Manejo perioperatorio del trasplante hepático en la polineuropatía amiloidótica familiar con afectación cardíaca / Perioperative management for liver transplant in a patient with familial amyloid polyneuropathy with heart involvement

La polineuropatía amiloidótica familiar (PAF) es una amiloidosis sistémica causada por la mutación de la transtirretina. La amiloidosis cardíaca, el determinante pronóstico principal de la amiloidosis sistémica, se caracteriza por infiltración del miocardio, dando lugar a cardiomiopatía y alteraciones de la conducción. El trasplante hepático es la única opción curativa para los pacientes afectos de PAF. Presentamos el caso de un paciente de 36 años con PAF tipo i con afectación cardíaca propuesto para cirugía de trasplante hepático, la cual fue llevada a cabo con éxito sin eventos perioperatorios de interés (AU)

Familial amyloid polyneuropathy (FAP) is a systemic amyloidosis caused by mutated transthyretin. Cardiac amyloidosis, the major prognostic determinant in systemic amyloidosis, is characterized by infiltration of the myocardium, leading to cardiomyopathy and conduction disturbances. Liver transplantation is the only curative option for patients with FAP. The case is presented of a 36-year-old patient with type i FAP with cardiac involvement, proposed for liver transplant surgery, which was successfully performed without any preoperative event of interest (AU)

[Perioperative management for liver transplant in a patient with familial amyloid polyneuropathy with heart involvement]. / Manejo perioperatorio del trasplante hepático en la polineuropatía amiloidótica familiar con afectación cardíaca.

Familial amyloid polyneuropathy (FAP) is a systemic amyloidosis caused by mutated transthyretin. Cardiac amyloidosis, the major prognostic determinant in systemic amyloidosis, is characterized by infiltration of the myocardium, leading to cardiomyopathy and conduction disturbances. Liver transplantation is the only curative option for patients with FAP. The case is presented of a 36-year-old patient with type i FAP with cardiac involvement, proposed for liver transplant surgery, which was successfully performed without any preoperative event of interest.

Changes of lactate levels during cardiopulmonary bypass in patients undergoing cardiac transplantation: possible early marker of morbidity and mortality.

OBJECTIVES: High levels of lactate are associated with tissue hypoperfusion during cardiac surgery resulting in postoperative morbidity and mortality among patients undergoing cardiopulmonary bypass (CBP). Our goal was to evaluate the change in lactate levels during CBP for their possible predictive value for complications after heart transplant surgery. MATERIALS AND METHODS: From January to December 2010 we studied lactate levels in 16 heart transplant patients. Arterial blood samples were collected before, during, and after cardiopulmonary bypass on admission to the intensive care unit (ICU). Lactate levels were measured using the cobas B221 (Roche Diagnostic). The neurological, lung, and kidney complications were associated with mortality within 30 days. RESULTS: One patient displayed lactate levels > 2 mmol/L before bypass while 4 (25%) showed levels > 4 mmol/L during CPB. Lactate values higher than or equal to 4 mmol/L on ICU admission occurred in nine patients (56%). Postoperative mortality was higher among the group with levels above below 4 mmol/L on ICU admission (18.7% vs 6.2%). Neurological complications were observed in 22% of patients with elevated levels as opposed to none of the patients with levels below 4 mmol/L. Pulmonary complications were noted in 22% of patients with high lactate values versus 0% among the other group. CONCLUSION: Hyperlactemia above certain levels occurring during CPB serve as a biomarker to identify early postoperative morbidity and mortality.