Hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber disease is a rare disease characterized by the presence of arteriovenous malformations.
Hepatic involvement can lead to life-threatening conditions.
Dr Lerut Orlando and colleagues from France analyzed 40 patients reported to the European Liver Transplant Registry.
The researchers defined the role of liver transplantation in the treatment of the hepatic disease form.
The research team classified indications for transplantation according to Garcia-Tsao.
|10-year actuarial patient and graft survival rates were 83%|
|Annals of Surgery|
There were 14 patients classified as having cardiac failure, 12 with biliary necrosis causing hepatic failure, and 5 with severe portal hypertension.
The team noted that 6 had cardiac failure with biliary necrosis, and 2 patients had cardiac failure with portal hypertension.
Only 1 patient presented with cardiac failure associated with biliary necrosis and portal hypertension.
The researchers found that 18 of 22 patients had pulmonary artery hypertension.
The team noted that 30% patients had pretransplant hepatic interventions.
Follow-up was complete for all patients with a mean of 69 months.
The researchers observed that 1-, 5- and 10-year actuarial patient and graft survival rates were 83%.
The team found that 6 of the 7 pretransplant procedures performed on the hepatic artery were severely complicated.
Cardiovascular function documented in 24 patients improved in 18 patients and remained stable in 5 patients.
The researchers noted that 1 patient died perioperatively of acute heart failure.
About 60% of patients had post-transplant complications.
All but 1 complication occurred within the first 4 posttransplant months.
The team reported that 7 patients died perioperatively.
Of these, 6 died of bleeding, 1 due to cardiac failure, and 1 died late due to chronic rejection.
The researchers identified 2 possible recurrences.
Quality of life markedly improved in all 32 surviving patients.
Dr Orlando's team concludes, “The results of the largest reported transplant series in the treatment of hepatic-based hereditary hemorrhagic telangiectasia are excellent.”
“Elimination of hepatobiliary sepsis and reversal of cardiopulmonary changes dramatically improve quality of life of the recipients.”
“Liver transplantation should be proposed earlier in the course of symptomatic hepatic hereditary hemorrhagic telangiectasia presenting with life-threatening conditions.”
“Palliative interventions, especially on the hepatic artery, should be avoided in view of their high infectious complication rate.”