Abstract
Background
Patients who have undergone the Fontan operation for palliation of congenital heart disease with single-ventricle pathophysiology are at high risk for developing progressive liver fibrosis. Pathological assessment from percutaneous liver biopsy is central to the management of Fontan-associated liver disease, but liver biopsy in this vulnerable population poses unique challenges and potential risks.
Objective
This retrospective study describes our experience with percutaneous liver biopsy performed to assess changes of Fontan-associated liver disease, with particular regard to procedural outcomes.
Materials and methods
Data from liver biopsy procedure reports, pathology reports, cardiac angiography pressure measurements and laboratory values of patients with single ventricle heart disease after the Fontan operation who underwent ultrasound-guided percutaneous liver biopsy performed in interventional radiology at a pediatric tertiary care center during a 3-year period were retrospectively analyzed.
Results
Sixty-eight liver biopsies were performed in 67 patients (mean age: 20.2 years, range: 7.2–39 years). The technical success rate was 100%, and tissue was adequate for assessing liver disease in 100% of the procedures, including biopsies performed with a single pass. Anticoagulation was routinely suspended before biopsy, and no cardiac complications were encountered due to this suspension. A coaxial biopsy system using an 18-gauge (G) full-core instrument through a 17-G introducer trocar was most commonly used, in 41/68 cases (60%). The most common trough length was 2.3 cm, used in 37 cases (54%). One pass was made in 27 procedures (40%) and two passes in 30 (44%); tract embolization with gelatin sponge was performed in 52 (76%). The only complication was hemorrhage, which occurred in 5/68 (7.4%) of the biopsies, minor in four (5.9%) and major in one (1.5%) -- similar to rates reported for liver biopsy in non-Fontan patients. Hemorrhage had a delayed presentation in three of these five cases. Immediate post-biopsy hemoglobin decrease of ≥2 mg/dL showed a low sensitivity for hemorrhage. The mean Fontan pressure measured during cardiac angiography was 13.8 mmHg, and shunt pressures were not associated with an increased risk of hemorrhage.
Conclusion
Percutaneous liver biopsy in Fontan patients can be performed safely with high technical success rates and without increased complication rates. Meticulous technique and close observation are recommended to reduce post-biopsy complications. The degree of right heart pressure elevation was not associated with hemorrhage.
Similar content being viewed by others
References
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
Rogers LS, Glatz AC, Ravishankar C et al (2012) 18 years of the Fontan operation at a single institution: results from 771 consecutive patients. J Am Coll Cardiol 60:1018–1025
Rychik J, Veldtman G, Rand E et al (2012) The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Pediatr Cardiol 33:1001–1012
Goldberg DJ, Surrey LF, Glatz AC et al (2017) Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study. J Am Heart Assoc 6:1–8
DiPaola FW, Schumacher KR, Goldberg CS et al (2016) Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol 27:2434–2442
Wallihan DB, Podberesky DJ (2013) Hepatic pathology after Fontan palliation: Spectrum of imaging findings. Pediatr Radiol 43:330–338
Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757
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
Guha IN, Bokhandi S, Ahmad Z et al (2013) Structural and functional uncoupling of liver performance in the Fontan circulation. Int J Cardiol 164:77–81
Friedrich-Rust M, Koch C, Rentzsch A et al (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
Romero R (2013) Liver in congenital heart disease: implications of the fontan procedure for pediatric and adult liver specialists. Clin Liver Dis 2:210–214
Rockey DC, Caldwell SH, Goodman ZD et al (2009) Liver biopsy. Hepatology 49:1017–1044
Sacks D, McClenny TE, Cardella JF, Lewis CA (2003) Society of Interventional Radiology clinical practice guidelines. J Vasc Interv Radiol 14:S199–S202
