Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel

Eiri Kisamori; Alyssia Venna; Hamzah Ejaz Chaudhry; Manan Desai; Aybala Tongut; Rittal Mehta; Sarah Clauss; Can Yerebakan; Yves d'Udekem

doi:10.1016/j.jtcvs.2024.04.013

Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel

J Thorac Cardiovasc Surg. 2024 Apr 28:S0022-5223(24)00311-8. doi: 10.1016/j.jtcvs.2024.04.013. Online ahead of print.

Authors

Eiri Kisamori¹, Alyssia Venna¹, Hamzah Ejaz Chaudhry¹, Manan Desai¹, Aybala Tongut¹, Rittal Mehta¹, Sarah Clauss², Can Yerebakan¹, Yves d'Udekem³

Affiliations

¹ Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, Washington, DC.
² Division of Cardiology, Children's National Heart Institute, Children's National Hospital, Washington, DC.
³ Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, Washington, DC. Electronic address: yves.dudekem@childrensnational.org.

PMID: 38688450
DOI: 10.1016/j.jtcvs.2024.04.013

Abstract

Background: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear.

Methods: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study.

Results: Median age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups.

Conclusions: The extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Keywords: Fontan; extracardiac conduit; lateral tunnel; liver cirrhosis.