Combined heart and liver transplantation for familial amyloidotic neuropathy: Considerations from the hepatic point of view

Abstract

Few cases of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy have been reported, and the technique for the operation is far from being consolidated. Three patients with amyloidogenic transthyretin (ATTR)-related (variant Glu89Gln to ATTR Glu89Gln) cardiomyopathy underwent CHLT at our institution. Patient 1 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All three patients underwent cardiac and sequential hepatic transplantation using the piggyback technique with organs procured from the same donor. Venovenous bypass (VVB) was used only in patient 1, with an uncomplicated procedure. After CHLT, his cardiac performance remained normal, and no progression of amyloidosis was observed. Patient 2 had no intraoperative complications, but experienced postoperative bleeding, renal failure, sepsis, and heart failure and eventually died of multiorgan failure 2 months after transplantation. In patient 3, right hemicolectomy was required intraoperatively because of intestinal ischemia without significant hemodynamic perturbations, whereas extracardiac symptoms of amyloidosis gradually worsened postoperatively. Two patients (no. 1 and 3) currently are alive after 38 and 18 months, respectively. CHLT for ATTR Glu89Gln 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. The need for VVB remains to be evaluated. (Liver Transpl 2003;9:986-992.)

Operative Risks of Domino Liver Transplantation for the Familial Amyloid Polyneuropathy Liver Donor and Recipient: A Double Analysis

“…To deal with these specific problems, two strategies can be used: first, the application of percutaneous veno-venous bypass (36)(37)(38), and second, the preservation of the retrohepatic vena cava. In this series (consisting of whole domino liver grafts), we used veno-venous bypass in two specific circumstances:…”

Treatment of End Stage Heart Failure Related to Cardiac Amyloidosis

“…Paradoxical acceleration of cardiac involvement after liver transplantation may occur in patients with mutation variants other than Val30Met [133]- [135], due to wild-type transthyretin deposition in addition to the background amyloid fibrils [136]- [138]. Therefore combined heart and liver transplantation rather heart transplant alone is considered in patients with significant cardiac involvement [139], [140]. Combined heart and liver transplantation can be performed in selected patients with results similar to heart transplant for other indications [141].…”

“…Initially transplantation of the heart and maintaining the patient on cardio-pulmonary bypass during the liver transplant was used. Subsequent concerns about substantial coagulopathy and increased bleeding changed the strategy to performing liver implantation after separation from cardiopulmonary bypass [139], [146], [147]. Later, improved surgical and anesthetic techniques during liver transplant and the potential benefits to the transplanted heart to remain on cardio-pulmonary bypass during liver implantation led to revising this strategy.…”

Cardiac and Multi-Organ Transplantation in Patients with Amyloidosis

“…In the Mayo Clinic report three of the four patients with ALA 60 ATTR mutation remain alive and one died of progressive renal failure; three patients with the TYR 77 ATTR variant remain alive and had no amyloid deposition on the last endomyocardial biopsy. Autonomic disturbances, modified body mass index, duration of symptoms, polyneuropathy, disability score, orthostatic hypotension, gastrointestinal and urinary tract dysfunction are important factors in the preoperative evaluation (Grazi et al, 2003;Sharma et al, 2003;Pilato et al, 2007) and optimizing the timing for CHLTx appears to be crucial.…”

“…Transplantation of the heart but maintaining the patient on cardio-pulmonary bypass during the liver transplant was described. Subsequent concerns about substantial coagulopathy and increased bleeding changed the strategy to performing liver implantation following separation from cardiopulmonary bypass (Befeler et al, 1999;Grazi et al, 2003;Shaw et al, 1985;Detry et al, 1997;Nardo et al, 2004). Improved surgical and anesthetic techniques during liver transplant, and the potential benefits to the transplanted heart to remain on cardio-pulmonary bypass during liver implantation led to revising this strategy for CHLTx.…”