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Combined heart and liver transplantation in four adults with familial amyloidosis: experience of a single center.

Abstract：

:There are few reports of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy (FAP). The technique for the operation remains to be defined. Four CHLTs were performed for amyloidogenic transthyretin-related (variant Glu89Gln-ATTR Glu89Gln) cardiomyopathy in our center. Patients 1 and 4 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 four patients underwent cardiac and sequential hepatic transplantation with organs procured from the same donor. Venovenous bypass was used in patients 1 and 4 who experienced uncomplicated procedures. The amyloidotic liver of patient 4 was successfully utilized for a domino procedure to treat a patient with hepatocellular carcinoma on cirrhosis. The cardiac performance of patients 1 and 4 remains normal; there has been no progression of amyloidosis at 42 and 1 months after transplantation. Patient 2 had no intraoperative complications but experienced postoperative bleeding, renal failure, sepsis, and heart failure, and finally died of multiorgan failure 2 months after transplant. In patient 3, right hemicolectomy was required intraoperatively due to intestinal ischemia, without significant hemodynamic instability, while extracardiac symptoms of amyloidosis gradually worsened postoperatively. In conclusion, CHLT for ATTR Glu89Gln may be performed even in patients with advanced disease. However, the most compromised patients are more likely to display intraoperative risks, postoperative complications, and worsening of extracardiac, extrahepatic symptoms.

journal_name

Transplant Proc

journal_title

Transplantation proceedings

authors

Nardo B,Beltempo P,Bertelli R,Montalti R,Vivarelli M,Cescon M,Grazi GL,Salvi F,Magelli C,Grigioni F,Arpesella G,Martinelli G,Cavallari A

doi

10.1016/j.transproceed.2004.03.076

subject

Has Abstract

pub_date

2004-04-01 00:00:00

pages

645-7

issue

eissn

0041-1345

issn

1873-2623

pii

S0041134504003379

journal_volume

pub_type

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Combined heart and liver transplantation in four adults with familial amyloidosis: experience of a single center.