Budd-Chiari Syndrome (BCS) is a complex and severe vascular liver disease that poses significant challenges to patients' health and survival. Transjugular intrahepatic portosystemic shunt (TIPS), a minimally invasive interventional procedure, has emerged as a promising treatment option for BCS in recent years. In this feature, Dr. Xuefeng Luo from West China Hospital of Sichuan University provides an in-depth review of a study published in Liver Transplantation, which evaluates the effectiveness and safety of TIPS in patients with BCS.Overview of the Study
BCS is a rare vascular disorder of the liver, characterized by obstruction of hepatic venous outflow, leading to portal hypertension and hepatic congestion. This potentially life-threatening condition presents with a broad spectrum of clinical manifestations, ranging from acute liver failure to chronic liver disease or even asymptomatic cases.
Anticoagulation is the cornerstone of treatment, often combined with diuretics. However, when these treatments fail to provide adequate control, interventional procedures such as TIPS are considered to restore blood flow or alleviate complications associated with portal hypertension. For patients with decompensated cirrhosis or acute liver failure, liver transplantation remains the definitive intervention.
To date, no studies have assessed whether TIPS can reduce mortality among BCS patients awaiting liver transplantation or decrease the urgency of transplantation. A study published in Liver Transplantation in 2024, titled “Impact of Transjugular Intrahepatic Portosystemic Shunt on Mortality and Urgency of Liver Transplantation in Budd-Chiari Syndrome Patients”, aimed to analyze the characteristics of BCS patients awaiting liver transplantation and those who had already undergone the procedure. The study also evaluated the effect of TIPS on mortality rates and the urgency of transplantation among those on the waiting list.
Key Findings from the Study
Using data from the United Network for Organ Sharing (UNOS), Akabane et al. identified 815 adult BCS patients between February 2002 and March 2024. Among them, 263 patients, representing 32.3% of the total cohort, underwent TIPS. The study found that patients who received TIPS had a lower liver transplant rate of 43.3% compared to 56.5% in those who did not receive TIPS. The waiting time was significantly longer for TIPS recipients, with a median of 350 days compared to 113 days for those who did not undergo the procedure.
Among the 426 liver transplant recipients, 134, or 31.5%, had previously undergone TIPS. Patients who received TIPS before transplantation had significantly lower MELD-Na scores, averaging 24 compared to 27 in non-TIPS patients. The proportion of early-phase patients from the 2002 to 2011 period was also lower in the TIPS group at 3.7%, compared to 12.3% in non-TIPS patients. Additionally, ICU admission rates were lower in TIPS patients at 14.9%, compared to 21.9% in the non-TIPS group. The waiting period before transplantation was also notably longer, with a median of 97 days for TIPS patients compared to just 26 days for those who did not receive TIPS.
These findings indicate that TIPS significantly reduces mortality and the urgency of liver transplantation in BCS patients, serving as an effective bridge to transplantation.
Detailed Analysis and Clinical Implications
The study included 815 BCS patients, with 263 having undergone TIPS and 552 who had not. TIPS recipients had lower MELD-Na scores, with a median of 20 compared to 22 in non-TIPS patients. The proportion of early-phase patients from 2002 to 2011 was lower among TIPS recipients at 2.7%, compared to 8.3% in those who did not undergo the procedure. Patients treated with TIPS had a lower overall liver transplant rate of 43.3%, while non-TIPS patients had a transplant rate of 56.5%. The waiting period for liver transplantation was significantly longer for those who had undergone TIPS, with a median of 350 days compared to 113 days in non-TIPS patients.
Among those who ultimately received liver transplants, patients who had undergone TIPS had a lower MELD-Na score at the time of transplantation, averaging 24 compared to 27 in non-TIPS patients. The proportion of early-phase patients from 2002 to 2011 was lower at 3.7% among TIPS patients, compared to 12.3% in those who did not undergo the procedure. ICU admission rates were also lower among TIPS patients at 14.9%, compared to 21.9% in the non-TIPS group. The median waiting time for transplantation was longer for TIPS patients at 97 days, while non-TIPS patients had a median wait of only 26 days.
Comparing early-phase patients from 2002 to 2011 with later-phase patients from 2012 to 2021, there was no significant difference in post-transplant survival rates at one, five, and ten years. One-year survival rates were 85.9% in early-phase patients and 90.7% in later-phase patients. Five-year survival rates were similar, with 79.1% in early-phase patients compared to 79.3% in later-phase patients. At ten years, survival rates were 70.5% in early-phase patients and 64.7% in later-phase patients. Graft survival rates followed a similar trend, with no significant differences between early- and later-phase patients.
Patients who had undergone TIPS before transplantation showed no significant difference in five-year or ten-year survival rates compared to those who had not received TIPS. Five-year survival rates were 80.8% in TIPS patients and 79.1% in non-TIPS patients, while ten-year survival rates were 71.8% and 69.1%, respectively. Graft survival rates at five years were 80.8% in TIPS patients compared to 75.2% in non-TIPS patients. At ten years, graft survival rates were 74.1% in TIPS patients and 64.3% in non-TIPS patients.
Multivariate analysis identified key factors that increased mortality among patients awaiting liver transplantation. Stage 3 to 4 hepatic encephalopathy significantly increased the risk of death, with a subdistribution hazard ratio (sHR) of 4.37. Higher MELD-Na scores were associated with an increased mortality risk, with an sHR of 1.09. Older age was also a significant factor, with an sHR of 1.03, as was a higher BMI, which had an sHR of 1.05.
TIPS was identified as a significant factor in reducing mortality among transplant candidates, with an sHR of 0.46, demonstrating its role in improving survival rates. Additionally, TIPS significantly reduced the urgency of liver transplantation, with an sHR of 0.65.
Conclusion and Future Perspectives
This study demonstrates that while TIPS does not significantly impact post-transplant survival, it plays a crucial role in reducing mortality and the urgency of liver transplantation for BCS patients on the waiting list. TIPS serves as an effective bridging therapy for liver transplantation, allowing patients to remain stable for longer periods while awaiting a suitable organ.
However, the study has certain limitations, highlighting the need for further prospective research to comprehensively assess the impact of TIPS on BCS patients awaiting transplantation. Future studies should focus on identifying the optimal timing for TIPS, refining patient selection criteria, and evaluating long-term outcomes.
