What is the primary treatment for Short QT syndrome?

From the Guidelines

The primary treatment for Short QT syndrome is implantable cardioverter-defibrillator (ICD) placement, which is recommended for patients at high risk of sudden cardiac death, as stated in the 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1.

Key Considerations

• The decision to implant an ICD is based on the patient's risk of sudden cardiac death, with those having a history of cardiac arrest or sustained ventricular arrhythmias being at higher risk.
• Antiarrhythmic medications, such as quinidine, may be used in addition to or instead of ICD placement in certain cases, particularly for patients with recurrent sustained ventricular arrhythmias or those who cannot undergo ICD implantation.
• Quinidine has been shown to be effective in prolonging the QT interval and reducing the risk of ventricular arrhythmias in patients with Short QT syndrome, with a typical dosage ranging from 600-900 mg daily, divided into multiple doses, and with blood level monitoring recommended 1.

Additional Recommendations

• Genetic testing may be considered to facilitate screening of first-degree relatives, as Short QT syndrome is a genetic cardiac channelopathy that follows autosomal dominant inheritance 1.
• Lifestyle modifications, including avoiding QT-shortening drugs and maintaining electrolyte balance, particularly potassium levels, are also important for managing patients with Short QT syndrome.
• In patients with Short QT syndrome and VT/VF storm, isoproterenol infusion can be effective in restoring sinus rhythm, as noted in the 2017 AHA/ACC/HRS guideline 1.

From the Research

Short QT Syndrome Treatment

The primary treatment for Short QT syndrome includes:

• Implantable cardioverter defibrillator (ICD) as the first-line therapy 2, 3, 4, 5
• Quinidine as a pharmacological treatment, especially in young children who are not feasible for ICD implantation 6, 2, 4

Rationale Behind the Treatment

The use of ICD and quinidine is based on the following:

• ICD is effective in preventing sudden cardiac death in patients with Short QT syndrome 2, 3
• Quinidine has the ability to prolong the QT interval, which can help prevent ventricular arrhythmias 6, 2, 4
• Quinidine is particularly important for young children who are not suitable for ICD implantation 6, 4

Considerations and Complications

It is essential to consider the following:

• ICD therapy is associated with significant risk of device-related complications, such as inappropriate shocks, infection, and psychological distress 3
• Quinidine is the only drug that has undergone clinical testing for the treatment of Short QT syndrome 2