Published online Jul 26, 2020. doi: 10.12998/wjcc.v8.i14.2930
Peer-review started: March 6, 2020
First decision: April 12, 2020
Revised: June 3, 2020
Accepted: July 4, 2020
Article in press: July 4, 2020
Published online: July 26, 2020
Budd-Chiari syndrome (BCS) is a rare disease, which is defined as hepatic venous outflow tract obstruction. For Chinese patients, the predominant obstructive lesions are membranous and segmental obstructions of the supra-hepatic or retro-hepatic portion of the inferior vena cava, and the most common treatment is percutaneous recanalization (percutaneous transluminal angioplasty (PTA) with or without stent implantation). Restenosis is the most common complication after recanalization. However, the management strategy and the long-term survival of BCS patients with restenosis are seldom reported.
For the treatment of restenosis after recanalization, there are different opinions; some researchers suggest stent implantation and others advocate repeated balloon dilatation (including the use of large-diameter balloons). Different treatments have their own advantages and disadvantages, and the reported results vary considerably. In brief, there is currently no consensus on the best treatment strategy. We are very interested in this issue and hope that we can present a stepwise treatment strategy adopted in our center.
The objectives were to report the long-term follow-up outcomes for the patients with restenosis treated by our stepwise invasiveness increasing strategy and to discuss the prognosis of different treatment options (active treatment or non-treatment).
We retrospectively analyzed the 30-year follow-up outcome of BCS patients at our center, and totally 60 patients with restenosis after recanalization were included in the analysis by case screening. According to their primary treatment methods, the patients were divided into two groups (PTA group and PTA + stent group) and were followed until the end of this study (December 31, 2014). Restenosis was defined as the recurrence of symptoms after recanalization due to the re-obstruction of primary recanalized vessels or the newly formed obstruction of the hepatic venous outflow tract, which is confirmed by imaging examinations (color Doppler ultrasonography, computed tomography, and/or magnetic resonance imaging). Cumulative survival rates were analyzed using Kaplan-Meier curves. Two-tailed P values less than 0.05 were considered statistically significant.
Among the 60 patients, 40 were primarily treated by PTA alone (PTA group) the rest were primarily treated by PTA plus stenting (PTA + stent group). In the PTA group, 19 restenosis patients were re-treated by PTA alone, 8 were re-treated by PTA plus stenting, and 13 refused further treatment; and among the 13 patients who refused active treatment, 7 died. In the PTA + stent group, 10 restenosis patients were treated by PTA alone, 1 underwent implantation of another stent after PTA, and 9 refused further treatment; the same point is that those who refused further treatment after restenosis died. There is a statistically significant difference between the two groups — the group of patients who received further treatment after restenosis and the other group of patients who did not (P < 0.001).
To the best of our knowledge, for Chinese BCS patients with restenosis, our follow-up period of more than 25 years is by far the longest. In this study, we focused on the treatment strategy for BCS restenosis. For these patients, we advocate regular follow-up and active treatment by gradually increasing the invasiveness, starting from PTA (including the use of large-diameter balloons). If PTA does not work, then stent implantation will be performed. It is vital to comprehensively weigh the pros and cons before performing stent implantation, also we should pay more attention to possible complications during the follow-up. Regular follow-up and active treatment can result in satisfactory prognosis in BCS patients with restenosis.
For patients with restenosis who refuse further treatment but have a relatively good prognosis, it is worth further investigating potential protective factors to provide us with new ideas for treatment. The shortcomings of our study are expected to be overcome by multicenter large sample randomized controlled trials in the future.