Congenital: Fontan| Volume 162, ISSUE 2, P372-380.e2, August 2021

Progression in Fontan conduit stenosis and hemodynamic impact during childhood and adolescence

Published:October 28, 2020DOI:



      To characterize changes in Fontan conduit size over time and determine if cross-sectional area (CSA) affects cardiac output, pulmonary artery growth, and exercise capacity.


      We conducted a retrospective cross-sectional study of patients with Fontan physiology who underwent cardiac magnetic resonance imaging or cardiac catheterization between January 2013 and October 2019. We collected Fontan and pulmonary artery measurements, hemodynamic data, and cardiopulmonary exercise test data. We identified 158 patients with an extracardiac Fontan. We measured minimum and mean Fontan conduit CSA and assessed whether these correlated with Nakata index, cardiac index, or exercise capacity.


      Minimum Fontan CSA decreased by a median of 33% (24%, 40%) during a mean follow-up of 9.6 years. Median percentage decrease in Fontan CSA did not differ among 16-, 18-, and 20-mm conduits (P = .29). There was a significant decrease in the minimum Fontan CSA (33% [25%, 41%]) starting less than 1-year post-Fontan. Median Nakata index was 177.6 mm2/m2 (149.1, 210.8) and was not associated with Fontan CSA/BSA (ρ = 0.09, P = .29). Fontan CSA/BSA was not associated with cardiac index (ρ = –0.003, P = .97). A larger Fontan CSA/BSA had a modest correlation with % predicted oxygen consumption (ρ = 0.31, P = .013).


      Fontan conduit CSA decreases as early as 6 months post-Fontan. The minimum Fontan CSA/BSA was not associated with cardiac index or pulmonary artery size but did correlate with % predicted peak oxygen consumption.

