Anesthetic Management of a Combined Heart and Liver Transplantation in an Amyloidotic Patient: A Case Report

Barrio, Itsaso Montalbán del; Guereñu, M.A. Mtnez de; Real, M. Isabel; Campo, Irene; Pérez-Cerdá, F; Moreno, Enrique

doi:10.1016/j.transproceed.2007.07.058

Cited by 11 publications

(7 citation statements)

References 5 publications

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“…However, a detailed characterization of cardiac symptoms has not been published so far. Heart transplantation has been performed along with LT in some centers 25–27. In the majority, heart‐liver transplantation has been performed in patients with Val30Met mutations suffering from rhythmic disturbances 28.…”

Section: Discussionmentioning

confidence: 99%

Liver transplantation and combined liver-heart transplantation in patients with familial amyloid polyneuropathy: A single-center experience

et al. 2010

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Liver transplantation (LT) is the only curative option for patients with familial amyloid polyneuropathy (FAP) at present. Twenty patients with FAP underwent LT between May 1998 and June 2007. Transthyretin mutations included predominantly the Val30Met mutation but also 10 other mutations. Seven patients received a pacemaker prior to LT, and because of impairment of mechanical cardiac function, 4 combined heart-liver transplants were performed, 1 simultaneously and 3 sequentially. The first patient, who underwent simultaneous transplantation, died. Seven patients died after LT, with 5 dying within the first year after transplantation. The causes of death were cardiac complications (4 patients), infections (2 patients), and malnutrition (1 patient). One-year survival was 75.0%, and 5-year survival was 64.2%. Gly47Glu and Leu12-Pro mutations showed an aggressive clinical manifestation: 2 patients with the Gly47Glu mutation, the youngest patients of all the non-Val30Met patients, suffered from severe cardiac symptoms leading to death despite LT. Two siblings with the Leu12Pro mutation, who presented only with grand mal seizures, died after LT because of sepsis. In conclusion, the clinical course in patients with FAP is very variable. Cardiac symptoms occurred predominantly in patients with non-Val30Met mutations and prompted combined heart-liver transplantation in 4 patients. Although early LT in Val30Met is indicated in order to halt the typical symptoms of polyneuropathy, additional complications occurring predominantly with other mutations may prevail and lead to life-threatening complications or a fatal outcome. Combined heart-liver transplantation should be considered in patients with restrictive cardiomyopathy. Liver Transpl 16:314-323,

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Section: Discussionmentioning

confidence: 99%

Liver transplantation and combined liver-heart transplantation in patients with familial amyloid polyneuropathy: A single-center experience

et al. 2010

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“…Basically, because of cardiac compromise, FAP patients undergoing LT are usually regarded as difficult to manage 20. In this context, there are 2 fundamental issues: the systematic pretransplant implantation of a biventricular pacemaker and, more importantly, the avoidance of an acute preload decrease during the procedure.…”

Section: Discussionmentioning

confidence: 99%

The influence of the explant technique on the hemodynamic profile during sequential domino liver transplantation in familial amyloid polyneuropathy patients

et al. 2009

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Familial amyloidotic polyneuropathy (FAP) patients present adrenergic cardiac input blockade secondary to amyloid deposits and sympathetic neuropathy. Consequently, their capacity to compensate for hemodynamic changes is limited. To avoid hemodynamic disturbances in sequential liver transplants, a standard procedure with venovenous bypass or inferior vena cava preservation is contemplated. The aim of this study was to evaluate the impact of both techniques on the hemodynamic management and outcome of patients affected by FAP and scheduled for a domino liver transplantation program. We evaluated 36 FAP patients. Venovenous bypass was performed for 20 patients (the venovenous bypass group), whereas the vena cava preservation technique was used for the remaining 16 patients (the cava preservation group). The time that elapsed from FAP diagnosis to liver transplantation was 3.2 Ϯ 2.7 years. Peripheral neuropathy was present in all patients, autonomic dysfunction was present in 71%, and cardiac involvement was present in 69%. Renal and gastrointestinal manifestations were reported in 19% and 53% of patients, respectively. The 1-, 3-, and 5-year survival rates were 97%, 93%, and 93%, respectively. Intraoperative hemodynamic and cardiac disorders, need for vasoactive drugs, blood loss, and transfusion requirements were recorded. Postoperative outcome and cardiac and renal complications were also recorded. No significant differences in disease severity or demographic characteristics were observed. Among all the variables studied, only the total ischemia time and time in surgery were significantly longer in the venovenous bypass group patients (P Յ 0.05). During the postoperative period, the incidence of minor cardiovascular events, incidence of acute renal dysfunction, and outcomes were similar in the 2 groups. In conclusion, either preservation of the vena cava or the standard technique with venovenous bypass can be safely used in FAP patients during liver transplantation. Liver Transpl 15:869-875, 2009.

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“…Even though arrhythmias are frequent in AC, bradyarrhythmias are rare [20], but are identifiable under anesthesia, the main cause being, most likely, the presence of infiltrative lesions at the level of the cardiac conduction system, leading to an increased susceptibility to the depressant action of various anesthetic substances [19]. Another possible cause is autonomic heart dysfunction cause by amyloid neuropathy [19,21].…”

Section: Discussionmentioning

confidence: 99%

Intraoperative death due to nodular amyloidosis cardiomyopathy associated with fat pulmonary embolism

Ceauşu¹,

Luca²,

Hostiuc³

et al. 2013

RJLM

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Cardiac amyloidosis is caused by extracellular deposits containing low molecular weight protein subunits arranged in a beta sheet configuration. By gross examination amyloid deposits are identifiable as localized tan, waxy appearing lesions affecting almost always the atria (usually atrial endocardium), valve leaflets, ventricular, but also in the coronary lumen, sometimes leading to severe coronary stenosis. Fat pulmonary embolism is a known complication of femoral fractures, being more frequent in untreated cases compared to those suffering surgical interventions. We present a case in which a female patient with cardiac amyloidosis died on the operating table, the direct cause of death being fat pulmonary emboli, and discuss the involvement of the associated cardiac amyloidosis in thanatogenesys.

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Anesthetic Management of a Combined Heart and Liver Transplantation in an Amyloidotic Patient: A Case Report

Cited by 11 publications

References 5 publications

Liver transplantation and combined liver-heart transplantation in patients with familial amyloid polyneuropathy: A single-center experience

Liver transplantation and combined liver-heart transplantation in patients with familial amyloid polyneuropathy: A single-center experience

The influence of the explant technique on the hemodynamic profile during sequential domino liver transplantation in familial amyloid polyneuropathy patients

Intraoperative death due to nodular amyloidosis cardiomyopathy associated with fat pulmonary embolism

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