Lungren MP, Lindquester WS, Seidel FG et al (2016) Ultrasound-guided liver biopsy with gelatin sponge pledget tract embolization in infants weighing less than 10 kg. J Pediatr Gastroenterol Nutr 63:e147–e151
Wolff D, van Melle JP, Ebels T et al (2014) Trends in mortality (1975-2011) after one- and two-stage Fontan surgery, including bidirectional Glenn through Fontan completion. Eur J Cardiothorac Surg 45:602–609
Mair DD, Puga FJ, Danielson GK (2001) The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 37:933–939
d’Udekem Y, Cheung MM, Setyapranata S et al (2009) How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias. Ann Thorac Surg 88:1961–1969
Rychik J (2016) The relentless effects of the Fontan paradox. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 19:37–43
Johnson JA, Cetta F, Graham RP et al (2013) Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis. J Thorac Cardiovasc Surg 146:140–145
Baek JS, Bae EJ, Ko JS et al (2010) Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors. Heart 96:1750–1755
Kutty SS, Peng Q, Danford DA et al (2014) Increased hepatic stiffness as consequence of high hepatic afterload in the Fontan circulation: a vascular doppler and elastography study. Hepatology 59:251–260
Eipel C, Abshagen K, Vollmar B (2010) Regulation of hepatic blood flow: the hepatic arterial buffer response revisited. World J Gastroenterol 16:6046–6057
Sundareswaran KS, Kanter KR, Kitajima HD et al (2006) Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study. Ann Thorac Surg 82:1267–1275
Tsochatzis EA, Gurusamy KS, Ntaoula S et al (2011) Elastography for the diagnosis of severity of fibrosis in chronic liver disease: a meta-analysis of diagnostic accuracy. J Hepatol 54:650–659
Serai SD, Wallihan DB, Venkatesh SK et al (2014) Magnetic resonance elastography of the liver in patients status-post Fontan procedure: feasibility and preliminary results. Congenit Heart Dis 9:7–14
Sandrasegaran K, Akisik FM, Lin C et al (2009) Value of diffusion-weighted MRI for assessing liver fibrosis and cirrhosis. AJR Am J Roentgenol 193:1556–1560
Govender P, Jonas MM, Alomari AI et al (2013) Sonography-guided percutaneous liver biopsies in children. AJR Am J Roentgenol 201:645–650
Meng HC, Lin HC, Huang CC et al (1994) Transjugular liver biopsy: comparison with percutaneous liver biopsy. J Gastroenterol Hepatol 9:457–461
Tulin-Silver S, Obi C, Kothary N, Lungren MP (2018) Comparison of transjugular liver biopsy and percutaneous liver biopsy with tract embolization in pediatric patients. J Pediatr Gastroenterol Nutr 67:180–184
Sehmbhi M, Doufexi D, Schoutens L et al (2015) Transjugular liver biopsy versus percutaneous liver biopsy-indications, adequacy, quality of specimens and complications. Gut 64:A272
Hardman RL, Perrich KD, Silas AM (2011) Single-institution results of image-guided nonplugged percutaneous versus transjugular liver biopsy. Cardiovasc Intervent Radiol 34:369–375
Plecha DM, Goodwin DW, Rowland DY et al (1997) Liver biopsy: effects of biopsy needle caliber on bleeding and tissue recovery. Radiology 204:101–104
Tsang WK, Luk WH, Lo A (2014) Ultrasound-guided plugged percutaneous biopsy of solid organs in patients with bleeding tendencies. Hong Kong Med J 20:107–112
Fandrich CA, Davies RP, Hall PM (1996) Small gauge gelfoam plug liver biopsy in high risk patients: safety and diagnostic value. Australas Radiol 40:230–234
Kamphuisen PW, Wiersma TG, Mulder CJ, de Vries RA (2002) Plugged-percutaneous liver biopsy in patients with impaired coagulation and ascites. Pathophysiol Haemost Thromb 32:190–193
Riddle C, Ahmed B, Doyle J, Connolly BL (2008) Migration of Gelfoam to the gallbladder after liver biopsy. Pediatr Radiol 38:806–809
Robbins KA, Keet CA (2015) Intraoperative anaphylaxis likely due to Gelfoam in a pediatric patient undergoing liver biopsy. Ann Allergy Asthma Immunol 114:531–533
Patel IJ, Davidson JC, Nikolic B et al (2013) Addendum of newer anticoagulants to the SIR consensus guideline. J Vasc Interv Radiol 24:641–645
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
None
Rights and permissions
About this article
Cite this article
Srinivasan, A., Guzman, A.K., Rand, E.B. et al. Percutaneous liver biopsy in Fontan patients. Pediatr Radiol 49, 342–350 (2019). https://doi.org/10.1007/s00247-018-4311-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-018-4311-9