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MR assessment of abdominal circulation in Fontan physiology

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Abstract

The Fontan operation allows for longer survival of those born with functionally single ventricle physiology. Although it effectively eliminates cyanosis, increased systemic venous pressure is an unavoidable consequence and low cardiac output is frequent. The abdomen is particularly vulnerable to these alterations in hemodynamics because the hepatic blood flow consists predominantly of portal venous flow, which relies on a low pressure gradient between the portal and hepatic veins. Therefore, any subtle increase in systemic venous pressure will adversely affect the hemodynamic balance of the liver as well as the intestine. As the clinical manifestations and routine laboratory findings of abdominal complications can lag behind the hemodynamic and pathologic changes in the abdominal organs, regular imaging surveillance is critical. Magnetic resonance (MR) provides excellent visualization of both cardiovascular and abdominal systems. It provides robust anatomic and hemodynamic data which can be used for timely implementation of treatment options. In this review paper, we discuss the pathogenesis and MR findings of abdominal complications following the Fontan operation. Specifically we explore the utility of phase-contrast MR for assessment of the abdominal circulation in children following the Fontan palliation.

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References

  1. Rychik J (2010) Forty years of the Fontan operation: a failed strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 13:96–100

    Article  PubMed  Google Scholar 

  2. Goldberg DJ, Dodds K, Rychik J (2010) Rare problems associated with the Fontan circulation. Cardiol Young 20:113–119

    Article  PubMed  Google Scholar 

  3. Rogers LS, Glatz AC, Ravishankar C, Spray TL, Nicolson SC, Rychuk J, Rush CH, Gaynor JW, Goldberg DJ (2012) 18 years of the Fontan operation at asingle institution. Results from 771 consecutive patients. J Am Coll Cardiol 60:1018–1025

    Article  PubMed  Google Scholar 

  4. Eipel C, Abshagen K, Vollmar B (2010) Regulation of hepatic blood flow: the hepatic arterial buffer response revisited. World J Gastroenterol 16:6046–6057

    Article  PubMed Central  PubMed  Google Scholar 

  5. Rychik J, Gui-Yang S (2002) Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy. Am J Cardiol 90:672–674

    Article  PubMed  Google Scholar 

  6. Rychik J (2007) Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2:288–300

    Article  PubMed  Google Scholar 

  7. Asrani SK, Asrani NA, Freese DK, Phillips S, Warnes CA, Heimbach J, Kamath PS (2012) Congenital heart disease and the liver. Hepatology 56:1160–1169

    Article  PubMed  Google Scholar 

  8. Wu FM, Ukomadu C, Odze RD, Valente AM, Mayer JE Jr, Earing MG (2011) Liver disease in the patient with fontan circulation. Congenit Heart Dis 6:190–201

    Article  PubMed  Google Scholar 

  9. Meadows J, Jenkins K (2011) Protein-losing enteropathy: integrating a new disease paradign into recommendations for prevention and treatment. Cardiol Young 21:363–377

    Article  PubMed  Google Scholar 

  10. Johnson JN, Driscoll DJ, O’Leary PW (2012) Protein-losing enteropathy and Fontan operation. Nutr Clin Pract 27:375–384

    Article  PubMed  Google Scholar 

  11. 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 129:1348–1352

    Article  PubMed  Google Scholar 

  12. Kiesewetter CH, Sheron N, Vettukattill JJ, Hacking N, Stedman B, Millward-Sadler H et al (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584

    Article  PubMed Central  PubMed  Google Scholar 

  13. Johnson JA, Cetta F, Graham RP, Smyrk TC, Driscoll DJ, Phillips SD, John AS (2013) Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis. J Thorac Cardiovasc Surg 146:140–145

    Article  PubMed  Google Scholar 

  14. Schwartz MC, Sullivan LM, Glatz AC, Rand E, Russo P, Goldberg DJ, Rome JJ, Cohen MS (2013) Portal and sinusoidal fibrosis are common on liver biopsy after Fontan surgery. Pediatr Cardiol 34:135–142

