Budd-Chiari syndrome: A single-center experience

Table 1 Clinical and laboratory characteristics n (%)

Clinical or laboratory feature	Entire cohort (n = 47)	TIPS with anticoagulation (n = 19)	Anticoagulation alone (n = 21)	P value
Age (yr) (Median, IQR)	42.4 (31.1, 52.1)	33.3 (29.7, 45.8)	46.9 (39.5, 57.3)	0.01
Gender
Male	16 (34.0)	6 (31.6)	7 (33.3)	0.91
Race/Ethnicity
African-American	11 (23.4)	5 (26.3)	3 (14.3)	0.69
White	32 (68.1)	12 (63.2)	16 (76.2)
Other	4 (8.5)	2 (10.5)	2 (9.5)
Hispanic/Latino	2 (4.3)	2 (10.5)	0 (0.0)	0.22
Comorbidities
Alcohol	2 (4.3)	0 (0.0)	1 (4.8)	1.00
Anemia	9 (19.1)	2 (10.5)	6 (28.6)	0.24
Cerebrovascular accident	1 (2.1)	1 (5.3)	0 (0.0)	0.47
Coronary artery disease	2 (4.3)	1 (5.3)	1 (4.8)	1.00
Chronic kidney disease	10 (21.3)	6 (31.6)	2 (9.5)	0.12
Chronic obstructive pulmonary disease	0 (0.0)	0 (0.0)	0 (0.0)
Congestive heart failure	1 (2.1)	0 (0.0)	1 (4.8)	1.00
Cirrhosis	26 (55.3)	13 (68.4)	8 (38.1)	0.05
Diabetes mellitus	8 (17.0)	1 (5.3)	4 (19.0)	0.34
Hypercholesterolemia	2 (4.3)	1 (5.3)	1 (4.8)	1.00
Peripheral vascular disease	0 (0.0)	0 (0.0)	0 (0.0)
Risk factors
Essential thrombocytosis	3 (6.4)	2 (10.5)	1 (4.8)	0.60
Factor V leiden mutation	4 (8.5)	1 (5.3)	2 (9.5)	1.00
Oral contraceptive use	2 (4.3)	2 (10.5)	0 (0.0)	0.23
Paroxysmal nocturnal hemoglobinuria	3 (6.5)	1 (5.3)	2 (9.5)	1.00
Protein C deficiency	2 (4.26)	1 (5.3)	1 (4.8)	1.00
Protein S deficiency	1 (2.1)	0 (0.0)	1 (4.8)	1.00
Polycythemia vera	14 (29.8)	9 (47.4)	4 (19.0)	0.09
Laboratory tests (Median, IQR) or (mean ± SD)
Albumin (g/dL)	2.9 ± 0.5	3.1 ± 0.6	2.7 ± 0.5	0.07
Alkaline phosphatase (U/L)	131 (98, 161)	131 (98, 177)	116 (96, 160)	0.69
Alanine aminotransferase (IU/L)	59 (36, 119)	76 (55, 190)	52 (39, 87)	0.11
Aspartate aminotransferase (IU/L)	67 (42, 113)	78 (54, 221)	66 (37, 96)	0.33
Bilirubin, total (mg/dL)	1.6 (1.2, 2.9)	1.7 (1.2, 4.0)	1.5 (0.9, 2.1)	0.42
Creatinine (mg/dL)	0.9 (0.6, 1.3)	0.8 (0.6, 1.2)	0.9 (0.6, 1.4)	0.80
Hemoglobin (mg/dL)	13.4 ± 2.7	14.5 ± 2.1	12.3 ± 2.9	0.02
International normalized ratio	1.7 (1.3, 2.2)	1.7 (1.4, 2.2)	1.5 (1.2, 2.6)	0.66
Platelet count (THO/μL)	217 (97, 329)	262 (181, 376)	173 (80, 238)	0.05
Disease severity (Diagnosis)
Child-Pugh (CP) score	9.2 ± 1.5	9.3 ± 1.3	8.9 ± 1.8	0.49
Model for end stage liver disease	17.1 ± 6.2	16.7 ± 4.9	18.9 ± 7.3	0.63
Rotterdam score
I	22	10	10	1.00
II	0	0	0
III	11	5	4
Follow-up time (d) (Median, IQR)	974 (393, 2697)	1101 (386, 2697)	834 (190, 1791)	0.36

TIPS: Transjugular intrahepatic portosystemic shunt; IQR: Interquartile range.

