Redefining Budd-Chiari syndrome: A systematic review

Table 5 Management and outcomes in classical Budd-Chiari syndrome and hepatic vena cava-Budd Chiari syndrome

	Classical BCS									HVC-BCS
	Perellóet 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)

¹Medical management includes anticoagulation, diuretics, and medical treatment of any underlying causes;

²After PTA failed;

³Of the 22 patients who initially were treated with PTA/thrombolytics, 12 subsequently underwent TIPS and 2 underwent OLT;

⁴Among the 62 patients who underwent TIPS, 4 subsequently underwent OLT;

⁵Patients 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;

⁶50% success rate (16/32);

⁷Per study, 7 out of 21 patients died before hospital discharge;

⁸No patients underwent liver transplantations due to a lack of donor availability;

⁹Median follow-up post liver transplantation was 40 mo and median follow-up of patients with MPN was 73 mo;

¹⁰Mean survival time was 65 mo;

¹¹10 patients died by the 5-yr follow-up period;

¹²At the end of the follow-up period, 2 patients were waiting to receive OLT from another hospital;

¹³Mean, 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.