    Article  PubMed  Google Scholar 

  15. Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D’Antiga L (2008) Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg 86:177–182

    Article  PubMed  Google Scholar 

  16. Baek JS, Bae EJ, Ko JS, Kim GB, Kwon BS, Lee SY, Noh CI, Park EA, Lee W (2010) Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors. Heart 96:1750–1755

    Article  CAS  PubMed  Google Scholar 

  17. Bryant T, Ahmad Z, Millward-Sadler H, Burney K, Stedman B, Kendall T, Vettukattil J, Haw M, Salmon AP, Cope R, Hacking N, Breen D, Sheron N, Veldtman GR (2010) Arterialised hepatic nodules in the Fontan circulation: hepatico–cardiac interactions. Int J Cardiol 151:268–272

    Article  PubMed  Google Scholar 

  18. Wanless IR, Callea F, Craig JR, et al (International Working Party) (1995) Terminology of nodular hepatocellular lesions. Hepatology 22:983–993

    Google Scholar 

  19. Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencione R (2002) Large regenerative nodules in Budd–Chiari syndrome and other vascular disorders of the liver: CT and MR imaging findings with clinicopathologic correlation. Am J Roentgenol 178:877–883

    Article  Google Scholar 

  20. Saliba T, Dorkhom S, O’Reilly EM, Ludwig E, Gansukh B, Abou-Alfa GK (2010) Hepatocellular carcinoma in two patients with cardiac cirrhosis. Eur J Gastroenterol Hepatol 22:889–891

    Article  PubMed  Google Scholar 

  21. Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757

    Article  CAS  PubMed  Google Scholar 

  22. Elder RW, Parekh S, Book WM (2013) More on hepatocellular carcinoma after the Fontan procedure. N Engl J Med 369:490

    Article  CAS  PubMed  Google Scholar 

  23. Wallihan DB, Podberesky DJ (2013) Hepatic pathology after Fontan palliation: spectrum of imaging findings. Pediatr Radiol 43:330–338

    Article  PubMed  Google Scholar 

  24. Bulut OP, Romero R, Mahle WT, McConnell M, Braithwaite K, Shehata BM, Gupta NA, Vos M, Alazraki A (2013) Magnetic resonance imaging identifies unsuspected liver abnormalities in patients after the Fontan procedure. J Pediatr 163:201–206

    Article  PubMed  Google Scholar 

  25. Elder RW, McCabe NM, Hebson C, Veledar E, Romero R, Ford RM, Mahle WT, Kogon BE, Sahu A, Jokhadar M, McConnell ME, Book WM (2013) Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol 168:3764–3769

    Article  PubMed  Google Scholar 

  26. Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M (1998) Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 115:1063–1073

    Article  CAS  PubMed  Google Scholar 

  27. Ohuchi H, Yasuda K, Miyazaki A, Kitano M, Sakaguchi H, Yazaki S, Tsuda E, Yamada O (2013) Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation. Eur J Cardiothorac Curg 43:e49–e57

    Article  Google Scholar 

  28. Silvilairat S, Cabalka AK, Cetta F, Grogan M, Hagler DJ, O’Leary PW (2008) Protein-losing enteropathy after the Fontan operation: associations and predictors of clinical outcome. Congenit Heart Dis. 3:262–268

    Article  PubMed  Google Scholar 

  29. Menon S, Hagler D, Cetta F, Gloviczki P, Driscoll D (2008) Role of caval venous manipulation in treatment of protein-losing enteropathy. Cardiol Young 18:275–281

    PubMed  Google Scholar 

  30. Horai T, Bhama JK, Fontes PA, Toyoda Y (2011) Combined heart and liver transplantation in a patient with situs ambiguous. Ann Thorac Surg 91:600–601. doi:10.1016/j.athoracsur.2010.07.078

    Article  PubMed  Google Scholar 

  31. Vallabhajosyula P, Komlo C, Wallen TJ, Olthoff K, Pochettino A (2013) Combined heart–liver transplant in a situs-ambiguous patient with failed Fontan physiology. J Thorac Cardiovasc Surg 145:e39–e41

