Table of Cardiac Ion Channelopathies.
Long QT syndrome (LQTS) 17 26 27 28 75 | Short QT syndrome (SQTS) 17 18 29 | Catecholaminergic polymorphic ventricular tachycardia (CPVT) 5 17 30 31 | Brugada syndrome (BrS) 17 32 33 34 35 | |
---|---|---|---|---|
Prevalence | 1:5,000 to 1:2,000 | Rare - fewer than 30 cases published | 1:7,000 to 1:10,000 | 1:800 (Japan); 1:6,000 (U.S. & Europe) type 1 ECG pattern |
Annual mortality rate | 0.3% (LQT1) 0.6% (LQT2) 0.56% (LQT3) |
Unidentified | 3.1% | 4% (pts. with type 1 ECG pattern) |
Mean age of first event | 14 ± 12 yrs. | 40 ± 24 yrs. | 15 ± 10 yrs. | 42 ± 16 yrs. (pts. with type 1 ECG) |
Diagnostic Yield - Genetic Testing | 75-80% | 15-20% | 65-75% | 11-40% (see Clinical Validity) |