The Long QT Syndrome长QT综合症.pptVIP

The Long QT Syndrome Overview and Management Edited by A.Kharazi M.D Cardiac electrophysiologist LQTS Outline Background Identification Therapies Available Current Management Ongoing Research Case Studies Conclusions Long QT Syndrome Background and the Risk of Sudden Cardiac Death Sudden Cardiac Death (SCD) Affects 350,000 - 400,000 each year in the US alone Only 5% of victims survive Causes of SCD may include structural heart disease or a genetic channelopathy Recognition of risk factors can help identify those at risk of SCD Risk Factors for SCD in Young People Structural congenital heart disease - before and after corrective surgery Congenital anomalies of coronaries Myocarditis Hypertrophic and other cardiomyopathies Wolff-Parkinson-White Syndrome Long QT Syndrome LQTS: Historical Aspects 1957: 1st LQTS family reported 1963-1964: Romano-Ward Syndrome 1958-1970: 25 LQTS cases reported 1971: 1st LQTS Rx (left stellate ganglionectomy) 1979: LQTS Registry Started 1991-2001: 6 LQTS genes identified Long QT Syndrome Genetic disorder (1:5,000-10,000) ECG evidence: QTc interval prolonged 440 ms in males 450 ms in females Hallmark arrhythmia: Torsade de pointes VT Primary presenting symptom: Syncope SCD in children or young adults LQTS: Identification LQTS: Identification of Risk Most common presenting symptom: unexplained syncope. Syncope on exertion in pediatric patients should be considered malignant until proven otherwise. History ECG: Onset and offset of syncopal episode Siblings, or family members with unexplained syncope or sudden death Family history of “seizures” or congenital deafness Prolonged QTc on ECG Syncope Causes of Arrhythmic Syncope Very rapid VT or TdP, with hypotension Atrial fibrillation or atrial flutter with very rapid ventricular response as in WPW AV block Sinus arrest LQTS: Clinical Features Symptoms Syncope Seizures Sudden death Palpitations or “chest pain” ECG Signs Prolonged QTc Torsade de pointes LQTS ECG Patterns