    Article  PubMed  Google Scholar 

  32. Guha IN, Bokhandi S, Ahmad Z, Sheron N, Cope R, Marshall C, Veldtman G (2013) Structural and functional uncoupling of liver performance in the Fontan circulation. Int J Cardiol 164:77–81

    Article  PubMed  Google Scholar 

  33. Kaulitz R, Haber P, Strum E, Hofbeck M. Serial evaluation of hepatic function profile after Fontan operation. Herz 2013 [Epub ahead of print]

  34. Goldberg DJ, Dodds K, Rychik J (2011) New concepts: development of a survivorship programme for patients with a functionally univentricular heart. Cardiol Young 21:77–79

    Article  PubMed  Google Scholar 

  35. Rychik J, Veldtman G, Rand E, Russo P, Rome JJ, Krok K, Goldberg DJ, Cahill AM, Wells RG (2012) The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Pediatr Cardiol 33:1001–1012

    Article  PubMed Central  PubMed  Google Scholar 

  36. Van der Hulst AE, Roest AAW, Westenberg JJM, Kroft LJM, de Roos A (2012) Cardiac MRI in postoperative congenital heart disease patients. J Magn Reson Imaging 36:511–528

    Article  PubMed  Google Scholar 

  37. Fogel MA, Khiabani RH, Yoganathan A (2013) Imaging for preintervention planning. Pre- and post-Fontan procedures. Circulation Cardiovasc. Imaging 6:1092–1101

    Google Scholar 

  38. Khana G, Bhalla S, Krishnamurthy R, Canter C (2012) Extracarddiac complications of the Fontan circuit. Pediatr Radiol 42:233–241

    Article  Google Scholar 

  39. Giamberti A, Anderson RH, de Leval MR (2002) Intrahepatic right-to-left shunting after the Fontan operation. Cardiol Young 12:308–310

    Article  PubMed  Google Scholar 

  40. Friedrich-Rust M, Koch C, Rentzsch A, Sarrazin C, Schwarz P, Herrmann E, Lindinger A, Sarrazin U, Poynard T, Schäfers HJ, Zeuzem S, Abdul-Khaliq H (2008) Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers. J Thorac Cardiovasc Surg 135:560–567

    Article  PubMed  Google Scholar 

  41. Serai SD, Wallihan DB, Venkatesh SK, Ehman RL, Campbell KM, Sticka J, Marino BS, Podberesky DJ (2014) Magnetic resonance elastography of the liver in patients status-post Fontan procedure: feasibility and preliminary results. Congenit Heart Dis 9:7–14

    Article  PubMed  Google Scholar 

  42. Kutty SS, Peng Q, Danford DA, Fletcher SE, Perry D, Talmon GA, Scott C, Kugler JD, Duncan KF, Quiros-Tejeira RE, Kutty S (2014) Increased hepatic stiffness as consequence of high hepatic afterload in the Fontan circulation: a vascular Doppler and elastography study. Hepatology 59:251–260

    Article  PubMed  Google Scholar 

  43. Sandrasegaran K, Akisik FM, Lin C, Tahir B, Rajan J, Saxena R, Aisen AM (2009) Value of diffusion-weighted MRI for assessing liver fibrosis and cirrhosis. AJR Am J Roentgenol 193:1556–1560

    Article  PubMed  Google Scholar 

  44. Oommen R, Kurien G, Balakrishnan N, Narasimhan S (1992) Tc-99 m albumin scinitgraphy in the localization of protein loss in the gut. Clin Nucl Med 17:787–788

    Article  CAS  PubMed  Google Scholar 

  45. Bhatnagar A, Kashyap R, Chauhan UPS, Mishra P, Choppa MK, Khanna CM (1995) Diagnosing protein losing enteropathy. A new approach using Tc-99 m human immunoglobulin. Clin Nucl Med 20:969–972

    Article  CAS  PubMed  Google Scholar 

  46. Bhatnagar A, Lahoti D, Singh AK, Shankar LR, Sharma B, Singh T (1995) Scintigraphic diagnosis of protein losing enteropathy using Tc-99 m dextran. Clin Nucl Med 20:1070–1073

