Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center

Table 1 Baseline characteristics of the 256 patients

	Medicine	Surgery	Intervention	Untreated
Total number	30	14	178	34
Demographic data
Sex
Male	16	4	109	24
Female	14	10	69	10
Age (yr)1	36 (7-74)	35 (24-47)	41 (14-80)	44.5 (14-66)
Duration of symptoms
≤ 1 mo	5	0	28	7
1-6 mo	7	2	35	12
≥ 6 mo	18	12	115	15
Clinical manifestations
Abdominal distention	24	12	82	15
Abdominal wall varicosis	12	13	105	16
Lower-extremity edema	17	13	104	17
Gastroesophageal variceal bleeding	8	3	26	4
Hepatic encephalopathy	0	0	0	2
Laboratory tests12
Hemoglobin level (g/L)	133 (64-172)	149 (101-176)	130.5 (30-180)	134 (80-180)
Platelet count (× 109/L)	130 (49-479)	92 (47-160)	108.5 (33-603)	139.5 (50-341)
Alanine transaminase level (× ULN)	0.6 (0.2-7.6)	0.6 (0.3-1.6)	0.6 (0.2-28)	0.7 (0.3-3.6)
Albumin level (g/L)	34.5 (13.1-54)	36 (22-41)	37.4 (16.7-57.7)	35 (16-58)
Total bilirubin level (μmol/L)	26.2 (6-146.2)	28.6 (17.1-68.4)	26 (7-292)	24.9 (6.1-168)
International normalized ratio	1.4 (0.9-1.9)	1.3 (1.1-1.7)	1.3 (0.9-2.9)	1.3 (0.9-1.8)
Creatinine level (μmol/L)	106.3 (85-341)	74 (71-77)	74.1 (30-254)	75 (29.6-146)
Blood urea nitrogen level (mmol/L)	5.3 (1.8-26.6)	5.1 (2.5-18.6)	5.3 (2.5-39.1)	5.6 (3.6-11.8)
Imaging features
Type of obstruction
HV	9	0	25	10
IVC	3	2	41	9
Com	18	12	112	15
Pattern of IVC obstruction
No obstruction	7	0	25	10
Membranous	14	8	108	15
Segmental	8	4	36	4
Long segmental	1	2	9	5
Ascites	17	9	85	14
AHV compensation	1	3	34	4
IVC thrombosis	11	5	57	14
Portal vein thrombosis	1	0	3	3
Prognostic index
Child-Pugh score12	7 (5-9)	6 (5-7)	7 (5-12)	6 (5-11)
Child-Pugh class2
A	3	1	59	6
B	6	1	61	4
C	0	0	6	1

¹Data are shown as median with range in parentheses;

²Data are incomplete because some laboratory tests were not performed prior to the year 2000. Except where indicated, data are shown as number of patients. ULN: Upper limit of normal; HV: Hepatic vein; IVC: Inferior vena cava; Com: Combination; AHV: Accessory hepatic vein.

Table 2 Detailed information on surgical operations and interventional procedures

Department	Operations/procedures	No.	Complications
Surgery	Cavoatrial shunt	10	Hemorrhagic shock (n = 1)
	Radical resection	1
	Meso-cavo-atrial shunt	1	Acute hepatic failure (n = 1)
	Splenopneumopexy	1
	Splenocaval shunt	1
Interventional radiology	Technic failure	6
	PTA	96	Abdominal pain (n = 4), DIC (n = 1)
	PTA combined with stent	69	Abdominal pain (n = 2), Stent fracture (n = 1), Supraventricular tachycardia (n = 2)
	TIPS	7
	Catheter directed thrombolysis	191	Hematuria (n = 2), Hemoptysis (n = 1)

¹The total number of patients treated by interventional procedures was 178, and the 19 patients treated by catheter directed thrombolysis were repeatedly counted among the patients treated by PTA (n = 5) and PTA combined with stent implantation (n = 14). PTA: Percutaneous transluminal angioplasty; TIPS: Transjugular intrahepatic portosystemic shunt; DIC: Disseminated intravascular coagulation.

