Potential prognostic implications of myocardial thallium-201 and iodine-123-beta-methylpentadecanoic acid dual scintigraphy in patients with Anderson–Fabry disease
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.
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’.
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.
TDS on 123I-BMIPP was a better prognostic determinant in patients with AFD than TDS on 201Tl. Myocardial 201Tl/123I-BMIPP dual scintigraphy may thus be a useful noninvasive modality for evaluating prognosis in patients with AFD.
KeywordsAnderson–Fabry disease 201Tl/123I-BMIPP dual scintigraphy Defect score Prognosis
This research received no grant from any funding agency in the public, commercial or not-for-profit sectors.
Compliance with ethical standards
Conflict of interest
T.O. received lecture fees from Ono Yakuhin, Medtronics, and Otsuka, and research grants from Ono Yakuhin, Bayer, Daiichi-Sankyo, and Amgen Astellas, not in connection with the submitted work. S.I. received lecture fee from FUJIFILM RI Pharma and Nihon Medi-Physics. T.M. received lecture fees and unrestricted research grants for the Department of Cardiology, Nagoya University Graduate School of Medicine, from Bayer, Daiichi-Sankyo, Dainippon Sumitomo, Kowa, MSD, Mitsubishi Tanabe, Boehringer Ingelheim, Novartis, Pfizer, Sanofi-Aventis, Takeda, Astellas, Otsuka, and Teijin.
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