Observational Study
Copyright ©The Author(s) 2017.
World J Hepatol. Jul 28, 2017; 9(21): 945-952
Published online Jul 28, 2017. doi: 10.4254/wjh.v9.i21.945
Table 1 Baseline characteristics of the patients at presentation
Patient ID1234567
Age at diagnosis (yr)34213021312425
Symptoms at presentationAscitesOesophageal variceal haemorrhage, abdominal painAbdominal pain; ascitesAbdominal pain, ascitesAbdominal pain, fever, mouth ulcersAscites, renal failure and sepsis (ITU admission)Abdominal pain
Risk factors for BCSJAK 2 positive MPD; OCPJAK 2 positive mutationNone identifiedFactor V Leiden; OCPJAK2 positive MPD (Essential Thrombocythaemia); Factor V LeidenJAK 2 positive mutationFactor V Leiden
AscitesModerateMildMildMildNone initiallySevereModerate
Albumin (g/L)28374949492526
Bilirubin (umol/L)19182018115132
ALT (U/L)-31-57---
AST (U/L)1344920342727743
Urea (mmol/L)
Creatinine (mmol/L)72437068519270
Sodium (mmol/L)143137143142140130133
Hb (g/L)13712115512815014788
WCC (109/L)7.99.610.95.75.728.86.8
Platelets (109/L)345183307247411400226
Rotterdam PI1.1160.0721.
Clichy PI4.391.993.134.043.447.547.55
Liver biopsyNot doneNot doneNot doneSuggestive of hepatic vein obstructionConsistent with Hepatic venous outflow obstructionNot doneNot done
Level of obstructionLeft hepatic veinHepatic veinHepatic veinHepatic VeinRight Hepatic VeinLeft Hepatic veinHepatic vein
Radiological interventionTIPSSTIPSSNoneAngioplasty and Stenting to Hepatic veinRight Hepatic Vein dilatationTIPSSTIPSS
Type of TIPSSViatorr (covered)Viatorr (covered)---Memotherm, then ViatorrMemotherm (Uncovered)
Medications post interventionWarfarinWarfarinN/AWarfarinWarfarinWarfarin, InterferonWarfarin
Duration of follow up (yr)4573131414
Comments/ complications following interventionTIPSS Stent redilatation after a week of insertionTIPSS stent stenosis - needed to be re-dilated in 2 yrMaintained on oral anticoagulation (warfarin) and did not require any interventionVascular Wallstent was re-canalized after 2 yrInferior 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-
Table 2 Gestational course and perinatal complications in 16 pregnancies
Patient No.Pregnancy No.Age at gestation (yr)Anticoagulation during pregnancyMode of deliveryWeeks gestationBirth weightFoetal/infant conditionMaternal condition
1137LMWHVaginal362645 gNeonatal jaundice, treatment with antibiotics for suspected infection
2224LMWHEmergency caesarean section352140 gFetal distress (reduced foetal movements)- Healthy babyICP OGDs during pregnancy, no varices seen
3335LMWHVaginal delivery352600 gMild JaundiceIn-vitro fertilization treatment
3437LMWHVaginal delivery372450 gHealthyIn-vitro fertilization treatment Primary post-partum haemorrhage secondary to retained placenta that was surgically evacuated
4523LMWHCaesarean section372645 gFetal distress - Healthy baby post delivery
5636LMWH and Aspirin (switched from warfarin and Hydroxyurea at 22 wk when pregnancy was diagnosed)Emergency caesarean section373115 gBreech presentationHad several gastroscopies (OGD) and banding to Oesophageal Varices during pregnancy
5739WarfarinMiscarriage5--PV bleeding; was not aware of conception
6831LMWHEmergency Caesarean Section27Not availableHealthy boyBleeding secondary to placental abruption ICP from 25 wk
6937LMWH, Aspirin, interferon for MPD (Myeloproliferative disorder)Emergency Caesarean section35Not availableFetal distress. Healthy babyICP 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
71127LMWHMiscarriage20-Congenital pneumonia and mild amnionitisWeakness of cervix;
71228LMWHMiscarriage19--Placental abruption Weakness of cervix; Placental abruption
71329LMWHEmergency Caesarean Section352974 gHealthy boyDyspnoeic during 3rd Trimester; ICP in 20 wk onwards; C-Section for difficult labour (cervical suture could not be removed)
71431LMWHFailed Pregnancy10--Surgical removal of retained products of Contraception
71634LMWHEmergency Caesarean Section352440 gHealthy boyPre-eclampsia; Breathlessness during 3rd trimester, PH diagnosed after pregnancy