From the Guidelines
The management of Long QT syndrome focuses on preventing life-threatening arrhythmias through medication, lifestyle modifications, and in some cases, devices or surgery, with beta-blockers being the first-line medication therapy, as recommended by the 2015 ESC guidelines 1. The goal of treatment is to reduce the risk of sudden cardiac death and improve quality of life.
- Beta-blockers, such as nadolol, propranolol, or metoprolol, are the primary medication used to manage Long QT syndrome, as they block sympathetic stimulation of the heart and reduce the risk of dangerous heart rhythms 1.
- Patients should avoid QT-prolonging medications, such as certain antibiotics, antipsychotics, and antihistamines, and maintain normal electrolyte levels, particularly potassium, magnesium, and calcium, to prevent worsening of QT prolongation 1.
- For high-risk patients who have survived cardiac arrest or have recurrent syncope despite beta-blocker therapy, an implantable cardioverter-defibrillator (ICD) may be recommended, as it can prevent sudden cardiac death 1.
- Left cardiac sympathetic denervation, a surgical procedure that interrupts sympathetic nerves to the heart, can be considered for patients who cannot tolerate beta-blockers or have breakthrough events despite medication 1.
- All patients should avoid competitive sports, strenuous exercise, and sudden loud noises (particularly for LQT2), as these can trigger arrhythmias 1.
- Family screening is essential since Long QT syndrome is often genetic, and first-degree relatives should undergo ECG testing and possibly genetic testing if a pathogenic variant has been identified in the family 1.
From the Research
Management and Treatment of Long QT Syndrome
The management and treatment of Long QT syndrome (LQTS) is crucial to prevent sudden cardiac death, especially among youngsters. The available therapies are quite effective, and most patients with LQTS could have an essentially normal life if they are correctly managed 2.
Therapies for LQTS
Some of the therapies currently available for LQTS include:
- Beta-blockers: These are often used as the first line of treatment for LQTS, and have been shown to be effective in reducing the risk of sudden cardiac death 3, 4, 5.
- Left cardiac sympathetic denervation: This is a surgical procedure that involves removing or disrupting the nerves that regulate heart rate, and can be effective in reducing the risk of sudden cardiac death 2.
- Mexiletine: This is an anti-arrhythmic medication that can be used to treat LQTS, although its use is still experimental 2.
- Implantable cardioverter-defibrillator (ICD): This is a device that is implanted in the chest and can detect and correct life-threatening arrhythmias, and is often used in patients with LQTS who are at high risk of sudden cardiac death 3, 4, 5, 6.
Lifestyle Modifications
In addition to these therapies, lifestyle modifications can also play an important role in managing LQTS. This can include avoiding triggers for malignant cardiac arrhythmias, such as certain medications or activities, and making changes to diet and exercise habits 4, 5.
Risk Stratification
Risk stratification is also an important part of managing LQTS, and involves identifying patients who are at high risk of sudden cardiac death and providing them with more intensive treatment and monitoring 4, 5, 6.
Regular Reassessment
Regular reassessment of the arrhythmic risk of patients with LQTS is also essential, as the risk can decrease in response to therapy or increase for unknown reasons 2. This regular reassessment allows for yearly therapeutic optimization, which is key to patient survival.