Potential prognostic implications of myocardial thallium-201 and iodine-123-beta-methylpentadecanoic acid dual scintigraphy in patients with Anderson–Fabry disease

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 ²⁰¹Tl/¹²³I-BMIPP dual scintigraphy may thus be a useful noninvasive modality for evaluating prognosis in patients with AFD.