Liver transplantation for familial amyloid polyneuropathy non-VAL30MET variants: are cardiac complications influenced by prophylactic pacing and immunosuppressive weaning?
BACKGROUND
Cardiac complications represent a cause of morbidity and mortality after liver transplantation among patients with familial amyloid polyneuropathy (FAP), especially for the non-VAL30MET variant types.


METHODS
We retrospectively evaluated 11 recipients from a nonendemic area including 90.9% affected by FAP variants. Preoperative cardiovascular symptoms were present in 81% of patients. An intraoperative pacemaker was placed prophylactically in 90.9% of all recipients. Since tacrolimus has been reported in the international literature to display cardiac toxicity, we evaluated the influence of intraoperative prophylactic pacing and rapid postoperative weaning from tacrolimus, mainly allowed by thymoglobulin on the occurrence of posttransplantation cardiac complications.


RESULTS
One patient received a combined heart-liver transplant, another, living donor liver transplantation. We did not observe any significant intraoperative cardiac complications. Postoperatively, the pacemaker was removed from all patients but 1. Five patients received tacrolimus and steroids; a subsequent, second group of 6 patients (54.5%) was treated with thymoglobulin followed by tacrolimus. At discharge the mean tacrolimus level was 10.6 ng/mL, whereas after 1 month it was 7.5 ng/mL. We observed a case of acute cellular rejection before discharge, which was successfully treated with intravenous steroids and OKT3. After a mean follow-up of 17.4 months (range, 1-31), 2 patients had died (18.1%): 1 due to sepsis and another, to MI. Two recipients experienced cardiac complications (18.1%), namely, the patient who died due to an myocardial infarction and a second one with a tachyarrhythmia, which was treated successfully with beta-blockers and amiodarone.


CONCLUSIONS
Prophylactic pacing and rapid weaning from immunosuppression are still associated with a significant rate of postoperative cardiac complications.
Lauro
A
A
UO Chirurgia dei Trapianti di Fegato e Multiorgano, PAD 25-Policlinico S. Orsola-Malpighi, Universitá di Bologna, Via Massarenti 9, 40138 Bologna, Italy. augustola@yahoo.com
Diago Usò
T
T
Masetti
M
M
Di Benedetto
F
F
Cautero
N
N
De Ruvo
N
N
Dazzi
A
A
Quintini
C
C
Begliomini
B
B
Siniscalchi
A
A
Ramacciato
G
G
Risaliti
A
A
Miller
C M
CM
Pinna
A D
AD
D000328:Adult; D000368:Aged; D019943:Amino Acid Substitution; D028227:Amyloid Neuropathies, Familial / Q000235:genetics* / Q000601:surgery*; D004334:Drug Administration Schedule; D005260:Female; D014644:Genetic Variation; D006084:Graft Rejection / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000493:pharmacokinetics / Q000627:therapeutic use*; D016031:Liver Transplantation; D008297:Male; D008715:Methionine; D008875:Middle Aged; D009154:Mutation; D011228:Prealbumin / Q000235:genetics*; D012189:Retrospective Studies; D016559:Tacrolimus / Q000493:pharmacokinetics / Q000627:therapeutic use; D014633:Valine
D007166:Immunosuppressive Agents; D011228:Prealbumin; D008715:Methionine; D014633:Valine; D016559:Tacrolimus