Congenital long QT syndrome can lead to –
**Core Concept**
Congenital long QT syndrome (LQTS) is a disorder of the heart's electrical system, characterized by a prolongation of the QT interval on the electrocardiogram (ECG). This condition increases the risk of potentially life-threatening arrhythmias, particularly torsades de pointes.
**Why the Correct Answer is Right**
The prolongation of the QT interval in LQTS is caused by mutations in genes encoding cardiac ion channels, such as KCNH2 (encoding Kv11.1 potassium channels) or SCN5A (encoding sodium channels). These mutations disrupt the normal functioning of the cardiac action potential, leading to a prolongation of the QT interval. This, in turn, increases the risk of torsades de pointes, a type of polymorphic ventricular tachycardia that can degenerate into ventricular fibrillation and sudden cardiac death.
**Why Each Wrong Option is Incorrect**
**Option A:** Tachyarrhythmias, such as atrial fibrillation or supraventricular tachycardia, are not directly related to the prolongation of the QT interval in congenital LQTS.
**Option B:** Hypokalemia can contribute to QT interval prolongation, but it is not a direct cause of congenital LQTS.
**Option C:** Ventricular pre-excitation, such as Wolff-Parkinson-White syndrome, is a different condition characterized by a pre-excitation of the ventricles, not a prolongation of the QT interval.
**Clinical Pearl / High-Yield Fact**
Congenital LQTS is often associated with a family history of sudden cardiac death, particularly in young females, and can be triggered by various factors, such as loud noises, stress, or certain medications. It is essential to recognize the ECG characteristics and clinical presentation of LQTS to prevent sudden cardiac death.
**Correct Answer:** D. Torsades de pointes.