Congenital long QT syndrome, LQTS

Introduction

Congenital long QT syndrome (LQTS) is a rare inherited heart rhythm disorder that affects the electrical activity of the heart. It is characterized by a prolonged QT interval on an electrocardiogram (ECG), which increases the risk of abnormal heart rhythms (arrhythmias) that can lead to fainting, seizures, or even sudden cardiac arrest. Early diagnosis and proper management are essential for reducing the risk of serious complications.

Causes

LQTS is caused by mutations in genes that control the flow of electrical signals in the heart. These genetic changes are usually inherited in an autosomal dominant or, less commonly, autosomal recessive pattern. Family history is a significant risk factor. Certain medications, electrolyte imbalances, or other medical conditions can also trigger or worsen symptoms in people with LQTS.

Symptoms

Symptoms of LQTS can vary. Some people have no symptoms, while others may experience fainting (often triggered by exercise, excitement, or sudden noises), palpitations, seizures, or sudden cardiac arrest. Symptoms often appear in childhood or adolescence but can occur at any age. In some cases, the first sign of LQTS may be a life-threatening arrhythmia.

Diagnosis

Diagnosis involves a combination of medical and family history, physical examination, and ECG to measure the QT interval. Additional tests may include exercise or stress testing, genetic testing, and evaluation of family members. If you or a family member experience unexplained fainting, seizures, or have a family history of sudden cardiac death, consult a doctor or cardiologist for evaluation.

Treatment Options

Treatment for LQTS aims to prevent dangerous arrhythmias and sudden cardiac events. Beta-blocker medications are commonly prescribed to help control heart rhythm. In some cases, an implantable cardioverter-defibrillator (ICD) may be recommended for those at high risk. Lifestyle changes, such as avoiding strenuous exercise, sudden loud noises, and certain medications, are also important.

Simple measures such as regular check-ups, informing healthcare providers about your condition, and family screening can help manage risk. If symptoms worsen or new issues arise, consult a doctor promptly.

Prevention

LQTS cannot be prevented, but early diagnosis, family screening, and appropriate management can significantly reduce the risk of complications. Genetic counseling can help families understand their risk and make informed decisions.

Takeaway

Congenital long QT syndrome is a serious but manageable heart rhythm disorder. With early recognition, regular monitoring, and proper treatment, many people with LQTS can lead active, healthy lives. If you have symptoms or a family history of LQTS, consult a doctor or cardiologist for assessment and support.

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