{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T23:41:21Z","timestamp":1769125281765,"version":"3.49.0"},"reference-count":33,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2015,10,21]],"date-time":"2015-10-21T00:00:00Z","timestamp":1445385600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Transplantation"],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Familial transthyretin (<jats:styled-content style=\"fixed-case\">TTR<\/jats:styled-content>) amyloidosis is caused by different <jats:styled-content style=\"fixed-case\">TTR<\/jats:styled-content> mutations resulting in different clinical phenotypes of the disease. The <jats:italic>Leu111Met<\/jats:italic> mutation causes severe restrictive cardiomyopathy. Liver transplantation (<jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x) is an established treatment option for patients with <jats:styled-content style=\"fixed-case\">TTR<\/jats:styled-content> amyloidosis; however, information on outcome after isolated <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x in patients with <jats:italic><jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>eu111Met<\/jats:italic> mutation amyloidosis is limited.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Between 2005 and 2012, six patients with <jats:styled-content style=\"fixed-case\">TTR <\/jats:styled-content><jats:italic>Leu111Met<\/jats:italic> amyloidosis underwent isolated orthotopic <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x. None suffered from neuropathy. Prior to <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x, patients presented with echocardiographic manifestations of early cardiac amyloid involvement and in five endomyocardial biopsy was positive for <jats:styled-content style=\"fixed-case\">TTR<\/jats:styled-content> amyloid.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Median age at <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x was 45.5 yr (range 39\u201354), and four were male (67%). All patients were alive at a median follow\u2010up of 56.6 months (range 18\u2013104). No surgical complications occurred. Two patients (33%) underwent cardiac transplantation during follow\u2010up due to progressive cardiomyopathy. The remaining four patients experienced no echocardiographic or clinical deterioration of cardiac function following <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Isolated <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x appears to be a valuable treatment option for patients with familial <jats:styled-content style=\"fixed-case\">TTR<\/jats:styled-content> amyloidosis due to <jats:italic><jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>eu111Met<\/jats:italic> mutation. Appropriate timing of <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>x is of utmost importance to avoid development of severe amyloid cardiomyopathy and the need for combined heart and liver transplantation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ctr.12633","type":"journal-article","created":{"date-parts":[[2015,9,11]],"date-time":"2015-09-11T08:09:54Z","timestamp":1441958994000},"page":"1098-1104","source":"Crossref","is-referenced-by-count":6,"title":["Outcome in patients treated with isolated liver transplantation for familial transthyretin amyloidosis to prevent cardiomyopathy"],"prefix":"10.1111","volume":"29","author":[{"given":"L\u00e6rke M.","family":"Nelson","sequence":"first","affiliation":[{"name":"Department of Cardiology The Heart Centre Rigshospitalet Copenhagen University Hospital Copenhagen Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luit","family":"Penninga","sequence":"additional","affiliation":[{"name":"Department of 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