Table 5.
Management and outcomes in classical Budd-Chiari syndrome and hepatic vena cava-Budd Chiari syndrome
|
Classical BCS |
HVC-BCS |
||||||||||||||
| Perelló et al[40] | Rautou et al[37] | Raszeja-Wyszomirska et al[45] | Westbrook et al[32] | Harmanci et al[42] | Seijo et al[11] | Nozari et al[47] | Pavri et al[38] | De et al[23] | Xu et al[41] | Ebrahimi et al[46] | Park et al[51] | Cheng et al[13] | Gao et al[49] | ||
| Country | Spain | France | Poland | United Kingdom | Turkey | Europe | Iran | United States | India | China | Iran | South Korea | China | China | |
| n (%) | 21 | 94 | 20 | 66 | 62 | 157 | 55 | 47 | 40 | 1360 | 21 | 67 | 145 | 471 | |
| Medical management | 21 (100) | 94 (100) | 20 (100) | 61 (92) | 61 (98) | 139 (89) | 55 (100) | ≥ 40 (85) | 12 (57) | 32 (48) | |||||
| Medical management only (%)1 | 7 (33) | 69 (54) | 0 (0) | 4 (3) | 31 (7) | ||||||||||
| 49 (71) alive | |||||||||||||||
| Interventional therapy | 14 (67) | 34 (52)5 | 88 (56) | 10 (18) | 23 (58) | 1360 (100) | 9 (43) | 141 (97) | 440 (93) | ||||||
| 72 (82) alive | 1318 alive | ||||||||||||||
| PTA | 1 (5) | 17 (18) | 2 (3) in IVC | 22 (14) | 23 (58) | 9 (43) | 27 (40) | 134 (92) | |||||||
| Shunt operation | 2 (10) | 32 (48.5)6 | 2 (4) | 330 (24) | 3 (14) | 4 (5.9) | |||||||||
| TIPS2 | 13 (62) | 28 (30) | 2 (10) | 4 (6) | 62 (39)3 | 2 (4) | 21 (45) | 3 (4.5) | 12 | ||||||
| Liver transplantation | 15 (16) | 10 (50) | 36 (55) | 0 (0)8 | 204 (13) | 5 (9) | 8 (17) | 0 (0) | 0 (0)12 | ||||||
| 2 (1)12 | |||||||||||||||
| Median follow-up (in months) | 5813 | 3813 | 43 | 17 | 40-739 | 25.212 | 50 | 32 | 56 | 81.6 | 103 | 12 | 19 | ||
| Survival | 7 (100) | 12 (86) | 74 (79) | 15 (75) | 95 (73) | 65 mo10 | 37 (79) | ||||||||
| At 1 yr | 80% | 88% | 96% | 37 (79) | 67%7 | 99% | 401 (94) | ||||||||
| At 5 yr | 74 (79) | 56% | 95 (73) | 75% | 86% | ||||||||||
| Mortality | 1011 | 2 (1) | |||||||||||||
Medical management includes anticoagulation, diuretics, and medical treatment of any underlying causes;
After PTA failed;
Of the 22 patients who initially were treated with PTA/thrombolytics, 12 subsequently underwent TIPS and 2 underwent OLT;
Among the 62 patients who underwent TIPS, 4 subsequently underwent OLT;
Patients who failed medical management (namely anticoagulation with heparin and warfarin and diuretics) as defined by persistent transaminitis, resistant ascites or worsening hepatic function) moved on to receive stenting, shunting, or TIPS;
50% success rate (16/32);
Per study, 7 out of 21 patients died before hospital discharge;
No patients underwent liver transplantations due to a lack of donor availability;
Median follow-up post liver transplantation was 40 mo and median follow-up of patients with MPN was 73 mo;
Mean survival time was 65 mo;
10 patients died by the 5-yr follow-up period;
At the end of the follow-up period, 2 patients were waiting to receive OLT from another hospital;
Mean, not median values provided. Interventional therapy includes both endovascular and surgical procedures. OLT: Orthotopic liver transplant; TIPS: Transjugular intrahepatic portosystemic shunt; PTA: Percutaneous transluminal angioplasty; BCS: Budd-Chiari syndrome; HVC: Hepatic vena cava; IVC: Inferior vena cava.