Outcomes of pregnancy in patients with known Budd-Chiari syndrome

Table 1 Baseline characteristics of the patients at presentation

Patient ID	1	2	3	4	5	6	7
Age at diagnosis (yr)	34	21	30	21	31	24	25
Symptoms at presentation	Ascites	Oesophageal variceal haemorrhage, abdominal pain	Abdominal pain; ascites	Abdominal pain, ascites	Abdominal pain, fever, mouth ulcers	Ascites, renal failure and sepsis (ITU admission)	Abdominal pain
Risk factors for BCS	JAK 2 positive MPD; OCP	JAK 2 positive mutation	None identified	Factor V Leiden; OCP	JAK2 positive MPD (Essential Thrombocythaemia); Factor V Leiden	JAK 2 positive mutation	Factor V Leiden
Encephalopathy	None	None	None	None	None	None	None
Ascites	Moderate	Mild	Mild	Mild	None initially	Severe	Moderate
INR	1.7	1.4	1.2	1.3	1.7	1.4	1.5
Albumin (g/L)	28	37	49	49	49	25	26
Bilirubin (umol/L)	19	18	20	18	11	51	32
ALT (U/L)	-	31	-	57	-	-	-
AST (U/L)	134	49	20	34	27	277	43
Urea (mmol/L)	2.7	2.3	2.9	4.7	2.9	4.4	2
Creatinine (mmol/L)	72	43	70	68	51	92	70
Sodium (mmol/L)	143	137	143	142	140	130	133
MELD	19	14	6	10	12.37	14	17
UKELD	53	53	48	49	49	49	55
Hb (g/L)	137	121	155	128	150	147	88
WCC (109/L)	7.9	9.6	10.9	5.7	5.7	28.8	6.8
Platelets (109/L)	345	183	307	247	411	400	226
Rotterdam PI	1.116	0.072	1.12	0.07	1.08	1.244	1.168
Clichy PI	4.39	1.99	3.13	4.04	3.44	7.54	7.55
Liver biopsy	Not done	Not done	Not done	Suggestive of hepatic vein obstruction	Consistent with Hepatic venous outflow obstruction	Not done	Not done
Level of obstruction	Left hepatic vein	Hepatic vein	Hepatic vein	Hepatic Vein	Right Hepatic Vein	Left Hepatic vein	Hepatic vein
Radiological intervention	TIPSS	TIPSS	None	Angioplasty and Stenting to Hepatic vein	Right Hepatic Vein dilatation	TIPSS	TIPSS
Type of TIPSS	Viatorr (covered)	Viatorr (covered)	-	-	-	Memotherm, then Viatorr	Memotherm (Uncovered)
Medications post intervention	Warfarin	Warfarin	N/A	Warfarin	Warfarin	Warfarin, Interferon	Warfarin
Duration of follow up (yr)	4	5	7	3	13	14	14
Comments/ complications following intervention	TIPSS Stent redilatation after a week of insertion	TIPSS stent stenosis - needed to be re-dilated in 2 yr	Maintained on oral anticoagulation (warfarin) and did not require any intervention	Vascular Wallstent was re-canalized after 2 yr	Inferior RHV dilated 5 yr after the diagnosis (developed ascites and had compliance issues).	Bleeding from hepatic nodule (with INR > 9). Managed conservatively. Later stent was changed to a covered one for TIPSS stenosis	-

MPD: Myeloproliferative disorder; TIPSS: Trans-jugular intrahepatic posto-systemic shunt; OCP: Oral contraceptive pills; INR: International normalised ratio; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; MELD: Model for end-stage liver disease; UKELD: United Kingdom model for end-stage liver disease; Hb: Haemoglobin; WCC: White cell count.

Table 2 Gestational course and perinatal complications in 16 pregnancies

Patient No.	Pregnancy No.	Age at gestation (yr)	Anticoagulation during pregnancy	Mode of delivery	Weeks gestation	Birth weight	Foetal/infant condition	Maternal condition
1	1	37	LMWH	Vaginal	36	2645 g	Neonatal jaundice, treatment with antibiotics for suspected infection
2	2	24	LMWH	Emergency caesarean section	35	2140 g	Fetal distress (reduced foetal movements)- Healthy baby	ICP OGDs during pregnancy, no varices seen
3	3	35	LMWH	Vaginal delivery	35	2600 g	Mild Jaundice	In-vitro fertilization treatment
3	4	37	LMWH	Vaginal delivery	37	2450 g	Healthy	In-vitro fertilization treatment Primary post-partum haemorrhage secondary to retained placenta that was surgically evacuated
4	5	23	LMWH	Caesarean section	37	2645 g	Fetal distress - Healthy baby post delivery	…
5	6	36	LMWH and Aspirin (switched from warfarin and Hydroxyurea at 22 wk when pregnancy was diagnosed)	Emergency caesarean section	37	3115 g	Breech presentation	Had several gastroscopies (OGD) and banding to Oesophageal Varices during pregnancy
5	7	39	Warfarin	Miscarriage	5	-	-	PV bleeding; was not aware of conception
6	8	31	LMWH	Emergency Caesarean Section	27	Not available	Healthy boy	Bleeding secondary to placental abruption ICP from 25 wk
6	9	37	LMWH, Aspirin, interferon for MPD (Myeloproliferative disorder)	Emergency Caesarean section	35	Not available	Fetal distress. Healthy baby	ICP Minor subchorionic bleeding at 12 and 23 wk. LMWH reduced, aspirin stopped temporarily. Changes resolved on subsequent scans. Presentation with PH and suspected placental abruption at 35 wk Secondary post-partum haemorrhage treated with surgical of uterine clot evacuation and Rusch Balloon
7	10	25	LMWH	Miscarriage	9	-	-	-
7	11	27	LMWH	Miscarriage	20	-	Congenital pneumonia and mild amnionitis	Weakness of cervix;
7	12	28	LMWH	Miscarriage	19	-	-	Placental abruption Weakness of cervix; Placental abruption
7	13	29	LMWH	Emergency Caesarean Section	35	2974 g	Healthy boy	Dyspnoeic during 3rd Trimester; ICP in 20 wk onwards; C-Section for difficult labour (cervical suture could not be removed)
7	14	31	LMWH	Failed Pregnancy	10	-	-	Surgical removal of retained products of Contraception
7	15	33	LMWH	Miscarriage	7	-	-	-
7	16	34	LMWH	Emergency Caesarean Section	35	2440 g	Healthy boy	Pre-eclampsia; Breathlessness during 3rd trimester, PH diagnosed after pregnancy

LMWH: Low molecular weight heparin; ICP: Intrahepatic cholestasis of pregnancy; PH: Pulmonary hypertension.