Table 3 Follow-up results of 256 Budd-Chiari syndrome patients

Treatment	Total	Lost	Death	Remission	Non-remission/progression
Medicine	30	16	Variceal bleeding (n = 4), liver or multiple organ failure (n = 3), and hepatocellular carcinoma (n = 1)	3	Abdominal distention (n = 3)
Surgery	14	5	Liver or multiple organ failure (n = 3), hepatocellular carcinoma (n = 1), variceal bleeding (n = 1), anastomotic infection (n = 1), and hepatic encephalopathy (n = 1)	1	Abdominal distention (n = 1)
Interventional radiology
Technic failure	6	0	Liver or multiple organ failure (n = 3)	1	Abdominal distention (n = 1) and lower-extremity edema (n = 1)
PTA	96	6	Liver or multiple organ failure (n = 8), hepatocellular carcinoma (n = 5), variceal bleeding (n = 3), cholangiocarcinoma (n = 1), intracranial hemorrhage induced by hypertension (n = 1), DIC (n = 1), and accidental death (n = 1)	57	Abdominal distention (n = 7), lower-extremity edema (n = 4), and lower-extremity varix (n = 2)
PTA combined with stent placement	69	5	Liver or multiple organ failure (n = 7), hepatocellular carcinoma (n = 3), variceal bleeding (n = 2), hepatic encephalopathy (n = 2), and hemoptysis (n = 1)	44	Abdominal distention (n = 3), lower-extremity edema (n = 1), and muscle wasting (n = 1)
TIPS	7	2	Liver or multiple organ failure (n = 2), and variceal bleeding (n = 1)	1	Jaundice (n = 1)
Untreated	34	10	Liver or multiple organ failure (n = 7), variceal bleeding (n = 1), hepatocellular carcinoma (n = 1), hepatic encephalopathy (n = 1), and chronic leukemia (n = 1)	6	Abdominal distention (n = 4), muscle wasting (n = 2), and lower-extremity edema (n = 1)

PTA: Percutaneous transluminal angioplasty; TIPS: Transjugular intrahepatic portosystemic shunt; DIC: Disseminated intravascular coagulation.

Table 4 Univariate and multivariate analyses of the predictors of survival for treated patients

Variable	Univariate analysis			Multivariate analysis
	HR	95%CI	P value	HR	95%CI	P value
Sex (Male/Female)	0.558	0.297-1.050	0.071
Age	0.992	0.968-1. 017	0.550
History of BCS since first presentation	0.994	0.987-1.000	0.055
Abdominal distention (Yes/No)	0.943	0.531-1.643	0.813
Abdominal wall varicosis (Yes/No)	0.819	0.461-1.457	0.497
Gastroesophageal variceal bleeding (Yes/No)	2.928	1.647-5.270	< 0.001	3.043	1.363-6.791	0.007
Lower-extremity edema (Yes/No)	1.318	0.757-2.294	0.330
Hemoglobin level	0.994	0.983-1.005	0.311
Platelet count	1.002	0.998-1.006	0.308
Alanine transaminase level	1.003	1.001-1.005	0.005	1.002	0.999-1.005	0.274
Albumin level	0.975	0.928-1.024	0.307
Total bilirubin level	1.007	0.997-1.017	0.183
INR	1.280	0.367-4.468	0.699
Creatinine level	1.005	0.991-1.019	0.489
Blood urea nitrogen level	1.049	0.971-1.133	0.225
Ascites (Yes/No)	2.108	1.205-3.686	0.009	1.849	0.812-4.213	0.143
Accessory hepatic vein (Yes/No)	2.126	0.842-5.366	0.110
Associated IVC thrombosis (Yes/No)	1.000	0.553-1.809	0.999
Restenosis (Yes/No)	5.309	2.378-11.852	< 0.001	4.610	1.916-11.091	0.001
Child-Pugh score	1.108	0.852-1.440	0.444

HR: Hazard ratio; BCS: Budd-Chiari syndrome; INR: International normalized ratio; IVC: Inferior vena cava.