Table 1.
Overall | PKP2 Negative | PKP2 Positive | |
---|---|---|---|
Patients Diagnosed with CPVT (N) | 18 | 13 | 5 |
Males/Females | 9/9 | 7/6 | 2/3 |
Average Age (years) | 19.6 ± 12.8 | 19.7 ± 14.7 | 19.4 ± 6.3 |
Age Range (years) | 6 to 58 | 6 to 58 | 12 to 28 |
Average QTc (ms) | 417 ± 26 | 416 ± 23.4 | 419 ± 35 |
Ventricular Ectopy | 13 (72%) | 8 (61.5%) | 5 (100%) |
Symptomatic | 15 (83%) | 10 (77%) | 5 (100%) |
Syncope | 13 (72%) | 10 (77%) | 3 (60%) |
Cardiac Arrest | 2 (11%) | 0 (0%) | 2 (40%) |
Exertional Trigger | 13 (72%) | 8 (61.5%) | 5 (100%) |
Positive Family History of Cardiac Events (syncope, seizures, cardiac arrest, etc.) | 5 (28%) | 4 (31%) | 1 (20%) |
Family History of SCD in the Young (< 40 years of age) | 2 (11%) | 1 (8%) | 1 (20%) |
Cases of Exercise-Associated SUDY (N) | 19 | 18 | 1 |
Males/Females | 13/6 | 12/6 | 1/0 |
Average Age (years) | 13.9 ± 3.2 | 13.8 ± 3.2 | 16 |
Age Range (years) | 6 to 19 | 6 to 19 | 16 |
Exertion Related Death | 19 (100%) | 18 (100%) | 1 (100%) |
Previous Personal History of Cardiac Event | 4 (21%) | 4 (22%) | 0 (0%) |
Positive Family History of Cardiac Events (syncope, seizures, cardiac arrest, etc.) | 4 (21%) | 4 (22%) | 0 (0%) |
Family History of SCD in the Young (< 40 years of age) | 1 (5%) | 1 (5.5%) | 0 (0%) |
SUDY= sudden death in the young; CPVT = catecholaminergic polymorphic ventricular tachycardia; SCD=sudden cardiac death