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Single Ventricle and Fontan Procedures

Chapter

Abstract

Fontan procedures are radical surgical reconstructions performed for children born with only one effective ventricle, or two that cannot be separated functionally. They entail the connection of the pulmonary vascular resistance downstream of the systemic vascular resistance, flow through both being delivered, in series, by the one ventricle, but at the cost of elevated systemic venous pressure (Fig. 10.1). This aims to eliminate shunting and the associated ventricular volume loading, and to achieve full pulmonary oxygenation.

Keywords

Cardiovascular Magnetic Resonance Pulmonary Vascular Resistance Fontan Surgery Fontan Procedure Fontan Patient 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

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Movie 10.1 Three types of Fontan connection illustrated by CMR cine images, (a) atrio-pulmonary connection, (band Movie 10.1) lateral tunnel and (c) extracardiac conduit. (b, c) Can both be called total cavo-pulmonary connection (TCPC). As a transitional stage to Fontan physiology, a limited residual shunt may be left in the form of a fenestration between the IVC pathway and the low-pressure atrial cavity to slightly alleviate systemic venous pressure. The TCPC avoids the progressive right atrial distension which can predispose to atrial arrhythmias, stagnation and thrombosis, and the coronary sinus drains to the low pressure part of the right atrium, which is marked *(AVI 3351 KB)
218193_1_En_10_MOESM2_ESM.avi (2.5 mb)
Movies 10.2 Complications of early atrio-pulmonary Fontan procedures. This sagittal cine image shows the dilated right atrium (RA) with a large thrombus attached to its floor. The solidified, ineffective leaflets of homograft atrial inflow valves can be seen in this case, mildly restricting inflow from the SVCand IVC. For this reason, atrial inflow valves were not included in later variants of the operation (AVI 2583 KB)
Movies 10.3

Complications of early atrio-pulmonary Fontan procedures. The dilated right atrium (RA) upstream of an atrio-pulmonary Fontan connection causing compression of the right lower pulmonary vein (arrow), which then tends to exacerbates right atrial pressure and distension (AVI 1722 KB)

Movie 10.4

Desaturating shunts in three different patients. (a, Movie 10.4) A diastolic leak through the detachment of a patch placed across the right atrio-ventricular valve of a patient with double inlet left ventricle and an atrio-pulmonary Fontan connection. (b) Magnetic resonance contrast angiogram showing evidence of right pulmonary arterio-venous malformations (arrows) in a patient after Kawashima operation in whom hepatic venous return was flowing to the left lung, but not the right. (c, d) Subcutaneous (black arrow) and intra-thoracic (white arrows) branches of systemic venous to pulmonary venous collateral veins (AVI 319 KB)

References

  1. 1.
    Gewillig M. The Fontan circulation. Heart. 2005;91(6):839–46.PubMedCrossRefGoogle Scholar
  2. 2.
    Cleuziou J, Schreiber C, Cornelsen JK, Horer J, Eicken A, Lange R. Bidirectional cavopulmonary connection without additional ­pulmonary blood flow in patients below the age of 6 months. Eur J Cardiothorac Surg. 2008;34(3):556–61.PubMedCrossRefGoogle Scholar
  3. 3.
    Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3):240–8.PubMedCrossRefGoogle Scholar
  4. 4.
    de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection. J Thorac Cardiovasc Surg. 1989;97(4):636.PubMedGoogle Scholar
  5. 5.
    Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990;82(5):1681–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Hsia TY, Migliavacca F, Pittaccio S, et al. Computational fluid dynamic study of flow optimization in realistic models of the total cavopulmonary connections. J Surg Res. 2004;116(2):305–13.PubMedCrossRefGoogle Scholar
  7. 7.
    Reinhardt Z, Uzun O, Bhole V, et al. Sildenafil in the management of the failing Fontan circulation. Cardiol Young. 2010;20(5):522–5.PubMedCrossRefGoogle Scholar
  8. 8.
    Seipelt RG, Franke A, Vazquez-Jimenez JF, et al. Thromboembolic complications after Fontan procedures: comparison of different therapeutic approaches. Ann Thorac Surg. 2002;74(2):556–62.PubMedCrossRefGoogle Scholar
  9. 9.
    Akagi T, Benson LN, Green M, et al. Ventricular performance before and after Fontan repair for univentricular atrioventricular connection: angiographic and radionuclide assessment. J Am Coll Cardiol. 1992;20(4):920–6.PubMedCrossRefGoogle Scholar
  10. 10.
    Kouatli AA, Garcia JA, Zellers TM, Weinstein EM, Mahony L. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation. 1997;96(5):1507–12.PubMedCrossRefGoogle Scholar
  11. 11.
    Prakash A, Khan MA, Hardy R, Torres AJ, Chen JM, Gersony WM. A new diagnostic algorithm for assessment of patients with single ventricle before a Fontan operation. J Thorac Cardiovasc Surg. 2009;138(4):917–23.PubMedCrossRefGoogle Scholar
  12. 12.
    Brown DW, Gauvreau K, Powell AJ, et al. Cardiac magnetic resonance versus routine cardiac catheterization before bidirectional Glenn anastomosis in infants with functional single ventricle: a prospective randomized trial. Circulation. 2007;116(23):2718–25.PubMedCrossRefGoogle Scholar
  13. 13.
    Jones BO, Ditchfield MR, Cahoon GD, et al. Cardiac magnetic resonance imaging prior to bidirectional cavopulmonary connection in hypoplastic left heart syndrome. Heart Lung Circ. 2010;19(9):535–40.PubMedCrossRefGoogle Scholar
  14. 14.
    Bell A, Beerbaum P, Greil G, et al. Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC Cardiovasc Imaging. 2009;2(11):1285–91.PubMedCrossRefGoogle Scholar
  15. 15.
    Kilner PJ, Geva T, Kaemmerer H, Trindade PT, Schwitter J, Webb GD. Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J. 2010;31(7):794–805.PubMedCrossRefGoogle Scholar
  16. 16.
    Garg R, Powell AJ, Sena L, Marshall AC, Geva T. Effects of metallic implants on magnetic resonance imaging evaluation of Fontan palliation. Am J Cardiol. 2005;95(5):688–91.PubMedCrossRefGoogle Scholar
  17. 17.
    Rathod RH, Prakash A, Powell AJ, Geva T. Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol. 2010;55(16):1721–8.PubMedCrossRefGoogle Scholar
  18. 18.
    Grosse-Wortmann L, Al-Otay A, Yoo SJ. Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging. 2009;2(3):219–25.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2012

Authors and Affiliations

  1. 1.Department of Paediatric CardiologyRoyal Brompton HospitalLondonUK
  2. 2.Cardiovascular Magnetic Resonance UnitRoyal Brompton Hospital and Imperial CollegeLondonUK

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