Table 1.
Summary of diagnostic delay from symptom onset by study and ATTR-CM population
| Publication | Population, n | Diagnostic delay (years) | |
|---|---|---|---|
| Mean (SD) | Median (IQR) | ||
| ATTRwt-CM | |||
| Cipriani 2019 [34] | 18 | 2.3 (1) | – |
| Connors 2016 [20] | 121 | – | 0.8 |
| Falk 2016 [21] | 35 | 2.7 | – |
| Garcia-Pavia 2019 [38]b | 758 | 7.2 (0.5)c | 3.9 |
| González-López 2017 [35] | 108 | 1.3 (1.1)c | – |
| Grogan 2017 [22]b | 275 | 6.4 (7.4) | 3.8 |
| Khella 2018 [39]b | 349 | – | 3.6 |
| Ladefoged 2020 [36] | 50 | – | 1.1 (0.2, 2.1) |
| Lane 2019 [3] | – | – | 3.3 (0.7, 6.5) |
| Lousada 2018 [25]a | 70 | – (25% went undiagnosed > 4 years)d | – |
| Lousada 2019 [26]a | 91 | – (27% went undiagnosed > 4 years)d | – |
| Nakagawa 2016 [31] | 14 | 1.9 (2.7) | – |
| Ochi 2020 [32] | 39 | 2.1 (1.2)c | – |
| ATTRv-CM | |||
| Bishop 2018 [18]e | 67 (original cohort) | – | 3.7 (1.5, 7.0) |
| 25 (secondary cohort) | – | 3.4 (1.9, 7.8) | |
| Falk 2016 [21] | 35 | 1.8 | – |
| Grogan 2017 [22] | 146 | 6.5 (9.9) | 3 |
| He 2019 [30] | 23 | – | 2.5 (1.5, 3.8) |
| Khella 2018 [39]b | 176 | – | 2.1 |
| Lane 2019 [3] | – | – | 2.1 (0.3, 5.0) |
| Lousada 2018 [25]a | 114 | – (16% went undiagnosed > 4 years)d | – |
| Lousada 2019 [26]a | 123 | – (15% went undiagnosed > 4 years)d | – |
| Swiecicki 2015 [29] | 161 | – | 3.3 |
| Mixed (ATTRwt-CM and ATTRv-CM patients) | |||
| Bishop 2018 [18] | 52 | – | 2.9 (1.3, 6.0) |
| Chaudhary 2013 [19] | 12 | – | 1.2 |
| Kessler 2019 [23] | 68 | – (75% went undiagnosed > 0.5 years)d | – |
| López-Sainz 2020 [37] | 116 | 2.8 (4.3) | – |
| Papoutsidakis 2017 [27] | 17 | 6.5 | – |
| Papoutsidakis 2018 [28] | 34 | – | 2.5 |
“–” Not reported by studies
ATTRv-CM hereditary transthyretin amyloid cardiomyopathy, ATTRwt-CM wild-type transthyretin amyloid cardiomyopathy, IQR interquartile range, SD standard deviation
Publications belong to the same parent study: aAmyloidosis Research Consortium, the Amyloidosis Foundation and Amyloidosis Support Groups; bThe Transthyretin Amyloidosis Outcomes Survey
cDiagnostic delay was derived as the difference between reported age at symptom onset and reported age at diagnosis
dNo point estimate for diagnostic delay was presented
eBishop et al. reported two unique ATTRv-CM cohorts: an original cohort (n = 67) and a secondary cohort (n = 25)