第56回日本小児循環器学会総会・学術集会

講演情報

AEPC-YIA Session

AEPC-YIA Session(I-YIA)

2020年11月22日(日) 09:30 〜 10:00 Track1

座長:小野 博(国立成育医療研究センター 循環器科)

[I-YIA-1] Hepatic Venous Oxygen Saturation As a Novel Marker for Fontan Associated Liver Disorder

Kanchi Saito, Hirofumi Saiki, Yurie Takizawa, Satoshi Nakano, Shin Takahashi, Kotaro Oyama (Department of Pediatrics, Iwate Medical University School of Medicine)

Introduction
Fontan associated liver disorder (FALD) is one of critical complications after Fontan procedure. Despite advancement of imaging and molecular technologies, prospective studies failed to identify sensitive biomarker to detect subclinical FALD, where its prevalence is estimated more than 80% in 10 years. As the main source of liver blood supply changes from portal vein (low oxygen saturation:SO2) to hepatic artery (high SO2) with the progression of hepatic fibrosis, we hypothesized that the hepatic venous SO2 increased with the development of liver fibrosis.
Methods
During catheterization, hemodynamics as well as hepatic circulation property including transhepatic pressure were assessed in consecutive 117 Fontan and 86 non-Fontan patients. Multiple markers for liver fibrosis were measured and their relationship with hemodynamic properties was analyzed.
Results
As compared with non-Fontan patients, Fontan patients had low cardiac output (CI), high central venous, hepatic venous, and hepatic wedge pressures (p values for all, <0.001), whereas transhepatic pressure was similar. As hepatic venous (HV) as well as inferior venous cava (IVC) SO2 were dependent on arterial SO2 and CI, SO2 ratio of HV/IVC (SR-HV/IVC), which was independent of them, was analyzed. The SR-HV/IVC in the Fontan patients was markedly lower than non-Fontan patients, suggesting unfavorable liver perfusion in the Fontan patients. Interestingly, although the SR-HV/IVC in the non-Fontan patients was consistent regardless of their age (SO2 HV/IVC= 0.90-0.001*age, p=0.83), it became markedly low at 1year of Fontan procedure in the Fontan patients, with subsequent increase to the level of non-Fontan patients after 10 years of Fontan procedure (SO2 HV/IVC= 0.67+0.01*age, p=0.0023), in compliance with the reported prevalence of subclinical FALD. Importantly, SRHV/IVC in the Fontan patients was negatively correlated with platelet counts (p=0.026) and albumin/IgG ratio (p=0.0035), and positively correlated with serum levels of total-bilirubin (p=0.037) and hyaluronic acid (p=0.055).
Conclusions
SR-HV/IVC, which supposed to reflect pathological feature of hepatic vascular remodeling, is a novel biomarker for subclinical FALD. Prospective studies to investigate utility of SR-HV/IVC to guide prevention of FALD would be warranted.