      Graphical abstract

      Key Words


      BSA (body surface area), CHLA (Children's Hospital Los Angeles), CPET (cardiopulmonary exercise test), CSA (cross-sectional area), IVC (inferior vena cava), LPA (left pulmonary artery), MRI (magnetic resonance imaging), Qp:Qs (ratio of pulmonary blood flow to systemic blood flow), RPA (right pulmonary artery), SVC (superior vena cava), VO2 (oxygen consumption)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The Journal of Thoracic and Cardiovascular Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Fontan F.
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. 1971; 26: 240-248
        • Amodeo A.
        • Galletti L.
        • Marianeschi S.
        • Picardo S.
        • Giannico S.
        • Di Renzi P.
        • et al.
        Extracardiac Fontan operation for complex cardiac anomalies: seven years' experience.
        J Thorac Cardiovasc Surg. 1997; 114 (discussion 1030-1): 1020-1030
        • De Leval M.R.
        • Kilner P.
        • Gewillig M.
        • Bull C.
        Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.
        J Thorac Cardiovasc Surg. 1988; 96: 682-695
        • D'Udekem Y.
        • Iyengar A.J.
        • Cochrane A.D.
        • Grigg L.E.
        • Ramsay J.M.
        • Wheaton G.R.
        • et al.
        The Fontan procedure: contemporary techniques have improved long-term outcomes.
        Circulation. 2007; 116: I157-I164
        • Cetta F.
        • Burkhart H.M.
        The Fontan extracardiac conduit: pne size does not fit all.
        Transl Pediatr. 2018; 7: 233-234
        • Itatani K.
        • Miyaji K.
        • Tomoyasu T.
        • Nakahata Y.
        • Ohara K.
        • Takamoto S.
        • et al.
        Optimal conduit size of the extracardiac Fontan operation based on energy loss and flow stagnation.
        Ann Thorac Surg. 2009; 88: 565-573
        • Lee C.
        • Lee C.H.
        • Hwang S.W.
        • Lim H.G.
        • Kim S.J.
        • Lee J.Y.
        • et al.
        Midterm follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure.
        Eur J Cardiothorac Surg. 2007; 31: 1008-1012
        • Hagler D.J.
        • Miranda W.R.
        • Haggerty B.J.
        • Anderson J.H.
        • Johnson J.N.
        • Cetta F.
        • et al.
        Fate of the Fontan connection: mechanisms of stenosis and management.
        Congenit Heart Dis. 2019; 14: 571-581
        • Ten Cate F.E.A.U.
        • Trieschmann U.
        • Germund I.
        • Hannes T.
        • Emmel M.
        • Bennink G.
        • et al.
        Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits.
        Heart. 2017; 103: 1111-1116
        • Tang E.
        • Wei Z.
        • Whitehead K.K.
        • Khiabani R.H.
        • Restrepo M.
        • Mirabella L.
        • et al.
        Effect of Fontan geometry on exercise haemodynamics and its potential implications.
        Heart. 2017; 103: 1806-1812
        • Lee S.-Y.
        • Song M.-K.
        • Kim G.-B.
        • Bae E.-J.
        • Kim S.-H.
        • Jang S.-I.
        • et al.
        Relation between exercise capacity and extracardiac conduit size in patients with Fontan circulation.
        Pediatr Cardiol. 2019; 40: 1584-1590
        • Patel N.D.
        • Sullivan P.M.
        • Sabati A.
        • Hill A.
        • Maedler-Kron C.
        • Zhou S.
        • et al.
        Routine surveillance catheterization is useful in guiding management of stable Fontan patients.
        Pediatr Cardiol. 2020; 41: 624-631
        • Nakata S.
        • Imai Y.
        • Takanashi Y.
        • Kurosawa H.
        • Tezuka K.
        • Nakazawa M.
        • et al.
        A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow.
        J Thorac Cardiovasc Surg. 1984; 88: 610-619
        • Cooper D.M.
        • Weiler Ravell D.
        Gas exchange response to exercise in children.
        Am Rev Respir Dis. 1984; 129: S47-S48
        • Cooper D.M.
        • Weiler-Ravell D.
        • Whipp B.J.
        • Wasserman K.
        Growth-related changes in oxygen uptake and heart rate during progressive exercise in children.
        Pediatr Res. 1984; 18: 845-851
        • Jones N.L.
        • Makrides L.
        • Hitchcock C.
        • Chypchar T.
        • McCartney N.
        Normal standards for an incremental progressive cycle ergometer test.
        Am Rev Respir Dis. 1985; 131: 700-708
        • Gewillig M.
        • Goldberg D.J.
        Failure of the Fontan circulation.
        Heart Fail Clin. 2014; 10: 105-116
        • Ovroutski S.
        • Ewert P.
        • Alexi-Meskishvili V.
        • Hölscher K.
        • Miera O.
        • Peters B.
        • et al.
        Absence of pulmonary artery growth after Fontan operation and its possible impact on late outcome.
        Ann Thorac Surg. 2009; 87: 826-831
        • Restrepo M.
        • Tang E.
        • Haggerty C.M.
        • Khiabani R.H.
        • Mirabella L.
        • Bethel J.
        • et al.
        Energetic implications of vessel growth and flow changes over time in fontan patients.
        Ann Thorac Surg. 2015; 99: 163-170
        • Lehner A.
        • Schuh A.
        • Herrmann F.E.M.
        • Riester M.
        • Pallivathukal S.
        • Dalla-Pozza R.
        • et al.
        Influence of pulmonary artery size on early outcome after the fontan operation.
        Ann Thorac Surg. 2014; 97: 1387-1393
        • Knott-Craig C.J.
        • Danielson G.K.
        • Schaff H.V.
        • Puga F.J.
        • Weaver A.L.
        • Driscoll D.D.
        The modified fontan operation. An analysis of risk factors for early postoperative death or takedown in 702 consecutive patients from one institution.
        J Thorac Cardiovasc Surg. 1995; 109: 1237-1243
        • Senzaki H.
        • Isoda T.
        • Ishizawa A.
        • Hishi T.
        Reconsideration of criteria for the Fontan operation: influence of pulmonary artery size on postoperative hemodynamics of the Fontan operation.
        Circulation. 1994; 89: 1196-1202
        • Chowdhury U.K.
        • Airan B.
        • Sharma R.
        • Bhan A.
        • Kothari S.S.
        • Saxena A.
        • et al.
        Univentricular repair in children under 2 years of age: early and midterm results.
        Heart Lung Circ. 2001; 10: 3-13
        • Evans W.N.
        • Winn B.J.
        • Yumiaco N.S.
        • Galindo A.
        • Rothman A.
        • Acherman R.J.
        • et al.
        Transvenous hepatic biopsy in stable fontan patients undergoing cardiac catheterization.
        Pediatr Cardiol. 2014; 35: 1273-1278
        • Silva-Sepulveda J.A.
        • Fonseca Y.
        • Vodkin I.
        • Vaughn G.
        • Newbury R.
        • Vavinskaya V.
        • et al.
        Evaluation of Fontan liver disease: correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.
        Congenit Heart Dis. 2019; 14: 600-608
        • Ohuchi H.
        • Negishi J.
        • Noritake K.
        • Hayama Y.
        • Sakaguchi H.
        • Miyazaki A.
        • et al.
        Prognostic value of exercise variables in 335 patients after the fontan operation: a 23-year single-center experience of cardiopulmonary exercise testing.
        Congenit Heart Dis. 2015; 10: 105-116
        • Paridon S.M.
        • Mitchell P.D.
        • Colan S.D.
        • Williams R.V.
        • Blaufox A.
        • Li J.S.
        • et al.
        A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation.
        J Am Coll Cardiol. 2008; 52: 99-107
        • Fernandes S.M.
        • McElhinney D.B.
        • Khairy P.
        • Graham D.A.
        • Landzberg M.J.
        • Rhodes J.
        Serial cardiopulmonary exercise testing in patients with previous fontan surgery.
        Pediatr Cardiol. 2010; 31: 175-180
        • Alexi-Meskishvili V.
        • Ovroutski S.
        • Ewert P.
        • Dähnert I.
        • Berger F.
        • Lange P.E.
        • et al.
        Optimal conduit size for extracardiac Fontan operation.
        Eur J Cardiothorac Surg. 2000; 18: 690-695
        • Bockeria L.A.
        • Svanidze O.
        • Kim A.
        • Shatalov K.
        • Makarenko V.
        • Cox M.
        • et al.
        Total cavopulmonary connection with a new bioabsorbable vascular graft: first clinical experience.
        J Thorac Cardiovasc Surg. 2017; 153: 1542-1550
        • Liang F.
        • Senzaki H.
        • Kurishima C.
        • Sughimoto K.
        • Inuzuka R.
        • Liu H.
        Hemodynamic performance of the Fontan circulation compared with a normal biventricular circulation: a computational model study.
        Am J Physiol Heart Circ Physiol. 2014; 307: H1056-H1072

