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Short QT syndrome (SQT) is a very rare genetic disease of the electrical system of the heart, and is associated with an increased risk of abnormal heart rhythms and sudden cardiac death. [1] The syndrome gets its name from a characteristic feature seen on an electrocardiogram (ECG) – a shortening of the QT interval .
ST segment depression and T-wave changes may be seen in patients with unstable angina; Depressed but upsloping ST segment generally rules out ischemia as a cause. Also, it can be a normal variant or artifacts, such as: Pseudo-ST-depression, which is a wandering baseline due to poor skin contact of the electrode [3]
Long QT syndrome, the most common form of cardiac channelopathy, is characterized by prolonged ventricular repolarization, predisposing to a high risk of ventricular tachyarrhythmias (e.g., torsade de pointes), syncope, and sudden cardiac death.
Lown–Ganong–Levine syndrome (LGL) is a pre-excitation syndrome of the heart. Those with LGL syndrome have episodes of abnormal heart racing with a short PR interval and normal QRS complexes seen on their electrocardiogram when in a normal sinus rhythm .
Rare diseases called ion channelopathies may play a role such as long QT syndrome (LQTS), Brugada syndrome (BrS), CPVT (catecholaminergic polymorphic ventricular tachycardia), progressive cardiac conduction defect (PCCD), early repolarization syndrome, mixed sodium channel disease, and short QT syndrome. [13]
Pulmonary heart disease, also known as cor pulmonale, is the enlargement and failure of the right ventricle of the heart as a response to increased vascular resistance (such as from pulmonic stenosis) or high blood pressure in the lungs.
Jervell and Lange-Nielsen syndrome (JLNS) is a rare type of long QT syndrome associated with severe, bilateral sensorineural hearing loss. [2] Those with JLNS are at risk of abnormal heart rhythms called arrhythmias , which can lead to fainting , seizures , or sudden death.
Romano–Ward syndrome is a descriptive term for a group of subtypes of long QT syndrome, specifically subtypes LQT1-6 and LQT9-16. [8] Several subtypes of Romano–Ward syndrome have been described based on the underlying genetic variant. [5] These subtypes differ in clinical presentation and their response to treatment.
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