Romano–Ward syndrome

Romano-Ward syndrome is a condition that causes a disruption of the heart's normal rhythm (arrhythmia). This condition is a form of long QT syndrome, which is a disorder that can cause potentially fatal irregular heartbeats (arrhythmias). In people with Romano-Ward syndrome, these arrhythmias occur without any other signs or symptoms.

Signs and Symptoms

People with Romano-Ward syndrome typically experience symptoms related to their irregular heartbeats. These symptoms can include fainting (syncope), seizures, and sudden death. These symptoms are often triggered by exercise or stress.

Causes

Romano-Ward syndrome is caused by mutations in one of several genes. These genes provide instructions for making proteins that form channels across cell membranes. These channels transport positively charged atoms (ions) in and out of cells. In heart muscle, these ion channels play critical roles in maintaining the heart's normal rhythm.

Diagnosis

Romano-Ward syndrome is diagnosed based on the results of an electrocardiogram (ECG), which measures the electrical activity of the heart. Other tests, such as exercise stress testing and genetic testing, may also be used to confirm the diagnosis.

Treatment

Treatment for Romano-Ward syndrome typically involves medications to control the heart's rhythm. In some cases, an implantable cardioverter-defibrillator (ICD) may be used to monitor the heart's rhythm and deliver electrical shocks to restore a normal rhythm if necessary.

Prognosis

With appropriate treatment, the prognosis for individuals with Romano-Ward syndrome is generally good. However, without treatment, the condition can be life-threatening.

See Also

• Long QT syndrome
• Arrhythmia
• Electrocardiogram
• Genetic testing

References

• National Institutes of Health
• American Heart Association

Romano–Ward syndrome Resources
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