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. 2017 Sep 8;26(1):174–187. doi: 10.1007/s12350-017-1059-9

Table 1.

Main results and patient outcome as reported in studies using Iodine-123 labelled metaiodobenzylguanidine scintigraphy in patients with amyloidosis

Study Author, year of publication Number of patients Tracer dose Collimator type Time point late HMR Amyloid typing Main results Patient outcome
1 Nakata et al4 1 patient 111 MBq (3 mCi) [I-123]-MIBG N/A 4 hours p.i. hATTR (TTR Val30Met) No cardiac tracer accumulation N/A
2 Tanaka et al5 12 patients 148 MBq (4 mCi) [I-123]-MIBG LE 3 hours p.i. hATTR No cardiac tracer accumulation in 8 of 12 Mean FU 15.5 ± 5.8 months: no lethal arrhythmia, no cardiac death
3 Delahaye et al6 17 patients, 12 healthy controls 300 MBq (8 mCi) [I-123]-MIBG LE 4 hours p.i. hATTR Mean late HMR in patients 1.36 ± 0.26 vs in healthy controls 1.98 ± 0.35 (P < 0.001), no difference in wash-out N/A
4 Delahaye et al35 21 patients, 12 healthy controls 150 and 180 MBq (4 and 5 mCi) [C-11]-MQNB and 300 MBq (8 mCi) [I-123]-MIBG LE 4 hours p.i. hATTR (20 patients TTR Val30Met, 1 patient TTR Thr49Ala) Mean muscarinic receptor density was higher in patients than in control subjects: B’max, 35.5 ± 8.9 vs 26.1 ± 6.7 pmol/mL (P = 0.003)
Mean late HMR in patients 1.43 ± 0.28 vs in healthy controls 1.98 ± 0.35 (P < 0.001), mean wash-out 29% ± 6.8% vs 21% ± 6% (P = 0.003). Individual muscarinic receptor density did not correlate with late HMR
N/A
5 Watanabe et al9 4 patients, 10 age-matched controls 111 MBq (3 mCi) [I-123]-MIBG N/A 4 hours p.i. hATTR (TTR Val30Met) Mean late HMR in patients 1.1 ± 0.2, vs 2.4 ± 0.2 in health controls (p-value N/A) N/A
6 Hongo et al8 25 patients, of which 16 patients without and 9 patients with autonomic neuropathy 111 MBq (3 mCi) [I-123]-MIBG LE 3 hours p.i. AL Mean late HMR in patients without autonomic neuropathy 1.53 ± 0.06 vs in with autonomic neuropathy 1.29 ± 0.05 (P < 0.001), mean wash-out 42 ± 4.8% vs 31 ± 4.0% (P < 0.001) N/A
7 Lekakis et al10 3 patients, 23 controls 185 MBq (5 mCi) [I-123]-MIBG LE 4 hours p.i. AL Mean late HMR 1.33 ± 0.1 vs in 2.13 ± 0.2 healthy controls (P value N/A) N/A
8 Coutinho et al28 34 patients, of which 2 patients without and 12 patients with autonomic neuropathy [I-123]-MIBG (dose N/A) N/A N/A hATTR Mean late HMR 1.75 ± 0.5 in all patients. Mean late HMR in patients without neuropathy 2.2 ± 0.5 vs patients with neuropathy 1.5 ± 0.4 (P = 0.001) N/A
9 Delahaye et al11 31 patients 300 MBq (8 mCi) [I-123]-MIBG LE 4 hours p.i. hATTR Mean late HMR 2 years after liver transplantation 1.46 ± 0.28 vs 6 months before liver transplantation 1.45 ± 0.29, P = not significant No cardiac death or lethal arrhythmia reported
10 Algalarrando et al36 32 patients 300 MBq (8 mCi) [I-123]-MIBG LE 4 hours p.i. hATTR Late HMR ≤1.6 in 26 out of 32 patients No cardiac death or lethal arrhythmia reported
11 Noordzij et al12 61 patients, 9 healthy control subjects 185 MBq (5 mCi) [I-123]-MIBG ME 4 hours p.i. AL (39 patients), AA (11 patients), ATTR (11 patients) Mean late HMR in all patients 2.3 ± 0.75 vs healthy control subjects 2.9 ± 0.58 (P < 0.005). Mean late HMR in ATTR patients 1.7 ± 0.75 vs AL patients 2.4 ± 0.75 (P < 0.05). Mean wash-out in patients 8.6% ± 14% vs in healthy control subjects −2.1% ± 10% (P < 0.05) No cardiac death or lethal arrhythmia
12 Noordzij et al37 2 patients 185 MBq (5 mCi) [I-123]-MIBG ME 4 hours p.i. wtATTR, hATTR (TTR Val122Ile) Patient A: late HMR 1.57, wash-out >20%, patient B: late HMR 1.13, wash-out 28% N/A
13 Coutinho et al21 143 patients 185 MBq (5 mCi) [I-123]-MIBG LE 3 hours p.i. hATTR (TTR Val30Met) Mean late HMR 1.83±0.43, and mean was-out 47±11% Mean FU 5.5 years: hazard ratio all-cause mortality 7 if HMR <1.6, progressive increase in 5-year mortality with decrease in late HMR
14 Takahashi et al38 6 patients [I-123]-MIBG (dose N/A) N/A N/A hATTR (TTR Val30Met) Mean late HMR at baseline 1.7 ± 0.9 vs after 3 year diflunisal treatment 1.9 ± 1.0 (P = 0.004). Mean wash-out at baseline 46% ± 20% vs after 3 years 43% ± 23% (P = 0.67) No cardiac death or lethal arrhythmia reported
15 Algalarrando et al22 215 patients 3 MBq/kg (0.08 mCi/kg) [I-123]-MIBG LE 4 hours p.i. hATTR (148 patients TTR Val30Met) Median late HMR 1.49 (Inter-quartile range 1.24–1.74, range 0.97–2.52) Median FU 5.9 years after liver transplantation: 5-year survival 64% if late HMR ≤1.43, vs 93% if HMR >1.43 (P < 0.0001)
16 Azevedo Coutinho et al23 232 patients 185 MBq (5 mCi) [I-123]-MIBG LE 3 hours p.i. hATTR (TTR Val30Met) Initial assessment: mean late HMR 1.83 ± 0.03, median wash-out 2.5 (Inter-quartile range −2.3–8.5)
During follow-up late HMR decreased with age and duration of neurological symptoms, but stabilized after liver transplantation.
Mean late HMR at inclusion was higher in patients who were still alive at the end of FU, compared to those who deceased: 1.90 ± 0.37 vs 1.58 ± 0.40, P < 0.001
Median FU 4.5 years (inter-quartile range 2.1–7.7 years). Initial HMR <1.55: HR mortality 9.36 (95% CI 4.27–20.56, P < 0.001)
Initial HMR 1.55–1.83: HR mortality 4.27 (95% CI 1.68–9.05, P = 0.002)

[I-123]-MIBG, Iodine-123 labelled metaiodobenzylguanidine; [C-11]-MQNB, carbon-11 labelled methylquinuclidinyl benzilate; Hattr, hereditary transthyretin-derived amyloid; wtATTR, wild-type transthyretin-derived amyloid; AL, immunoglobulin light chain-derived amyloid; FU, follow-up; HMR, heart-to-mediastinum ratio; HR, hazard ratio; LE, low energy; ME, medium energy; N/A, not available; p.i., post injection