Supplementary Table 1 Number of MIBG scans performed per patient.

No. of scans per patient No. of patients Percentage

1 101 43.5

2 58 25.0

3 22 9.5

4 15 6.5

5 20 8.6

6 3 1.3

7 8 3.4

8 4 1.7

9 1 0.4

Supplementary Table 2 Changes in heart-to-mediastinum ratio by age and duration of symptoms.

Variable

Age Duration of symptoms

Pearson’s correlation Spearman’s correlation Pearson’s correlation Spearman’s correlation

R R2 p Rho p R R2 p Rho p

Early H/M -0.308 0.095 <0.001 -0.258 <0.001 -0.201 0.040 <0.001 -0.225 <0.001

Late H/M -0.287 0.082 <0.001 -0.247 <0.001 -0.257 0.066 <0.001 -0.275 <0.001

Washout rate 0.097 0.009 0.023 0.073 NS (0.086) 0.153 0.024 0.002 0.162 0.001

H/M: heart-to-mediastinum ratio.

Supplementary Table 3 Causes of death.

Cause of death Total (n=47) Non-transplanted patients (n=30)

Transplanted patients (n=17)

Terminal stage of disease with cachexia and multiple organ failure 22 (47%) 19 (63%) 3 (18%)

Infectious complications 9 (19%) 7 (23%) 2 (12%)

Stroke or intracranial bleeding 3 (6%) 3 (10%) 0

Perioperative complications of liver transplantation 6 (13%) -- 6 (35%)

Acute transplant rejection 3 (6%) -- 3 (18%)

Chronic transplant rejection 3 (6%) -- 3 (18%)

Other causes unrelated to PAF 1 (2%) 1 (3%) 0

PAF: familial amyloid polyneuropathy.

Supplementary Figure 1 (A) changes in late H/M ratio by age in both sexes; (B) long-term survival by

quartiles of late H/M ratio in both sexes. H/M: heart-to-mediastinum.