Table 1.
Baseline characteristics of patients with Val122Ile ATTR-CA, by sex
| Women (n = 27) | Men (n = 46) | p value | |
|---|---|---|---|
| Clinical characteristics | |||
| Age at registry entry, y, mean (sd) | 76.4 (5.6) | 68.9 (7.77) | <0.0001 |
| Ethnicity, n (%) | |||
| Black | 26 (96.3) | 44 (95.6) | 0.89 |
| Other | 1 (3.7) | 2 (4.4) | |
| Systolic blood pressure, mmHg, mean (sd) | 115.6 (14.7) | 111.3 (18.1) | 0.13 |
| Diastolic blood pressure, mmHg, mean (sd) | 71.1 (10.6) | 70.1 (.1) | 0.77 |
| Mean arterial blood pressure, mean (sd) | 85.1 (10.8) | 83.0 (10.7) | 0.33 |
| Pulse pressure, mmHg, mean (sd) | 44.4 (11.2) | 41.2 (14.6) | 0.29 |
| Albumin, g/dL, mean (sd) | 3.9 (0.3) | 4.0 (0.4) | 0.38 |
| BMI, kg/m2, mean (sd) | 27.2 (6.2) | 26.2 (4.0) | 0.36 |
| Modified BMI, mean (sd) | 1,067 (248) | 1,058 (209) | 0.97 |
| BSA, m2, mean (sd) | 1.78 (0.22) | 1.99 (0.22) | <0.0001 |
| eGFR, mL/min/1.73m2, mean (sd) | 43.4 (12.6) | 46.9 (15.9) | 0.37 |
| NYHA class, n (%) | |||
| I | 0 (0) | 3 (6.5) | 0.20 |
| II | 9 (33.3) | 14 (30.4) | |
| III | 18 (66.7) | 25 (54.4) | |
| IV | 0 (0) | 4 (8.7) | |
| Year of diagnosis, n (%) | |||
| 2001–2006 | 0 (0) | 3 (6.5) | 0.0211 |
| 2007–2012 | 6 (22.2) | 22 (47.8) | |
| 2013–2018 | 21 (77.8) | 21 (45.6) | |
| Means of diagnosis, n (%) | |||
| EMB | 12 (44.4) | 32 (69.6) | 0.055 |
| Extra-cardiac biopsy | 0 (0) | 2 (4.4) | |
| 99mTc-PYP | 13 (48.2) | 9 (19.6) | |
| Imaging (MRI or TTE) | 2 (7.4) | 3 (6.5) | |
| History of carpal tunnel, n (%) | 13 (48.2) | 29 (63.0) | 0.021 |
| History of lumbar spinal stenosis, n (%) | 6 (22.2) | 9 (19.5) | 0.79 |
| Medications | |||
| Daily furosemide dose, mg, median [IQR]* | 40 [40–80] | 80 [40–100] | 0.022 |
| ACEi/ARB, n (%) | 14 (51.9) | 21 (45.7) | 0.81 |
| Beta-blocker, n (%) | 10 (37.0) | 24 (51.2) | 0.23 |
| Biomarkers† | |||
| BNP, pg/mL, median [IQR] | 740 [428–1092] | 816 [500–1652] | 0.60 |
| NT-proBNP, pg/mL, median [IQR] | 3,819 [2,227–9,406] | 2,098 [1,331–3,446] | 0.059 |
| Troponin T, ng/mL, median [IQR] | 0.06 [0.04–0.10] | 0.05 [0.01–0.11] | 0.45 |
| Troponin I, ng/mL, median [IQR] | 0.1 [0.05–0.17] | 0.2 [0.10–0.35] | 0.059 |
99mTc-PYP, Technetium-99m pyrophosphate cardiac scintigraphy; ACEi, angiotensin converting enzyme inhibitor; ARB, angiotensin receptor blocker; BMI, body mass index; BNP, brain natriuretic peptide; BSA, body surface area; dL, deciliter; eGFR, estimated glomerular filtration rate; EMB, endomyocardial biopsy; g, grams; IQR, interquartile range; min, minute; mL, milliliters; mmHg, millimeters of mercury; NT-proBNP, N-terminal-pro hormone BNP; NYHA, New York Heart Association; SD, standard deviation
Includes furosemide and equivalent diuretic dosages
For each biomarker, the number of patients with data available to calculate statistics are as follows: BNP = 41 patients, NT-proBNP = 45 patients, Troponin T = 38 patients, Troponin I = 54 patients