Liver transplant in Budd-Chiari syndrome: a single-center experience in Saudi Arabia.
OBJECTIVE
If they do not respond to other treatments, patients with Budd-Chiari syndrome are potential candidates for a liver transplant. Timing for transplant is controversial; however, before other systems deteriorate, early intervention in relatively stable patient may improve the outcome and survival of these patients.


METHODS
Six patients (2 women and 4 men) had Budd-Chiari syndrome (1.2%) among 475 patients who had undergone a liver transplant at our center between 2001 and 2012. Imaging modalities including duplex ultrasound, abdominal computed tomography angiography, and hematologic evaluation were part of our routine diagnostic work-up. Although we perform mostly living-donor liver transplants, these patients received a liver transplant from a deceased donor, because there was not enough evidence to justify a living-donor liver transplant. We thought that not replacing the caval vein might negatively influence the outcome. Postoperatively, these recipients were started on a heparin infusion and triple therapy immunosuppression; only then was warfarin introduced as long-term anticoagulant.


RESULTS
Two patients died, 1 from uncontrollable bleeding and disseminated intravascular coagulopathy, and the other died in the intensive care unit after 5 months because of multiorgan failure and sepsis. One patient had portal vein thrombosis 9 months after the liver transplant; the other patient needed a liver retransplant after 5 years owing to liver failure, secondary to chronic rejection. Graft survival rate was 75%, and patient survival rate was 66.6%.


CONCLUSIONS
This is the first article from Saudi Arabia to describe the outcome of a liver transplant in this subgroup of patients with Budd-Chiari syndrome. Treatment of Budd-Chiari syndrome follows a therapeutic algorithm that should start with anticoagulation and may end up with liver transplant; however, it should be considered early if other treatments fail.
King Faisal Specialist Hospital and Research Hospital, Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, Riyadh, Saudi Arabia.
Yahia
Y
Saleh
Firas Zahr
FZ
Eldeen
Yasser
Y
Kamel
Monther
M
Kabbani
Mohammed
M
Al Sebayel
Dieter
D
Broering
D000328:Adult; D000465:Algorithms; D000925:Anticoagulants / Q000627:therapeutic use; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D019091:Critical Pathways; D003952:Diagnostic Imaging / Q000379:methods; D005260:Female; D006085:Graft Survival; D006403:Hematologic Tests; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012086:Reoperation; D012529:Saudi Arabia; D013997:Time Factors; D016896:Treatment Outcome; D014859:Warfarin / Q000627:therapeutic use
D000925:Anticoagulants; D007166:Immunosuppressive Agents; D014859:Warfarin; D006493:Heparin