Alejandro Angel Borquez, M.D.
,
Rady Children's Hospital, University of California San Diego, San Diego, CA
Jose Silva Sepulveda, M.D.
,
Rady Children's Hospital, University of California San Diego, San Diego, CA
Jesse Lee, M.D.
,
Children's Hospital of San Antonio, San Antonio, TX
Vera Vavinskaya, MD
,
University of California, San Diego, San Diego, CA
Irine Vodkin, MD
,
University of California, San Diego, San Diego, CA
Hannah El-Sabrout
,
Rady Children's Hospital, University of California San Diego, San Diego, CA
James C Perry, MD
,
Rady Children's Hospital, University of California San Diego, San Diego, CA
John W. Moore, M.D., FSCAI
,
Rady Children's Hospital, University of California San Diego, San Diego, CA
Howaida El-Said, M.D., Ph.D., FSCAI
,
University of California (San Diego) Program, San Diego, CA
Background:
Fontan associated liver disease (FALD) is common due to limitations of the Fontan circulation resulting in congestive hepatopathy. The technique & outcomes of transjugular liver biopsy (TLB) in Fontan patients warrant definition as part of a rigorous FALD surveillance program.
Methods:
4 year retrospective review of patients who had the Fontan operation & underwent TLB to evaluate for FALD. Biopsy site, technique, histology, angiography, hemodynamics, & complications were reviewed.
Results:
125 patients (56% male, 44% female) had a TLB from 2014-2018. Median age 16.4 years (2-50.5), median BMI 20.2 (IQR 16.6-24.6). Technical success was 100% & all samples were diagnostic (125/125). Median number of samples was 2 (range 2-4). 17% (21) were accessed via the left internal jugular (IJ) vein, mostly secondary to right IJ occlusion, hypoplasia or heterotaxy. No patients had superior compartment obstruction that prevented transjugular approach. 85% (242/286) of biopsies were from segments 5 or 8. 3.2% complication rate (4/125). Complications were early in the experience, including capsular perforation (2), renal hematoma (1) & hemobilia (1), all without long-term effect & all avoidable. With experience, need for entry & exit angiography was recognized, resulting in decreased complication rate.
Conclusions:
Transjugular liver biopsy is a safe & effective diagnostic tool for obtaining critical surveillance data for patients at risk for FALD. Vascular anomalies in Fontan physiology appear common enough to warrant pre-biopsy assessment. Complication rates are lower than described for other biopsy techniques in non-Fontan & Fontan patients.
