Transthyretin-Derived (ATTR) Amyloidotic Cardiomyopathy After Receiving a Domino Liver Allograft

Berg, Maarten P. van den; Slart, Riemer H. J. A.; Blokzijl, Hans; Hazenberg, Bouke P. C.

doi:10.1161/circulationaha.115.016264

Cited by 9 publications

(5 citation statements)

References 4 publications

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“…1 One p.Val71Ala DLTX recipient aged 59 years developed neuropathy and cardiomyopathy 5 years after transplant but was still alive 9 years later. 4 Our patient was considerably older (70 years) at the time of transplant, and older age has been identified as an important accelerator of domino recipient amyloid neuropathy. 5 The limited inflammation in our case seems coincidental occurring in up to one-third or amyloid sural nerve biopsies.…”

Section: Discussionmentioning

confidence: 77%

Fatal TTR amyloidosis with neuropathy from domino liver p.Val71Ala transplant

Puffer

Spinner

et al. 2019

Neurol Genet

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Section: Discussionmentioning

confidence: 77%

Fatal TTR amyloidosis with neuropathy from domino liver p.Val71Ala transplant

Puffer

Spinner

et al. 2019

Neurol Genet

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“…Autopsy case of a DLT patient with asymptomatic amyloidosis at 8 years after transplantation (died from lymphoma) showed amyloid deposits in the heart, lungs, gastrointestinal tract (upper and lower), pancreas, spleen, testes, epididymis, prostate, skeletal muscle, thoracic sympathetic ganglia and median nerve (carpal tunnel syndrome)[ 26 ]. Amyloid deposition in the myocardium after DLT is usually asymptomatic, and so far only two cases of de novo cardiomyopathy after DLT have been reported with Val71Ala and Val30Met mutations[ 27 , 28 ]. One of the patients developed cardiac amyloidosis 5 years after re-transplantation for systemic de novo amyloidosis after DLT, and it is unclear whether cardiac amyloidosis was related to wild-type or mutant transthyretin[ 28 ].…”

Section: Discussionmentioning

confidence: 99%

Iatrogenic amyloid polyneuropathy after domino liver transplantation

Mnatsakanova

Živković

2017

WJH

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Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing de novo systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations. De novo amyloid neuropathy has been described in up to 23% of transplant recipients. Neuropathy may be preceded by asymptomatic amyloid deposition in various tissues and symptoms of neuropathy started after a median of 7 years following DLT (5.7 ± 3.2 years; range 2 mo to 10 years). Typical initial symptoms include neuropathic pain and sensory loss, while dysautonomia usually starts later. Progression of neuropathy may necessitate liver re-transplantation, and subsequent improvement of neuropathy has been reported in some patients. Explant allograft recipients need close monitoring for signs of systemic amyloidosis, neuropathy and dysautonomia as progressive symptoms may require re-transplantation.

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“…In contrast, there are only a few case reports on ATTR-CM after DLT. 5,6,14 Therefore, the time-dependent changes in cardiac function after DLT have not been fully clarified. The present study showed that LVEF, the E/A ratio, the E/e' ratio, e' wave velocity, LAVI, and TRV did not differ significantly between the pre-and post-DLT periods, indicating that LV systolic and diastolic function was preserved in the long term after DLT.…”

Section: Time-dependent Changes In Echocardiographic Findings In Dlt mentioning

confidence: 99%

“…1,2 there are only a few case reports of TTR amyloid cardiomyopathy (ATTR-CM) after DLT. 5,6 Thus, the safety of DLT has not been fully evaluated in the heart. The hallmarks of ATTR-CM on transthoracic echocardiography (TTE) are increased left ventricular (LV) thickness, left atrial domino liver transplantation (DLT) to save the lives of patients with end-stage liver disease who have no other appropriate donor liver (Figure 1).…”

mentioning

confidence: 99%

Temporal Change in Longitudinal Strain After Domino Liver Transplantation With Liver Grafts Explanted From Patients With Hereditary Amyloidogenic Transthyretin Amyloidosis

et al. 2020

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Background: Using transthoracic echocardiography, including 2D speckle tracking imaging (STI), this study examined cardiac function after domino liver transplantation (DLT) with liver grafts explanted from patients with hereditary amyloidogenic transthyretin amyloidosis. Methods and Results: In all, 14 patients who underwent DLT at Kumamoto University Hospital and for whom 2D STI information was available were enrolled in the study; time-dependent echocardiographic changes were evaluated in 7. Although left ventricular (LV) systolic and diastolic function did not differ between the pre- and post-DLT periods (mean [±SD] 5.4±1.0 years after DLT), there were significant (P<0.05 for all) increases in the post- vs. pre-DLT period in basal longitudinal strain (LS; −13.4±2.3 vs. −19.3±4.4), relative apical LS index (=apical LS/[basal LS+mid LS]; 0.75±0.20 vs. 0.58±0.08), and LV ejection fraction/global LS (3.91±0.58 vs. 3.06±0.44). Age at the time of DLT was significantly higher in the group with impaired (>−14%) than preserved basal LS (57.2±3.5 vs. 39.6±16.0 years; P<0.05). When control subjects (n=14) were added to the enrolled DLT recipients, multivariable logistic regression analysis revealed that a history of DLT was significantly associated with impaired basal LS (>−14%; odds ratio 28.39, 95% confidence interval 1.89–427.45, P<0.05). Conclusions: LV systolic and diastolic function was preserved in the long term after DLT. However, 2D STI revealed subtle cardiac dysfunction in DLT recipients, which may be an early manifestation of cardiac amyloidosis.

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Transthyretin-Derived (ATTR) Amyloidotic Cardiomyopathy After Receiving a Domino Liver Allograft

Cited by 9 publications

References 4 publications

Fatal TTR amyloidosis with neuropathy from domino liver p.Val71Ala transplant

Fatal TTR amyloidosis with neuropathy from domino liver p.Val71Ala transplant

Iatrogenic amyloid polyneuropathy after domino liver transplantation

Temporal Change in Longitudinal Strain After Domino Liver Transplantation With Liver Grafts Explanted From Patients With Hereditary Amyloidogenic Transthyretin Amyloidosis

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