Budd Chiari Syndrome

Good retrospective review of 510 consecutive patients and 618 procedures.
Budd Chiari Syndrome is rare (~11 per million). Subacute/chronic presentation is most common vs acute or hyperacute fulminant forms.
Outcomes of endovascular management
- Technical success 96%, angioplasty and stent in 71%, TIPS in 29%
- Vascular/stent restenosis in 19% with successful revascularization in 86%
- Patency rates at 1 and 5 yrs: (recan) 87 and 74%, (TIPS) 95 and 68%
- Survival at 1 and 5 yrs: (recan) 96 and 89%, (TIPS) 90 and 76%
Suggested protocol:

IVC blockage -> IVC plasty +/- stenting
HV blockage -> no intervention if at least 1 HV patent, HV plasty +/- stent for ostial or short obstruction, TIPS if long/diffuse obstruction
IVC + HV

HV reconstructable -> IVC and HV plasty +/- stent if HV is reconstructable
HV not reconstructable -> IVC plasty with TIPS if partial or no response after plasty

Recanalization technique: 10Fr sheath -> cross with hydrophilic glidewire -> exchange for stiff wire -> angioplasty +/- stenting. If refractory, transhepatic access +/- sharp recanalization with Rosch-Uchida catheter stylet set.
Major complications (2.8%): death from hemoperitoneum and shock or liver failure; hepatic necrosis, right atrial perforation, non-fatal hemoperitoneum, hemopericardium, diffuse alveolar hemorrhage, SVT