Evaluation of Myocardial Changes in Familial Amyloid Polyneuropathy after Liver Transplantation

Okamoto, Sadahisa; Yamashita, Taro; Ando, Yumiko; Ueda, Mitsuharu; Misumi, Yohei; Obayashi, Konen; Horibata, Yoko; Uchino, Makoto

doi:10.2169/internalmedicine.47.1399

Cited by 30 publications

(14 citation statements)

References 19 publications

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“…In some patients with pre-existing cardiac involvement, progression of amyloid apposition was not halted due to continued deposition of amyloid aggregates from normal TTR aggregates [37–39]. No therapeutic effect of liver transplantation has been observed in SSA patients.…”

Section: Discussionmentioning

confidence: 99%

Green tea halts progression of cardiac transthyretin amyloidosis: an observational report

et al. 2012

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Background Treatment options in patients with amyloidotic transthyretin (ATTR) cardiomyopathy are limited. Epigallocatechin-3-gallate (EGCG), the most abundant catechin in green tea (GT), inhibits fibril formation from several amyloidogenic proteins in vitro. Thus, it might also halt progression of TTR amyloidosis. This is a singlecenter observational report on the effects of GT consumption in patients with ATTR cardiomopathy. Methods 19 patients with ATTR cardiomyopathy were evaluated by standard blood tests, echocardiography, and cardiac MRI (n = 9) before and after consumption of GT and/or green tea extracts (GTE) for 12 months.Results Five patients were not followed up for reasons of death (n = 2), discontinuation of GT/GTE consumption (n = 2), and heart transplantation (n = 1). After 12 months no increase of left ventricular (LV) wall thickness and LV myocardial mass was observed by echocardiography. In the subgroup of patients evaluated by cardiac MRI a mean decrease of LV myocardial mass (-12.5 %) was detected in all patients. This was accompanied by an increase of mean mitral annular systolic velocity of 9 % in all 14 patients. Total cholesterol (191.9 ± 8.9 vs. 172.7 ± 9.4 mg/dL; p \ 0.01) and LDL cholesterol (105.8 ± 7.6 vs. 89.5 ± 8.0 mg/dL; p \ 0.01) decreased significantly during the observational period. No serious adverse effects were reported by any of the participants. Conclusions Our observation suggests an inhibitory effect of GT and/or GTE on the progression of cardiac amyloidosis. We propose a randomized placebo-controlled investigation to confirm our observation. K. Altland, H. A. Katus contributed equally to this work.Electronic supplementary material The online version of this article (doi:10.1007/s00392-012-0463-z) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Green tea halts progression of cardiac transthyretin amyloidosis: an observational report

et al. 2012

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“…Especially problematic is the fact that in some transplanted individuals, a rapid continued deposition is taking place in cardiac tissue, presumably caused by the addition of wtTTR to the amyloid [16, 17]. Mostly, this has been seen in patients with other mutations than V30M [18, 19], but progression of cardiac amyloidosis has been reported to occur also in some V30M patients [20–23]. The special vulnerability of heart tissue for continued deposition after transplantation and the reason why some patients benefit from the procedure, whereas amyloid progression is triggered in others, are not understood.…”

Section: Introductionmentioning

confidence: 99%

Variation in amount of wild-type transthyretin in different fibril and tissue types in ATTR amyloidosis

et al. 2010

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Familial transthyretin (TTR) amyloidosis is caused by a mutation in the TTR gene, although wild-type (wt) TTR is also incorporated into the amyloid fibrils. Liver transplantation (LT) is the prevailing treatment of the disease and is performed in order to eliminate the mutant TTR from plasma. The outcome of the procedure is varied; especially problematic is a progressive cardiomyopathy seen in some patients, presumably caused by continued incorporation of wtTTR. What determines the discrepancy in outcome is not clear. We have previously shown that two structurally distinct amyloid fibrils (with or without fragmented ATTR) are found among ATTRV30M patients. In this study, we investigated the proportion of wtATTR in cardiac and adipose amyloid from patients having either fibril type. It was found that cardiac amyloid more easily incorporates wtTTR than adipose amyloid, offering a potential explanation for the vulnerability of cardiac tissue for continued amyloidosis after LT. In cardiac tissue, fibrils with fragmented ATTR contained a higher wt proportion than fibrils without, suggesting that continued incorporation of wtTTR after LT, perhaps, can take place more easily in these patients. In adipose tissue, a rapid increase in wt proportion after LT indicates that a rather fast turnover of the deposits must occur. A difference in wt proportion between the fibril types was seen post-LT but not pre-LT, possibly caused by differences in turnover rate. Conclusively, this study further establishes the basic dissimilarities between the two fibril types and demonstrates that their role in LT outcome needs to be further investigated.

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“…[12][13][14][15][16] Moreover, liver transplantation sometimes shortens survival of patients with postoperative complications, such as acute rejection, portal vein thrombosis, or infectious diseases resulting from administration of immunosuppressive drugs. Also, some patients can have progressive amyloidosis, including cardiomyopathy, even after liver transplantation, perhaps because of continuing amyloid fibril formation from circulating wild-type TTR produced by the transplanted liver that traps preexisting deposits of amyloid.…”

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confidence: 99%

Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy

Yamashita¹,

Ando²,

Okamoto³

et al. 2012

Neurology

139

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Evaluation of Myocardial Changes in Familial Amyloid Polyneuropathy after Liver Transplantation

Abstract: Objective The progression of cardiac amyloidosis is a prognostic factor after liver transplantation (LT)

Cited by 30 publications

References 19 publications

Green tea halts progression of cardiac transthyretin amyloidosis: an observational report

Green tea halts progression of cardiac transthyretin amyloidosis: an observational report

Variation in amount of wild-type transthyretin in different fibril and tissue types in ATTR amyloidosis

Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy

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