| . | Infant-onset HCM <1 year (n = 184) . | Childhood-onset HCM 1–18 years (n = 1128) . | Adult-onset HCM >18 years (n = 6365) . |
|---|---|---|---|
| Age at diagnosis, years, mean (SD) | 0.2 (0.3) | 13.1 (4.3) | 48.0 (15.3) |
| Time from diagnosis to baseline SHaRe visit (years) | 2.0 | 2.1 | 2.6 |
| Male sex, n (%) | 104 (56.6) | 726 (64.4) | 3772 (59.3) |
| Genetic testing performed, n (%) | 103 (56.0) | 686 (60.8) | 4049 (63.6) |
| Sarcomeric, n (%)a | 37 (35.9) | 431 (62.8) | 1974 (48.8) |
| Non-sarcomeric, n (%)a | 54 (52.4) | 188 (27.4) | 1746 (43.1) |
| Proband, n (%) | 177 (96.2) | 960 (85.1) | 5568 (87.5) |
| Family history of HCM, n (%) | 48 (26.1) | 548 (48.6) | 2247 (35.3) |
| Max LV wall thickness, mm, mean (SD) | 8.5 (3.3) | 14.6 (7.0) | 17.1 (5.6)b |
| Max LV wall thickness, Z-score, mean (SD) | 11.8 (9.0) | 8.5 (7.2) | 7.9 (5.5) |
| Echo LVEF, % (SD) | 73 (15.2) | 72 (9.5) | 67 (8.0) |
| LVOT obstruction (gradient >30 mmHg), n (%) | 78 (42.4) | 293 (26.0) | 2441 (38.4) |
| Follow-up time (years), mean (SD) | 11.1 (11.8) | 12.3 (11.8) | 11.4 (10.4) |
| . | Infant-onset HCM <1 year (n = 184) . | Childhood-onset HCM 1–18 years (n = 1128) . | Adult-onset HCM >18 years (n = 6365) . |
|---|---|---|---|
| Age at diagnosis, years, mean (SD) | 0.2 (0.3) | 13.1 (4.3) | 48.0 (15.3) |
| Time from diagnosis to baseline SHaRe visit (years) | 2.0 | 2.1 | 2.6 |
| Male sex, n (%) | 104 (56.6) | 726 (64.4) | 3772 (59.3) |
| Genetic testing performed, n (%) | 103 (56.0) | 686 (60.8) | 4049 (63.6) |
| Sarcomeric, n (%)a | 37 (35.9) | 431 (62.8) | 1974 (48.8) |
| Non-sarcomeric, n (%)a | 54 (52.4) | 188 (27.4) | 1746 (43.1) |
| Proband, n (%) | 177 (96.2) | 960 (85.1) | 5568 (87.5) |
| Family history of HCM, n (%) | 48 (26.1) | 548 (48.6) | 2247 (35.3) |
| Max LV wall thickness, mm, mean (SD) | 8.5 (3.3) | 14.6 (7.0) | 17.1 (5.6)b |
| Max LV wall thickness, Z-score, mean (SD) | 11.8 (9.0) | 8.5 (7.2) | 7.9 (5.5) |
| Echo LVEF, % (SD) | 73 (15.2) | 72 (9.5) | 67 (8.0) |
| LVOT obstruction (gradient >30 mmHg), n (%) | 78 (42.4) | 293 (26.0) | 2441 (38.4) |
| Follow-up time (years), mean (SD) | 11.1 (11.8) | 12.3 (11.8) | 11.4 (10.4) |
HCM, hypertrophic cardiomyopathy; LV, left ventricular; LVEF, left ventricular ejection fraction; LVOT, left ventricular outflow tract; SD, standard deviation; SHaRe, Sarcomeric Human Cardiomyopathy Registry.
% reported is based on the % of patients with genetic testing. Patients with sarcomeric variants of unknown significance are not listed.
