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 ( 201 Tl) and iodine-123 beta-methyl 15-para-iodophenyl 3(R, S)-methylpentadecanoic acid ( 123 I-BMIPP) dual scintigraphy in patients with AFD. Methods Eighteen consecutive patients with AFD underwent resting myocardial 201 Tl/ 123 I-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 123 I-BMIPP—TDS on 201 Tl’. 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 123 I-BMIPP, and MS were all significantly greater in patients with MACEs compared with those without. Kaplan–Meier analysis indicated that high TDS on 123 I-BMIPP and high MS were associated with poor event-free survival. Conclusion TDS on 123 I-BMIPP was a better prognostic determinant in patients with AFD than TDS on 201 Tl. Myocardial 201 Tl/ 123 I-BMIPP dual scintigraphy may thus be a useful noninvasive modality for evaluating prognosis in patients with AFD.