        • Kirk R.
        • Crossland D.S.
        • Chaudhari M.
        • Griselli M.
        • Hamilton J.R.
        • Hasan A.
        Cardiac transplantation in adults with congenital heart disease.
        Heart. 2010; 96: 1217-1222
        • Kovach J.R.
        • Naftel D.C.
        • Pearce F.B.
        • Tresler M.A.
        • Edens R.E.
        • Shuhaiber J.H.
        • Blume E.D.
        • Fynn-Thompson F.
        • Kirklin J.K.
        • Zangwill S.D.
        Comparison of risk factors and outcomes for pediatric patients listed for heart transplantation after bidirectional Glenn and after Fontan: an analysis from the Pediatric Heart Transplant Study.
        J Heart Lung Transplant. 2012; 31: 133-139
        • Paniagua Martin M.J.
        • Almenar L.
        • Brossa V.
        • Crespo-Leiro M.G.
        • Segovia J.
        • Palomo J.
        • Delgado J.
        • González-Vílchez F.
        • Manito N.
        • Lage E.
        • García-Guereta L.
        • Rodríguez-Lambert J.L.
        • Albert D.C.
        Transplantation for complex congenital heart disease in adults: a subanalysis of the Spanish Heart Transplant Registry.
        Clin Transplant. 2012; 26: 755-763
        • Seddio F.
        • Gorislavets N.
        • Iacovani A.
        • Cugola D.
        • Fontana A.
        • Galletti L.
        • Terzi A.
        • Ferrazzi P.
        Heart transplantation in single-ventricle physiology with failed Fontan circulation. A 25-year single centre experience.
        Eur J Cardiothorac Surg. 2013; 43: 605-611
        • Delmo Walter E.
        • Hetzer R.
        Heart transplantation in single-ventricle physiology with failed Fontan circulation.
        J Cardiothorac Surg. 2013; 8: 136
        • Rungan S.
        • Finucane K.
        • Gentles T.
        • Gibbs H.C.
        • Hu R.
        • Ruygrok P.N.
        Heart transplantation in pediatric and congenital heart disease: a single-center experience.
        World J Pediatr Congenit Heart Surg. 2014; 5: 200-205
        • Iyengar A.J.
        • Sharma V.J.
        • Weintraub R.G.
        • Shipp A.
        • Brizard C.P.
        • d'Udekem R.
        • Konstantinov I.E.
        Surgical strategies to facilitate heart transplantation in children after failed univentricular palliations: the role of advanced intraoperative surgical preparation.
        Eur J Cardiothorac Surg. 2014; 46: 480-485
        • Michielon G.
        • van Melle J.P.
        • Wolff D.
        • Di Carlo D.
        • Jacobs J.P.
        • Mattila I.P.
        • Berggren H.
        • Lindberg H.
        • Padalino M.A.
        • Meyns B.
        • Prêtre R.
        • Helvind M.
        • Carrel T.
        • Ebels T.
        Favourable mid-term outcome after heart transplantation for late Fontan failure.
        Eur J Cardiothorac Surg. 2015; 47: 665-671
        • Burchill L.J.
        • Edwards L.B.
        • Dipchand A.I.
        • Stehlik J.
        • Ross H.J.
        Impact of adult congenital heart disease on survival and mortality after heart transplantation.
        J Heart Lung Transplant. 2014; 33: 1157-1163
        • Bernstein D.
        • Naftel D.
        • Chin C.
        • Addonizio L.J.
        • Gamberg P.
        • Blume E.D.
        • Hsu D.
        • Canter C.E.
        • Kirklin J.K.
        • Morrow W.R.
        • on behalf of the Pediatric Heart Transplant Study
        Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study.
        Circulation. 2006; 114: 273-280
        • Lamour J.M.
        • Kanter K.R.
        • Naftel D.C.
        • Chrisant M.R.
        • Morrow W.R.
        • Clemson B.S.
        • Kirklin J.K.
        • on behalf of the Cardiac Transplant Registry Database and the Pediatric Heart Transplant Study
        The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease.
        J Am Coll Cardiol. 2009; 54: 160-165
        • Prendergast T.W.
        • Furukawa S.
        • Beyer A.J.
        • Browne B.J.
        • Elsen H.J.
        • Jeevanandam V.
        The role of gender in heart transplantation.
        Ann Thorac Surg. 1998; 65: 88-94
        • d'Udekem Y.
        • Iyengar A.J.
        • Cochrane A.D.
        • Grigg L.E.
        • Ramsay J.M.
        • Wheaton G.R.
        • Penny D.J.
        • Brizard C.P.
        The Fontan procedure: contemporary techniques have improved long-term outcomes.
        Circulation. 2007; 116: I157-I164
        • Coskun O.
        • Coskun T.
        • El-Arousy M.
        • Parsa M.A.
        • Blanz U.
        • Von Knyphausen E.
        • Sandica E.
        • Schulz U.
        • Knobl H.
        • Tenderich G.
        • Bairaktaris A.
        • Keceioglu D.
        • Körfer R.
        Heart transplantation in adults with congenital heart disease: experience with 15 patients.
        ASAIO J. 2007; 53: 103-106
        • Karamlou T.
        • Hirsch J.
        • Welke K.
        • Ohye R.G.
        • Bove E.L.
        • Devaney E.J.
        • Gajarski R.J.
        A United Network for Organ Sharing analysis of heart transplantation in adults with congenital heart disease: outcomes and factors associated with mortality and retransplantation.
        J Thorac Cardiovasc Surg. 2010; 140: 161-168
        • Carey J.A.
        • Hamilton J.R.
        • Hilton C.J.
        • Dark J.H.
        • Forty J.
        • Parry G.
        • Hasan A.
        Orthotopic cardiac transplantation for the failing Fontan circulation.
        Eur J Cardiothorac Surg. 1998; 14: 7-14
        • Mitchell M.B.
        • Campbell D.N.
        • Ivy D.
        • Boucek M.M.
        • Sondheimer H.M.
        • Pietra B.
        • Das B.B.
        • Coll J.R.
        Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure.
        J Thorac Cardiovasc Surg. 2004; 128: 693-702
        • Magdo H.S.
        • Stillwell T.L.
        • Greenhawt M.J.
        • Stringer K.A.
        • Yu S.
        • Fifter C.G.
        • Russell M.W.
        • Schumacher K.R.
        Immune abnormalities in Fontan protein-losing enteropathy: a case-control study.
        J Pediatr. 2015; 167: 331-337
        • Dipchand A.I.
        • Edwards L.B.
        • Kucheryavaya A.Y.
        • Benden C.
        • Dobbels F.
        • Levvey B.J.
        • Lund L.H.
        • Meiser B.
        • Yusen R.D.
        • Stehlik J.
        The Registry of the International Society for Heart and Lung Transplantation: seventeenth official pediatric heart transplantation report- 2014; focus theme: retransplantation.
        J Heart Lung Transplant. 2014; 33: 985-995
        • Stehlik J.
        • Edwards L.B.
        • Kucheryavaya A.Y.
        • Aurora P.
        • Christie J.D.
        • Kirk R.
        • Dobbels F.
        • Rahmel A.O.
        • Hertz M.I.
        The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official adult heart transplant report- 2010.
        J Heart Lung Transplant. 2009; 29: 1089-1103