Alarming rate of liver cirrhosis after the small conduit Extracardiac Fontan. A comparative analysis with the Lateral Tunnel.
Presented During:
Sunday, April 28, 2024: 11:43AM - 11:50AM
Metro Toronto Convention Center
Posted Room Name:
Room 716
Abstract No:
149
Submission Type:
Abstract Submission
Authors:
Eiri Kisamori (1), Alyssia Venna (1), Rahul Rajeev (1), Manan Desai (1), Aybala Tongut (1), Rittal Mehta (1), Sarah Clauss (1), Can Yerebakan (1), Yves d’Udekem (1)
Institutions:
(1) Children's National Hospital, Washington, DC
Submitting Author:
Eiri Kisamori
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Children's National Hospital
Children's National Hospital
Co-Author(s):
Alyssia Venna
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Children's National Hospital
Children's National Hospital
Rahul Rajeev
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Children's National Hospital
Children's National Hospital
Manan Desai
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Children's National Hospital
Children's National Hospital
Aybala Tongut
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Children's National Hospital
Children's National Hospital
Rittal Mehta
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Children's National Hospital
Children's National Hospital
Sarah Clauss
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Children's National Hospital
Children's National Hospital
*Can Yerebakan
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Children's National Hospital
Children's National Hospital
*Yves d’Udekem
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Children's National Hospital
Children's National Hospital
Presenting Author:
Eiri Kisamori
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Okayama University Hospital
Okayama University Hospital
Abstract:
Objective: Liver cirrhosis is a well-recognized complication following the Fontan procedure. However, 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 between November 1989 and July 2021. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) were pooled with those having extracardiac conduit (ECC) (134) as the conduit went from the IVC to the pulmonary arteries. The 260 patients who underwent the ECC and the 68 who had the lateral tunnel (LT) Fontan constitute the core of the study. Diagnosis of liver cirrhosis was based on histopathology from liver biopsy or elastography.
Results: Patients who underwent the LT and the ECC Fontan shared the same characteristics. Median age at the Fontan procedure was 23.7 months (interquartile range [IQR]: 20.8-32.6) in the LT group, compared to 28.8 months (IQR: 24.6-39.5) in the ECC group (p<0.01). F
enestration rates were 96% (65) in the LT group and 84.6% (220) in the ECC group. The conduit size was reported in 258 patients: 10 mm (1); 12 mm (2); 14 mm (25);16 mm (187); 18 mm (9); 19 mm (24) and 20 mm (10). The median follow-up was 14.8 years (IQR: 12.5-16.5) in the lateral tunnel group and 7 years (IQR: 2.8-10.4) in the extracardiac conduit group. During the follow-up period, two patients (2.9%) with LT and 15 (5.8%) in the with ECC (9 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 2.4% in the lateral tunnel group, respectively, and 0%, 0.9%, 7.6% and 26.8% in the extracardiac conduit group (p<0.01 Figure). Rates of mortality, Fontan revision, Fontan takedown, and transplant were comparable between the two groups.
Conclusions: The Extracardiac Conduit Fontan seem to be associated with faster development of cirrhosis, especially in patients implanted with small conduits.
Methods: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution between November 1989 and July 2021. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) were pooled with those having extracardiac conduit (ECC) (134) as the conduit went from the IVC to the pulmonary arteries. The 260 patients who underwent the ECC and the 68 who had the lateral tunnel (LT) Fontan constitute the core of the study. Diagnosis of liver cirrhosis was based on histopathology from liver biopsy or elastography.
Results: Patients who underwent the LT and the ECC Fontan shared the same characteristics. Median age at the Fontan procedure was 23.7 months (interquartile range [IQR]: 20.8-32.6) in the LT group, compared to 28.8 months (IQR: 24.6-39.5) in the ECC group (p<0.01). F
enestration rates were 96% (65) in the LT group and 84.6% (220) in the ECC group. The conduit size was reported in 258 patients: 10 mm (1); 12 mm (2); 14 mm (25);16 mm (187); 18 mm (9); 19 mm (24) and 20 mm (10). The median follow-up was 14.8 years (IQR: 12.5-16.5) in the lateral tunnel group and 7 years (IQR: 2.8-10.4) in the extracardiac conduit group. During the follow-up period, two patients (2.9%) with LT and 15 (5.8%) in the with ECC (9 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 2.4% in the lateral tunnel group, respectively, and 0%, 0.9%, 7.6% and 26.8% in the extracardiac conduit group (p<0.01 Figure). Rates of mortality, Fontan revision, Fontan takedown, and transplant were comparable between the two groups.
Conclusions: The Extracardiac Conduit Fontan seem to be associated with faster development of cirrhosis, especially in patients implanted with small conduits.
CONGENTIAL:
Single Ventricle Management
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