    Article  CAS  PubMed  Google Scholar 

  47. Liu NF, Lu Q, Wang CG, Zhou JG (2008) Magnetic resonance imaging as a new method to diagnose protein losing enteropathy. Lymphology 41:111–115

    PubMed  Google Scholar 

  48. Lotz J, Meier C, Leppert A, Galanski M (2002) Cardiovascular flow measurement with phase-contrast MR imaging: basic facts and implementation. Radiographics 22:651–671

    Article  PubMed  Google Scholar 

  49. Powell AJ, Maier SE, Chung T, Geva T (2000) Phase-velocity cine magnetic resonance imaging measurement of pulsatile blood flow in children and young adults: in vitro and in vivo validation. Pediatr Cardiol 21:104–110

    Article  CAS  PubMed  Google Scholar 

  50. Goo HW, Al-Otay A, Grosse-Wortmann L, Wu S, Macgowan CK, Yoo SJ (2009) Phase-contrast magnetic resonance quantification of normal pulmonary venous return. J Magn Reson Imaging 29:588–594

    Article  PubMed  Google Scholar 

  51. Tariq U, Hsiao A, Alley M, Zhang T, Lustig M, Vasanawala SS (2013) Venous and arterial flow quantification are equally accurate and precise with parallel imaging compressed sensing 4D phase contrast MRI. J Magn Reson Imaging 37:1419–1426

    Article  PubMed Central  PubMed  Google Scholar 

  52. Bächler P, Valverde I, Pinochet N, Nordmeyer S, Kuehne T, Crelier G, Tejos C, Irarrazaval P, Beerbaum P, Uribe S (2013) Caval blood flow distribution in patients with Fontan circulation: quantification by using particle traces from 4D flow MR imaging. Radiology 267:67–75

    Article  PubMed  Google Scholar 

  53. Nilsson A, Bloch KM, Töger J, Heiberg E, Ståhlberg F (2013) Accuracy of four-dimensional phase-contrast velocity mapping for blood flow visualizations: a phantom study. Acta Radiol 54:663–671

    Article  PubMed  Google Scholar 

  54. Wentland AL, Grist TM, Wieben O (2013) Repeatability and internal consistency of abdominal 2D and 4D phase contrast MR flow measurements. Acad Radiol 20:699–704

    Article  PubMed Central  PubMed  Google Scholar 

  55. Stankovic Z, Csatari Z, Deibert P, Euringer W, Blanke P, Kreisel W (2012) Abdullah Zadeh Z, Kallfass F, Langer M, Markl M. Normal and altered three-dimensional portal venous hemodynamics in patients with liver cirrhosis. Radiology 262:862–873

    Article  PubMed  Google Scholar 

  56. Stankovic Z, Csatari Z, Deibert P, Euringer W, Jung B, Kreisel W, Geiger J, Russe MF, Langer M, Markl M (2013) A feasibility study to evaluate splanchnic arterial and venous hemodynamics by flow-sensitive 4D MRI compared with Doppler ultrasound in patients with cirrhosis and controls. Eur J Gastroenterol Hepatol 25:669–675

    Article  PubMed  Google Scholar 

  57. Stankovic Z, Jung B, Collins J, Russe MF, Carr J, Euringer W, Stehlin L, Csatari Z, Strohm PC, Langer M, Markl M. Reproducibility study of four-dimensional flow MRI of arterial and portal venous liver hemodynamics: Influence of spatio-temporal resolution. Magn Reson Med 2013 [Epub ahead of print]

  58. Landgraf BR, Johnson KM, Roldán-Alzate A, Francois CJ, Wieben O, Reeder SB. Effect of temporal resolution on 4D flow MRI in the portal circulation. Magn Reson Imaging 2013 [Epub ahead of print]

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Yoo, SJ., Prsa, M., Schantz, D. et al. MR assessment of abdominal circulation in Fontan physiology. Int J Cardiovasc Imaging 30, 1065–1072 (2014). https://doi.org/10.1007/s10554-014-0424-x

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