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The condition presents early in life and the average life expectancy is 2.5 years with death most commonly caused by ventricular arrhythmias. Many children with Timothy syndrome who survive longer than this have features of autism spectrum disorder. Timothy syndrome is caused by variants in the calcium channel Cav1.2 encoded by the gene CACNA1c.
The prognosis for TIC after treatment of the underlying tachyarrhythmia is generally good. Studies show that left ventricular function often improves within 1 month of treatment of the tachyarrhythmia, and normalization of the left ventricular ejection fraction occurs in the majority of patients by 3 to 4 months.
Junctional ectopic tachycardia (JET) is a rare syndrome of the heart that manifests in patients recovering from heart surgery. [1] It is characterized by cardiac arrhythmia, or irregular beating of the heart, caused by abnormal conduction from or through the atrioventricular node (AV node).
An automatic tachycardia is a cardiac arrhythmia which involves an area of the heart generating an abnormally fast rhythm, sometimes also called enhanced automaticity.These tachycardias, or fast heart rhythms, differ from reentrant tachycardias (AVRT and AVNRT) in which there is an abnormal electrical pathway which gives rise to the pathology.
Permanent junctional reciprocating tachycardia (PJRT) is a rare cardiac arrhythmia. It is a supraventricular tachycardia, and a cause of atrioventricular reentrant tachycardia (AVRT). PJRT can cause chronic tachycardia that, untreated, leads to cardiomyopathy.
The 2005 Joint European Resuscitation and Resuscitation Council (UK) guidelines [17] state that atropine is the first-line treatment especially if there were any adverse signs, namely: 1) heart rate < 40 bpm, 2) systolic blood pressure < 100 mm Hg, 3) signs of heart failure, and 4) ventricular arrhythmias requiring suppression. If these fail to ...
Ventricular arrhythmias are usually exercise-related, suggesting that they are sensitive to catecholamines. The ventricular beats typically have a right axis deviation. Multiple morphologies of ventricular tachycardia may be present in the same individual, suggesting multiple arrhythmogenic foci or pathways.
In those with short QT syndrome who have already experienced a life-threatening abnormal heart rhythm such as ventricular fibrillation, an implantable cardioverter-defibrillator (ICD) may be recommended to reduce the chance of sudden death. [3] This device is implanted under the skin and can continually monitor the heart rhythm.
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