Table II.
Clinical findings and outcome of DPD scintigraphy.
| Type A (n = 31) | Type B (n = 21) | p value | |
|---|---|---|---|
| Age at examination median (range), years | 71 (41–83) | 57 (30–75) | <0.01 |
| Sex male/female, n | 18/13 | 15/6 | 0.15 |
| V30M, n | 26 (83.9%) | 21 (100%) | |
| 99mTc-DPD-positive, n | 30 (96.8%) | 0 (0%) | <0.01 |
| Grade 0, n | 1 | 21 | |
| Grade 1, n | 0 | 0 | |
| Grade 2, n | 11 | 0 | |
| Grade 3, n | 19 | 0 | |
| Positive cardiac biopsy, n (total performed) | 2 (2) | 1 (1) | |
| Previous liver transplant, n | 5 (16.1%) | 8 (38.1%) | <0.01 |
| NT-proBNP median (range), ng/La | 1240 (37–9829) | 67 (10–711) | <0.01 |
| Hs-troponin T median (range), ng/La | 33 (10–95) | 10 (7–31) | <0.01 |
| CMR increased LV mass, n/total | 6/6 | 0/5 | <0.01 |
| CMR LGE, n/total | 3/6 | 1/5 | 0.35 |
| IVSD median (range), mm | 17 (12–25) | 11 (8–15) | <0.01 |
| IVSD >12 mm, n (%) | 29 (82.9%) | 6 (17.1%) | <0.01 |
| Left atrial diameter (range), mm | 42 (24–51) | 36 (27–52) | 0.02 |
| LV mass (range), g | 307 (136–552) | 175 (117–444) | <0.01 |
| LVEF (range), % | 50 (30–70) | 58 (51–68) | 0.01 |
| E/é median (range) | 14.0 (5.8–24.7) | 7.0 (5.1–16.3) | <0.01 |
| Normal ECG, n (%) | 5 (16.1%) | 13 (61.9%) | <0.01 |
| Pacemaker, n (%)b | 5 (16.1%) | 3 (14.3%) | 1.00 |
| Age of onset median (range), years | 65 (38-82) | 49 (26-75) | <0.01 |
| Primary symptomsc | 0.19 | ||
| Neuropathy, n (%) | 22 (71.0%) | 17 (81%) | |
| Cardiomyopathy, n (%) | 6 (19.4%) | 1 (4.8%) | |
| Other, n (%) | 3 (9.7%) | 3 (14.3%) | |
| Hypertension, n (%) | 16 (65.2%) | 6 (21.6%) | 0.15 |
Type A amyloid fibrils are composed of full-length and truncated transthyretin, type B of full-length transthyretin only.
Reference values: Left atrial diameter, 27–40 mm; Hs-troponin T, <14.51 ng/L; NT-proBNP, <150 ng/L if age <75 years, <450 ng/L if age >75 years.
Two patients were excluded from analysis due to advanced kidney disease.
Pacemaker: presence of cardiac pacemaker at the time of 99mTc-DPD scintigraphy.
Primary symptoms: patient reported symptoms leading up to diagnosis. Other symptoms included gastrointestinal tract disturbances, renal failure, and cranial nerve affection.
CMR = cardiovascular magnetic resonance; E/é = mitral E-wave velocity/lateral wall é-wave velocity; IVSD = end-diastolic interventricular septum thickness (normal reference 6–10 mm); LGE = late gadolinium enhancement; LV mass = left ventricular mass; LVEF = left ventricular ejection fraction.