Table 2 Treatment and outcomes n (%)

Outcome	Entire cohort (n = 47)	TIPS with anticoagulation (n = 19)	Anticoagulation alone (n = 21)	P value
Anticoagulation	40 (85.1)	19 (100.0)	21 (100)
Time to anticoagulation (d) (Median, IQR)	23 (0, 249)	28 (1, 91)	7 (0, 906)	0.92
TIPS	21 (44.7)	19 (100.0)	0 (0)
Bleeding	5 (10.6)	2 (10.5)	3 (14.3)	1.00
Hepatic encephalopathy	18 (38.3)	7 (36.8)
TIPS stenosis1	12 (57.1)	10 (52.6)
Resolution of ascites1	13 (61.9)	12 (63.2)
Variceal hemorrhage12	1 (4.76)	1 (5.26)
Liver transplantation	8 (17.0)	5 (26.3)	3 (14.3)	0.44
Time to liver transplantation (d) (Median, IQR)	11101 (295, 2021)	947 (519, 1561)	1863 (295, 3628)	0.48
Death	10 (21.3)	2 (10.5)	5 (23.8)	0.41
Time to death (d) (Median, IQR)	811 (686, 1142)	1030 (919, 1142)	686 (107, 789)	0.05

¹These outcomes represent those observed in the post-TIPS setting (at date of data collection);

²For variceal hemorrhage, data were unknown for 6 patients in the entire cohort, and for 3 patients in the TIPS with AC cohort, in the post-TIPS setting. TIPS: Transjugular intrahepatic portosystemic shunt; IQR: Interquartile range; AC: Anticoagulation.

Table 3 Predictors of liver transplant and death

Predictor	Univariable odds ratio (95%CI)	Univariable P value	Multivariable odds ratio (95%CI)	Multivariable P value
Age	1.04 (1.00-1.09)	0.090	1.06 (1.00-1.12)	0.062
Ascites (Diagnosis)	1.96 (0.19-20.4)	0.570
Chronic kidney Disease	10.80 (1.95-59.77)	0.006	7.67 (1.20-48.96)	0.031
CP score (Diagnosis)	0.79 (0.49-1.28)	0.339
Cirrhosis	8.18 (1.92-34.84)	0.004	6.25 (1.19-32.72)	0.030
Creatinine (Diagnosis)	1.76 (0.60-5.14)	0.300
MELD score	0.96 (0.85-1.08)	0.489
TIPS Placement	0.95 (0.26-3.42)	0.935

MELD: Model For End-Stage Liver Disease; TIPS: Transjugular intrahepatic portosystemic shunt; CP: Child-Pugh.

Table 4 Characteristics of patients who died after diagnosis of Budd-Chiari syndrome

Study ID	Age at death	Gender	Race	Ethnicity	Evaluated for LT? (Y/N)	Listed for LT? (Y/N)	Contraindication for LT? (Y/N) If “Yes,” specify	Time from listing to death (mo)	LT?(Y/N)	Cause of death
1	49	F	Black or AA	Not hispanic or Latino	Y	Y	N	26	N	Unknown
2	61	M	White	Not hispanic or Latino	N	N	Y; HCC with extensive vascular invasion and lung metastasis	N/A	N	Unknown
3	49	M	White	Not hispanic or Latino	Y	Y	N	65	N	Metastatic colon cancer
4	59	M	White	Not hispanic or Latino	Y	N	Y; poor LT candidate due to ESRD, history of CAD, and kidney/pancreas transplant	N/A	N	Vertebral osteomyelitis of the lumbar spine, MRSA bacteremia
5	34	M	White	Not hispanic or Latino	Y	Y	Y; Testicular cancer	12	N	Metastatic testicular cancer
6	34	M	White	Not hispanic or Latino	Y	Y	N	72	N	Hypercapnic respiratory failure, pneumonia
7	67	F	Black or AA	Not hispanic or Latino	Y	N	N (in transplant evaluation stage at date of death)	N/A	N	Severe sepsis
8	52	F	Other	Not hispanic or Latino	Y	N	Y; HCC with secondary malignant neoplasm of the lung	N/A	N	Liver failure, metastatic HCC
9	42	F	White	Not hispanic or Latino	N	N	Y; Breast cancer	N/A	N	Metastatic breast cancer
10	69	M	White	Not hispanic or Latino	Y	Y	N	16	N	Renal failure, liver failure, possible sepsis

LT: Liver transplantation; AA: African American; HCC: Hepatocellular carcinoma; CAD: Coronary artery disease; ESRD: End-stage renal disease; MRSA: Methicillin-resistant Staphylococcus aureus; F: Female; M: Male; Y/N: Yes/No; N/A: Not available.

Table 5 Comparison of alive and deceased cohorts n (%)

	Patients who died	Patients who survived
	(n = 10)	(n = 37)
Age at date of death (yr) (Median, IQR)	48.5 (41.3, 59.7)	39.5 (32.1, 48.8)
Gender
Male	6 (60)	10 (27)
Female	4 (40)	27 (73)
Race/Ethnicity
African-American	2 (20)	9 (24)
White	7 (70)	25 (68)
Other	1 (10)	3 (8.1)
Hispanic/Latino	0 (0)	2 (5.4)
Treatment and management
Anticoagulation alone	5 (50)	16 (43)
TIPS alone	1 (10)	1 (2.7)
TIPS with AC	2 (20)	17 (46)
Evaluated for LT	8 (80)	19 (51)
Listed for LT	5 (50)	32 (86)
LT	0 (0)	8 (22)
No therapy	2 (20)	5 (14)

LT: Liver transplantation; AC: Anticoagulation; TIPS: Transjugular intrahepatic portosystemic shunt; IQR: Interquartile range.