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Percutaneous liver biopsy in Fontan patients

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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.

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Authors and Affiliations

  1. Department of Radiology, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd., Radiology 3NW47, Philadelphia, PA, 19104, USA

    Abhay Srinivasan & Anne Marie Cahill

  2. Children Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA, USA

    Abhay Srinivasan, Anthony K. Guzman, Elizabeth B. Rand, Jack Rychik, David J. Goldberg, Pierre A. Russo & Anne Marie Cahill

  3. Department of Gastroenterology, Hepatology, and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Elizabeth B. Rand

  4. Department of Cardiology, University of Pennsylvania Peralman School of Medicine, Philadelphia, PA, USA

    Jack Rychik & David J. Goldberg

  5. Department of Pathology and Laboratory Medicine, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Pierre A. Russo

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  1. Abhay Srinivasan
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  2. Anthony K. Guzman
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  3. Elizabeth B. Rand
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  4. Jack Rychik
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Correspondence to Abhay Srinivasan.

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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

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  • DOI: https://doi.org/10.1007/s00247-018-4311-9

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