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Few cases of combined heart and liver transplantation (CHLT) have been reported for familial amyloidosis. Our first CHLT was performed on a female patient with familial amyloidosis due to a genetic defect in transthyretin, characterized by deposition of amyloid in various organs and tissues. This disease produced autonomic heart dysfunction that preceded the development of clinical manifestations and may be an important factor in determining the optimal timing for liver transplantation. CHLT can be performed successfully, even in patients with advanced disease. However, the most compromised patients are more exposed to intraoperative risks, postoperative complications, and worsening of extracardiac and extrahepatic symptoms. Our patient presented severe cardiac dysfunction requiring CHLT. The operative technique is far from being consolidated, despite this, both organs were transplanted in the same day with 2 hours in the intensive care unit (ICU) between surgeries. The outcome of both organs has been favorable. The amyloidotic liver was transplanted to another patient, a sequential (domino) transplantation.

Objective: To evaluate our application of indications, use and benefits of conventional hemodialysis during surgery in patients with advanced liver disease and acute or chronic renal failure undergoing liver transplantation (LP), liver retransplantation (LRT) or combined hepatorenal transplantation (CHRT)., Patients and Methods: We retrospectively reviewed the cases of 22 patients with advanced liver disease, 11 with acute renal failure and 11 with chronic renal failure. We performed 6 LT, 5 LRT and 11 CHRT. The following data were recorded in the periods before, during and immediately after surgery: metabolic, hemodynamic and coagulation parameters; bicarbonate, calcium and inotropic drug requirements; incidences during reperfusion of the graft; surgical technique used; and survival., Results: Seven patients (32%) needed hemodialysis, 4 (18%) needed ultrafiltration, 7 (32%) needed both and 4 (18%) required neither. For 6 patients total clamping of the inferior vena cava (ICV) was required with external venovenous bypass. For 8 patients total clamping of the IVC was performed without venovenous bypass. For 8 others IVC clamping was partial with retrohepatic preservation (piggy-back). There were 2 deaths during surgery, 4 more within the first month after surgery and 4 more in the second month. Overall survival was 36.4% among acute patients and 72.7% among CHRT patients., Conclusions: 1) Conventional hemodialysis during surgery is feasible and gives good results; 2) conventional "high efficiency" hemodialysis is more effective and useful in these patients than is either slow, continuous hemodialysis or filtration; 3) the survival rate of CHRT patients is similar to that of patients undergoing LT with normal kidney function, and 4) partial IVC clamping in the anhepatic phase may decrease the need for ultrafiltration.