The normal range of LV wall thickness in adults is 6–10 mm.
| . | Infant-onset HCM <1 year (n = 184) . | Childhood-onset HCM 1–18 years (n = 1128) . | Adult-onset HCM >18 years (n = 6365) . |
|---|---|---|---|
| Age at diagnosis, years, mean (SD) | 0.2 (0.3) | 13.1 (4.3) | 48.0 (15.3) |
| Time from diagnosis to baseline SHaRe visit (years) | 2.0 | 2.1 | 2.6 |
| Male sex, n (%) | 104 (56.6) | 726 (64.4) | 3772 (59.3) |
| Genetic testing performed, n (%) | 103 (56.0) | 686 (60.8) | 4049 (63.6) |
| Sarcomeric, n (%)a | 37 (35.9) | 431 (62.8) | 1974 (48.8) |
| Non-sarcomeric, n (%)a | 54 (52.4) | 188 (27.4) | 1746 (43.1) |
| Proband, n (%) | 177 (96.2) | 960 (85.1) | 5568 (87.5) |
| Family history of HCM, n (%) | 48 (26.1) | 548 (48.6) | 2247 (35.3) |
| Max LV wall thickness, mm, mean (SD) | 8.5 (3.3) | 14.6 (7.0) | 17.1 (5.6)b |
| Max LV wall thickness, Z-score, mean (SD) | 11.8 (9.0) | 8.5 (7.2) | 7.9 (5.5) |
| Echo LVEF, % (SD) | 73 (15.2) | 72 (9.5) | 67 (8.0) |
| LVOT obstruction (gradient >30 mmHg), n (%) | 78 (42.4) | 293 (26.0) | 2441 (38.4) |
| Follow-up time (years), mean (SD) | 11.1 (11.8) | 12.3 (11.8) | 11.4 (10.4) |
| . | Infant-onset HCM <1 year (n = 184) . | Childhood-onset HCM 1–18 years (n = 1128) . | Adult-onset HCM >18 years (n = 6365) . |
|---|---|---|---|
| Age at diagnosis, years, mean (SD) | 0.2 (0.3) | 13.1 (4.3) | 48.0 (15.3) |
| Time from diagnosis to baseline SHaRe visit (years) | 2.0 | 2.1 | 2.6 |
| Male sex, n (%) | 104 (56.6) | 726 (64.4) | 3772 (59.3) |
| Genetic testing performed, n (%) | 103 (56.0) | 686 (60.8) | 4049 (63.6) |
| Sarcomeric, n (%)a | 37 (35.9) | 431 (62.8) | 1974 (48.8) |
| Non-sarcomeric, n (%)a | 54 (52.4) | 188 (27.4) | 1746 (43.1) |
| Proband, n (%) | 177 (96.2) | 960 (85.1) | 5568 (87.5) |
| Family history of HCM, n (%) | 48 (26.1) | 548 (48.6) | 2247 (35.3) |
| Max LV wall thickness, mm, mean (SD) | 8.5 (3.3) | 14.6 (7.0) | 17.1 (5.6)b |
| Max LV wall thickness, Z-score, mean (SD) | 11.8 (9.0) | 8.5 (7.2) | 7.9 (5.5) |
| Echo LVEF, % (SD) | 73 (15.2) | 72 (9.5) | 67 (8.0) |
| LVOT obstruction (gradient >30 mmHg), n (%) | 78 (42.4) | 293 (26.0) | 2441 (38.4) |
| Follow-up time (years), mean (SD) | 11.1 (11.8) | 12.3 (11.8) | 11.4 (10.4) |
HCM, hypertrophic cardiomyopathy; LV, left ventricular; LVEF, left ventricular ejection fraction; LVOT, left ventricular outflow tract; SD, standard deviation; SHaRe, Sarcomeric Human Cardiomyopathy Registry.
% reported is based on the % of patients with genetic testing. Patients with sarcomeric variants of unknown significance are not listed.
The normal range of LV wall thickness in adults is 6–10 mm.
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