Advertisement

Single Ventricle and Fontan Procedures

  • Lucia FlorsEmail author
  • Patrick T. Norton
  • Klaus D. Hagspiel
Chapter
Part of the Medical Radiology book series (MEDRAD)

Abstract

Univentricular congenital heart diseases include a range of pathologies that result in a functionally single ventricular chamber. The most common pathologies in this group are tricuspid atresia, pulmonary atresia with an intact ventricular septum, hypoplastic left heart syndrome, and a double-inlet ventricle. Although the only curative therapy for these patients is cardiac transplantation, there are several palliative surgical techniques that divert part or all the systemic venous circulation into the pulmonary arteries bypassing the single ventricular chamber. The modern Fontan procedure consists in anastomosing both SVC and IVC to the right pulmonary artery; it is nowadays the last step of single ventricle palliation.

The importance of imaging in these pathologies lies not only in the understanding of the new circuit established after surgical correction, but also in the early detection of the wide spectrum of cardiac and extracardiac complications that can happen due to the new physiological condition. Due to the increased survival of these patients, long-term complications are becoming more common. Imaging patients with single ventricle physiology and particularly following single ventricle palliative procedures is challenging due to the altered anatomy and hemodynamics. While MRI and MRA should be considered the modality of choice due to the inherent lack of ionizing radiation in this patient population, CT angiography is an important alternative noninvasive imaging technique. In this chapter, we review the different palliative surgical techniques in patients with univentricular heart diseases, and we describe the optimal imaging protocol and the expected surgical anatomy as well as the long-term complications.

Keywords

Fontan procedure Hypoplastic left heart syndrome Tricuspid atresia Pulmonary atresia Magnetic resonance imaging Computed tomography 

Supplementary material

Video 1

Time-resolved contrast-enhanced MRA permits acquisition of images with a temporal resolution of approximately 2 s, with excellent depiction of the hemodynamics in patients with surgical correction for univentricular heart. In this 13-year-old girl after Fontan procedure for pulmonary atresia (same patient as in Fig. 10) time-resolved contrast-enhanced MRA shows early enhancement of Glenn conduit anastomosed to the right pulmonary artery and strong and early contrast opacification of the right upper pulmonary vein (white arrow) due to the presence of the PAVFs. (AVI 21063 kb)

