Title : Predictors of sudden death in congenital arrhythmogenic syndromes
Abstract:
Congenital arrhythmia syndromes are rare genetic disorders that can cause a high risk of sudden cardiac death in young, apparently healthy people. These conditions can be treated, and deaths can be prevented. Each child of an affected parent has a 50% chance of inheriting the condition. It is estimated that over half of the 4,000 SADS deaths each year of children, teens, or young adults have one of the top two warning signs: 1) family history of unexplained death of a family member, or 2) fainting.
Predictors of sudden death in early repolarization syndrome: Family history of SD; History of syncope J point elevation with ST elevation with upward slope - QRS fragmentation or slurring.
Predictors of SD in Brugada Syndrome Risk stratification: Manifest type one at rest without provocation; Fractionated R wave: Association with early repolarization, Inducible VF during EP study Mutations in SCN5A - family history of SD - history of syncope or resuscitated cardiac arrest.
Predictors of SD in Arrhythmogenic RV Cardiomyopathy: History of syncope or aborted SD. Magnetic resonance imaging showing RV outflow thinning, hypokinesia, and fatty infiltration. Family history of SD.
Predictors of sudden death in long QT syndrome: The longer the QT, the higher the risk (>500 msec.). T wave bifid, biphasic, history of syncope or Torsade de Point, resuscitated cardiac arrest. Family history of sudden death.
Wolff-Parkinson-White (WPW): Syncope, History of VT or SD, History or occurrence of atrial fibrillation, Family history of SD.
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT): Bidirectional ventricular tachycardia, history of syncope, resuscitated SD. Family history of SD.
Risk factors for sudden death in HOCM: Major SCD Risk Factors, Family history of sudden cardiac death-Unexplained syncope, severe left ventricular hypertrophy, and non-sustained ventricular tachycardia- Abnormal blood pressure response to exercise.
