神戸大学附属図書館デジタルアーカイブ
https://hdl.handle.net/20.500.14094/90009085

このアイテムのアクセス数:87件（2025-09-05 14:36 集計）

閲覧可能ファイル 

ファイル フォーマット サイズ 閲覧回数 説明
90009085 (fulltext) pdf 808 KB 39

メタデータ

ファイル出力

メタデータID
90009085
アクセス権
open access
出版タイプ
Version of Record
タイトル
Marked recovery of cardiac function by chemotherapy and autologous stem cell transplantation of a patient with heart failure with preserved ejection fraction due to primary amyloid light-chain amyloidosis: a case report
著者
Tanaka, Hidekazu ; Kitao, Akihito ; Minami, Hironobu ; Hirata, Ken-Ichi
著者ID
A1439
研究者ID
1000020590342
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=f08c30b91aeb7da2520e17560c007669
著者名
Tanaka, Hidekazu
田中, 秀和
タナカ, ヒデカズ
所属機関名
医学研究科
著者ID
A2850
研究者ID
1000040616455
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=6c79d27fc435bf9f520e17560c007669
著者名
Kitao, Akihito
北尾, 章人
キタオ, アキヒト
所属機関名
医学部附属病院
著者ID
A1398
研究者ID
1000060450574
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=29f74a5e8b26a0a9520e17560c007669
著者名
Minami, Hironobu
南, 博信
ミナミ, ヒロノブ
所属機関名
医学研究科
著者ID
A0883
研究者ID
1000020283880
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=383af6bc2b817735520e17560c007669
著者名
Hirata, Ken-Ichi
平田, 健一
ヒラタ, ケンイチ
所属機関名
医学研究科
言語
English (英語)
収録物名
European Heart Journal : Case Reports
巻(号)
6(3)
ページ
ytac016
出版者
Oxford University Press (OUP)
刊行日
2022-03-02
公開日
2022-03-29
抄録
Background Cardiac involvement of amyloid light-chain (AL) amyloidosis is strongly associated with poor outcome, but the early detection of cardiac involvement of AL amyloidosis can be challenging. Case summary We present a case of 49-year-old-female with heart failure with preserved ejection fraction. Echocardiography revealed normal left ventricular (LV) ejection fraction of 63% and an enlarged left atrium with a left atrial volume index (LAVI) of 54 mL/m2. Mild LV hypertrophy with an interventricular septum of 12.3 mm and posterior wall thickness of 11.0 mm was observed, and Doppler-derived LV diastolic filling showed a restrictive filling pattern. The conventional echocardiographic findings did not unequivocally indicate typical cardiac amyloidosis, but global longitudinal strain (GLS) was as low as 14.2%, and an apical sparing pattern was observed with relative apical longitudinal strain of 1.11. Finally, the patient was diagnosed as primary AL amyloidosis including histological examination of the endomyocardial specimen. After treatment with a regime of bortezomib and dexamethasone followed by high-dose melphalan followed by autologous peripheral blood stem cell transplantation (auto-PBSCT), Doppler-derived LV diastolic filling improved to normal filling pattern, and left atrial size had also decreased with an LAVI of 31 mL/m2. Moreover, GLS improved to 19.8%, and the apical sparing pattern had disappeared with relative apical longitudinal strain of 0.62. The patient has been asymptomatic during 18-month follow-up after auto-PBSCT, and recovered LV function has been maintained. Discussion An earlier diagnosis of cardiac amyloidosis by using apical sparing may therefore allow for earlier treatment intervention for AL amyloidosis.
カテゴリ
医学研究科
医学部附属病院
学術雑誌論文
権利
© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.
CC license
This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
関連情報
DOI
https://doi.org/10.1093/ehjcr/ytac016