Potential prognostic implications of myocardial thallium-201 and iodine-123-beta-methylpentadecanoic acid dual scintigra
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ORIGINAL ARTICLE
Potential prognostic implications of myocardial thallium‑201 and iodine‑123‑beta‑methylpentadecanoic acid dual scintigraphy in patients with Anderson–Fabry disease Tomoaki Haga1,2 · Takahiro Okumura1 · Satoshi Isobe1 · Fuji Somura2 · Naoaki Kano1 · Tasuku Kuwayama1 · Tsuyoshi Yokoi1 · Hiroaki Hiraiwa1 · Toru Kondo1 · Akinori Sawamura1 · Ryota Morimoto1 · Hiroshi Yamamoto3 · Kazuya Tsuboi3 · Toyoaki Murohara1 Received: 16 August 2019 / Accepted: 26 September 2019 © The Japanese Society of Nuclear Medicine 2019
Abstract Objectives Information on the relationship between myocardial damage assessed by myocardial scintigraphy and prognosis in patients with Anderson–Fabry disease (AFD) is lacking. We therefore aimed to investigate the prognostic impacts of myocardial thallium-201 (201Tl) and iodine-123 beta-methyl 15-para-iodophenyl 3(R, S)-methylpentadecanoic acid (123I-BMIPP) dual scintigraphy in patients with AFD. Methods Eighteen consecutive patients with AFD underwent resting myocardial 201Tl/123I-BMIPP dual scintigraphy. Total defect scores (TDS) on both images were calculated visually according to the 17-segment model using a 5-point scoring system. The mismatch score (MS) was calculated as ‘TDS on 123I-BMIPP—TDS on 201Tl’. Results Six major adverse cardiac events (MACEs) were recorded during a mean follow-up of 6.7 ± 4.2 years (three heart failure requiring hospitalization and three cardiac deaths). Left ventricular mass index, left atrial diameter, brain natriuretic peptide, TDS on 123I-BMIPP, and MS were all significantly greater in patients with MACEs compared with those without. Kaplan–Meier analysis indicated that high TDS on 123I-BMIPP and high MS were associated with poor event-free survival. Conclusion TDS on 123I-BMIPP was a better prognostic determinant in patients with AFD than TDS on 201Tl. Myocardial 201 Tl/123I-BMIPP dual scintigraphy may thus be a useful noninvasive modality for evaluating prognosis in patients with AFD. Keywords Anderson–Fabry disease · 201Tl/123I-BMIPP dual scintigraphy · Defect score · Prognosis
Introduction Anderson–Fabry disease (AFD) is a rare, X-linked recessive lysosomal metabolic disorder caused by deficiency or mutation of α-galactosidase A. This disease is characterized by progressive deposition of globotriaosylceramide in various cell types and tissues, leading to renal, cerebrovascular, and * Takahiro Okumura [email protected]‑u.ac.jp 1
Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai‑cho, Showa‑ku, Nagoya, Aichi 466‑8560, Japan
2
Department of Cardiology, Nagoya Central Hospital, 3‑7‑7 Taikou, Nakamura‑ku, Nagoya, Aichi 466‑8560, Japan
3
LSD Center, Nagoya Central Hospital, 3‑7‑7 Taikou, Nakamura‑ku, Nagoya, Aichi 466‑8560, Japan
cardiovascular dysfunction [1, 2]. Cardiac manifestations of this disease are frequently characterized by left ventricle (LV) hypertrophy, which mimics hypertrophic cardiomyopathy (HCM) [3]. Given that progressive cardiac involvement and subsequent fatal arrhythmia
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