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Transjugular liver biopsy for Fontan associated liver disease surveillance: Technique, outcomes and hemodynamic
Alejandro A Borquez1, Jose Silva-Sepulveda1, Jesse W Lee1
1San Diego School of Medicine, Department of Pediatrics, Division of Pediatric Cardiology, University of California, USA.
Insights
Transjugular liver biopsies (TJLB) offer a safe and effective method for Fontan associated liver disease (FALD) surveillance. This technique correlates well with hemodynamic parameters, aiding in FALD management.
Area of Science:
- Cardiology
- Hepatology
- Interventional Radiology
Background:
- Fontan associated liver disease (FALD) arises from Fontan circulation limitations, causing congestive hepatopathy.
- Rigorous surveillance is crucial for managing FALD in Fontan patients.
- Transjugular liver biopsy (TJLB) is a potential surveillance tool.
Purpose of the Study:
- To define the technique and outcomes of TJLB in Fontan patients.
- To assess TJLB as part of a FALD surveillance program.
- To correlate biopsy findings with hemodynamic data.
Main Methods:
- A four-year review of 125 Fontan physiology patients undergoing TJLB.
- Analysis of biopsy site, technique, histology, angiography, hemodynamics, and complications.
- Correlation of modified Ishak congestive hepatic fibrosis (ICHF) scores with catheterization hemodynamics.
Main Results:
- 100% technical success with diagnostic samples in all patients.
- A 3.2% complication rate, primarily early in the experience and avoidable.
- Significant correlation found between ICHF score, mean Fontan pressure, time from Fontan, and cardiac index.
Conclusions:
- TJLB is a viable alternative for FALD surveillance with low complication rates.
- Vascular anomalies in Fontan physiology necessitate pre-biopsy assessment.
- TJLB findings correlate significantly with key hemodynamic and temporal factors.
Background:
Fontan associated liver disease (FALD) is attributed to the limitations of the Fontan circulation, resulting in congestive hepatopathy. The technique and outcomes of transjugular liver biopsies (TJLB) in Fontan patients warrant definition as part of a rigorous FALD surveillance program.
Method:
Four year review of patients with Fontan physiology who underwent a TJLB during surveillance catheterizations. Biopsy site, technique, histology, angiography, hemodynamics, and complications were reviewed to assess correlation of biopsy scores with simultaneously obtained catheterization hemodynamics.
Results:
125 patients with a TJLB from 10/1/14 to 5/1/18. Median age 17 years (2-50.5). Technical success 100% (125/125), all samples diagnostic. 17% (21) accessed via the left internal jugular vein, secondary to right IJ occlusion or Heterotaxy syndrome. No patients had superior compartment obstruction preventing transjugular approach. 3.2% complication rate (4/125). Complications were early in the experience, including capsular perforation (2), renal hematoma (1) and hemobilia (1), all without long-term effect and all avoidable. After standardized entry/exit angiography was adopted, no further complications noted. There is a significant correlation between the newly described modified Ishak congestive hepatic fibrosis (ICHF) score with mean Fontan pressure, time from Fontan and cardiac index.
Conclusions:
TJLB is an alternate method for obtaining critical FALD surveillance data, with lower complication rates that traditional techniques. Vascular anomalies in Fontan physiology appear common and warrant pre-biopsy assessment. There is a significant correlation between biopsy score, time from Fontan, mean Fontan pressure and cardiac index.
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