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

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

Research output: Contribution to journalArticle

43 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)645-647
Number of pages3
JournalTransplantation Proceedings
Volume36
Issue number3
DOIs
Publication statusPublished - Apr 2004

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Familial Amyloidosis
Heart Transplantation
Liver Transplantation
Amyloidosis
Orthostatic Hypotension
Prealbumin
Gastrointestinal Motility
Polyneuropathies
Intraoperative Complications
Patient Rights
Peripheral Nervous System Diseases
Cardiomyopathies
Renal Insufficiency
Hepatocellular Carcinoma
Sepsis

ASJC Scopus subject areas

  • Surgery
  • Transplantation

Cite this

Nardo, B., Beltempo, P., Bertelli, R., Montalti, R., Vivarelli, M., Cescon, M., ... Cavallari, A. (2004). Combined heart and liver transplantation in four adults with familial amyloidosis: Experience of a single center. Transplantation Proceedings, 36(3), 645-647. https://doi.org/10.1016/j.transproceed.2004.03.076

Combined heart and liver transplantation in four adults with familial amyloidosis : Experience of a single center. / Nardo, B.; Beltempo, P.; Bertelli, R.; Montalti, R.; Vivarelli, M.; Cescon, M.; Grazi, G. L.; Salvi, F.; Magelli, C.; Grigioni, F.; Arpesella, G.; Martinelli, G.; Cavallari, A.

In: Transplantation Proceedings, Vol. 36, No. 3, 04.2004, p. 645-647.

Research output: Contribution to journalArticle

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 2004, 'Combined heart and liver transplantation in four adults with familial amyloidosis: Experience of a single center', Transplantation Proceedings, vol. 36, no. 3, pp. 645-647. https://doi.org/10.1016/j.transproceed.2004.03.076
Nardo, B. ; Beltempo, P. ; Bertelli, R. ; Montalti, R. ; Vivarelli, M. ; Cescon, M. ; Grazi, G. L. ; Salvi, F. ; Magelli, C. ; Grigioni, F. ; Arpesella, G. ; Martinelli, G. ; Cavallari, A. / Combined heart and liver transplantation in four adults with familial amyloidosis : Experience of a single center. In: Transplantation Proceedings. 2004 ; Vol. 36, No. 3. pp. 645-647.
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