References

  1. Ashrafian H, Swan L (2002) The mechanism of formation of pulmonary arteriovenous malformations associated with the classic Glenn shunt (superior cavopulmonary anastomosis). Heart 88:639CrossRefGoogle Scholar
  2. Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757CrossRefGoogle Scholar
  3. Bachler P, Valverde I, Pinochet N et al (2013) Caval blood flow distribution in patients with Fontan circulation: quantification by using particle traces from 4D flow MR imaging. Radiology 267:67–75CrossRefGoogle Scholar
  4. Bove EL, de Leval MR, Migliavacca F, Guadagni G, Dubini G (2003) Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 126:1040–1047CrossRefGoogle Scholar
  5. Bradley SM (2006) Extracardiac conduit Fontan procedure. Oper Tech Thorac Cardiovasc Surg 11:123–140CrossRefGoogle Scholar
  6. Bridges ND, Mayer JE Jr, Lock JE et al (1992) Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 86:1762–1769CrossRefGoogle Scholar
  7. Bryant T, Ahmad Z, Millward-Sadler H et al (2011) Arterialised hepatic nodules in the Fontan circulation: hepatico-cardiac interactions. Int J Cardiol 151:268–272CrossRefGoogle Scholar
  8. ACR NASCI SIR SPR practice parameters for the performance and interpretation of body computed tomography angiography (CTA) (2016). Available: https://www.acr.org/~/media/168A72F0C6004CA9A649DBD6EA9368DE.pdf. Accessed 2 Jan 2017
  9. Duncan BW, Desai S (2003) Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg 76:1759–1766CrossRefGoogle Scholar
  10. Feldt RH, Driscoll DJ, Offord KP et al (1996) Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 112:672–680CrossRefGoogle Scholar
  11. Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248CrossRefGoogle Scholar
  12. Fredenburg TB, Johnson TR, Cohen MD (2011) The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics 31:453–463CrossRefGoogle Scholar
  13. Gewillig M (2005) The Fontan circulation. Heart 91:839–846CrossRefGoogle Scholar
  14. Ghadimi Mahani M, Agarwal PP, Rigsby CK et al (2016) CT for assessment of thrombosis and pulmonary embolism in multiple stages of single-ventricle palliation: challenges and suggested protocols. Radiographics 36:1273–1284CrossRefGoogle Scholar
  15. Ghaferi AA, Hutchins GM (2005) Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 129:1348–1352CrossRefGoogle Scholar
  16. Goo HW, Yang DH, Park IS et al (2007) Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience. J Magn Reson Imaging 25:727–736CrossRefGoogle Scholar
  17. Goo HW, Jhang WK, Kim YH et al (2008) CT findings of plastic bronchitis in children after a Fontan operation. Pediatr Radiol 38:989–993CrossRefGoogle Scholar
  18. Grosse-Wortmann L, Al-Otay A, Yoo SJ (2009) Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging 2:219–225CrossRefGoogle Scholar
  19. Hashemi S, Parks WJ, Slesnick TC (2014) 3D inversion recovery gradient echo respiratory navigator imaging using Gadofosveset Trisodium in a Fontan Y-graft patient. Int J Cardiovasc Imaging 30:993–994CrossRefGoogle Scholar
  20. Khanna G, Bhalla S, Krishnamurthy R, Canter C (2012) Extracardiac complications of the Fontan circuit. Pediatr Radiol 42:233–241CrossRefGoogle Scholar
  21. Kiesewetter CH, Sheron N, Vettukattill JJ et al (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584CrossRefGoogle Scholar
  22. Kim SJ, Bae EJ, Lee JY, Lim HG, Lee C, Lee CH (2009) Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thorac Surg 87:548–553CrossRefGoogle Scholar
  23. Latus H, Gerstner B, Kerst G et al (2016) Effect of inhaled nitric oxide on blood flow dynamics in patients after the Fontan procedure using cardiovascular magnetic resonance flow measurements. Pediatr Cardiol 37:504–511CrossRefGoogle Scholar
  24. de Leval MR (1998) The Fontan circulation: what have we learned? What to expect? Pediatr Cardiol 19:316–320CrossRefGoogle Scholar
  25. Lewis G, Thorne S, Clift P, Holloway B (2015) Cross-sectional imaging of the Fontan circuit in adult congenital heart disease. Clin Radiol 70:667–675CrossRefGoogle Scholar
  26. Lu JC, Dorfman AL, Attili AK, Ghadimi Mahani M, Dillman JR, Agarwal PP (2012) Evaluation with cardiovascular MR imaging of baffles and conduits used in palliation or repair of congenital heart disease. Radiographics 32:E107–E127CrossRefGoogle Scholar
  27. McCrindle BW, Manlhiot C, Cochrane A et al (2013) Factors associated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure. J Am Coll Cardiol 61:346–353CrossRefGoogle Scholar
  28. Norwood WI, Jacobs ML (1993) Fontan’s procedure in two stages. Am J Surg 166:548–551CrossRefGoogle Scholar
  29. Ohye RG, Schranz D, D’Udekem Y (2016) Current therapy for hypoplastic left heart syndrome and related single ventricle lesions. Circulation 134:1265–1279CrossRefGoogle Scholar
  30. Pushparajah K, Tzifa A, Bell A et al (2015) Cardiovascular magnetic resonance catheterization derived pulmonary vascular resistance and medium-term outcomes in congenital heart disease. J Cardiovasc Magn Reson 17:28CrossRefGoogle Scholar
  31. Rathod RH, Prakash A, Powell AJ, Geva T (2010) 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 55:1721–1728CrossRefGoogle Scholar
  32. Rathod RH, Prakash A, Kim YY et al (2014) Cardiac magnetic resonance parameters predict transplantation-free survival in patients with fontan circulation. Circ Cardiovasc Imaging 7:502–509CrossRefGoogle Scholar
  33. Rodbard S, Wagner D (1949) By-passing the right ventricle. Proc Soc Exp Biol Med 71:69CrossRefGoogle Scholar
  34. Rychik J (2016) The relentless effects of the Fontan paradox. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 19:37–43CrossRefGoogle Scholar
  35. Sandler KL, Markham LW, Mah ML, Byrum EP, Williams JR (2014) Optimizing CT angiography in patients with Fontan physiology: single-center experience of dual-site power injection. Clin Radiol 69:e562–e567CrossRefGoogle Scholar
  36. Schwartz MC, Sullivan L, Cohen MS et al (2012) Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study. J Thorac Cardiovasc Surg 143:904–909CrossRefGoogle Scholar
  37. Soler R, Rodriguez E, Alvarez M, Raposo I (2007) Postoperative imaging in cyanotic congenital heart diseases: part 2 complications. AJR Am J Roentgenol 189:1361–1369CrossRefGoogle Scholar
  38. Spray TL (2013) Hemi-Fontan procedure. Oper Tech Thorac Cardiovasc Surg 18:124–137CrossRefGoogle Scholar
  39. Srivastava D, Preminger T, Lock JE et al (1995) Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 92:1217–1222CrossRefGoogle Scholar
  40. Talwar S, Nair VV, Choudhary SK, Airan B (2014) The Hemi-Fontan operation: a critical overview. Ann Pediatr Cardiol 7:120–125CrossRefGoogle Scholar
  41. Whitehead KK, Gillespie MJ, Harris MA, Fogel MA, Rome JJ (2009) Noninvasive quantification of systemic-to-pulmonary collateral flow: a major source of inefficiency in patients with superior cavopulmonary connections. Circ Cardiovasc Imaging 2:405–411CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Lucia Flors
    • 1
    • 2
    Email author
  • Patrick T. Norton
    • 1
  • Klaus D. Hagspiel
    • 1
  1. 1.Department of Radiology and Medical ImagingUniversity of Virginia Health SystemCharlottesvilleUSA
  2. 2.Department of RadiologyUniversity of Missouri Health SystemColumbiaUSA

Personalised recommendations