      Linked Article

      • Commentary: You should occasionally look at the results!!
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          How often are we delusional, even in our most simple beliefs? We have for long implanted 18-mm or 20-mm Gore-Tex conduits (W. L. Gore & Associates, Inc, Flagstaff, Ariz) as extracardiac Fontan conduits because we believed that this was the size of an adult inferior vena cava and we wanted to have the most streamlined venous. Patel and colleagues1 report in this issue of the Journal a detailed analysis of Fontan circuit sizes by both magnetic resonance imaging and catheterization in 156 patients over a mean follow-up period of 10 years.
        • Full-Text
        • PDF
      • Commentary: As we learn more, we know little
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          The Fontan operation is the goal for patients with a single ventricle, and the procedure has undergone various modifications over the years. Originally introduced in 1990,1 extracardiac conduit Fontan possesses some advantages such as improved flow dynamics, lower arrhythmias, and technically less challenging. Nonetheless, lack of growth potential and thrombogenecity are important drawbacks and lead to reinterventions. Patel and colleagues2 have provided us with an important study looking at the intermediate outcome of the extracardiac Fontan.
        • Full-Text
        • PDF
      • Commentary: The heart of the matter: Close clinical follow-up and exercise capacity in Fontan circulation
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          In this issue of the Journal, Patel and colleagues1 evaluate the cross-sectional area (CSA) of extracardiac Fontan conduits and associated hemodynamic impact at various times during follow-up. The authors are to be commended for their compilation of a large number of patients with Fontan circulation (FC) with magnetic resonance imaging, catheter, and cardiopulmonary exercise testing (CPET) data. Their analysis revealed a median CSA decline of 68% compared with the original CSA of the conduit implanted.
        • Full-Text
        • PDF