Table 7

Case series of treatment for liver vascular malformations (VMs) in hereditary haemorrhagic telangiectasia (HHT)

StudyNoClinical typesTreatmentMedian follow-up (months)Outcomes of treatmentFrequency of outcomesComplicationsFrequency of complications
Lerut et al 200612740
  • 14 HF

  • 12 BIL

  • 5 PHT

  • 6 HF+BIL

  • 2 HF+PHT

  • 1 HF+PHT+BIL

Trans58
  • 5-year survival

  • HF improved

  • HF Stable

  • HF alone Death

  • BIL+/− HF Death

  • PHT +/− HF Death

  • 83%

  • 18/24 (75%)

  • 5/24 (21%)

  • 1/24 (4%)

  • 4/18 (22%)

  • 3/8 (38%)

  • Intraoperative bleed*

  • GI bleed*

  • CHF*

  • Acute rejection*

  • Chronic rejection*

  • Graft failure*

  • Cerebral bleed*

  • PAVM bleed*

  • Non-fatal complications

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 1/40 (3%)

  • 24/40 (60%)

Chavan et al 200412515
  • 11 HF

  • 5 Steal

  • 4 PHT

Staged HA embolisation28
  • Alive

  • HF alive

  • HF improved

  • Steal alive

  • Steal improved

  • PHT alive

  • PHT improved

  • 11/15 (73%)

  • 10/11 (91%)

  • 10/11 (91%)

  • 5/5 (100%)

  • 5/5 (100%)

  • 2/4 (50%)

  • 2/4 (50%)

  • Hepatic necrosis*

  • Cholangitis/cholecystitis*

  • 1/15 (7%)

  • 3/15 (20%)

Azoulay et al 20021266
  • 3 BIL

  • 2 PHT

  • 1 HF+BIL

Transplant57
  • Alive

  • BIL alive

  • PHT alive

  • HF+BIL alive

  • 4/6 (67%)

  • 3/3 (100%)

  • 1/2 (50%)

  • 0/1 (0%)

  • GI bleeding*

  • Peritonitis*

  • 1/6 (17%)

  • 1/6 (17%)

  • * Causing death.

  • CHF, congestive heart failure; GI, gastrointestinal; HA, hepatic artery; HF, high-output heart failure; PAVM, pulmonary arteriovenous malformation; PHT, portal hypertension; BIL=biliary.