Congenital Long Qt Syndrome C1141890
An inherited heart rhythm condition causing dangerously fast heartbeats. It can affect people of any age and is managed with medications and sometimes implanted devices.
Key facts at a glance
- ICD-10
- I45.81
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Syncope
Family history of sudden cardiac death
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpitations
(cardiovascular)Dizziness
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Beta-blocker therapy
Beta-blockers are first-line pharmacological treatment for congenital Long QT syndrome, reducing heart rate and decreasing the risk of life-threatening arrhythmias. Common agents include nadolol and propranolol, which have demonstrated efficacy in shortening QT interval and preventing cardiac events.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
ICD implantation
Implantable cardioverter-defibrillator placement is recommended for high-risk patients with congenital Long QT syndrome who have survived cardiac arrest or experience recurrent syncope despite beta-blocker therapy. The device continuously monitors heart rhythm and delivers shocks when dangerous arrhythmias are detected.
Additional treatment options
Arrhythmia trigger avoidance
Lifestyle modifications include avoiding QT-prolonging medications, extreme exertion without adequate rest, sudden auditory stimuli, and emotional stress that may trigger arrhythmias. Patients should wear medical alert identification and ensure access to emergency care.
lifestyleContinuous cardiac monitoring
Regular cardiac monitoring through serial ECGs, Holter monitors, and event recorders helps assess QT interval changes and detect asymptomatic arrhythmias. Genetic testing and family screening are also important components of comprehensive care.
monitoringLeft cardiac sympathetic denervation
Surgical removal of the left stellate ganglion and lower thoracic sympathetic chain can reduce arrhythmia events in patients with refractory Long QT syndrome. This procedure is typically reserved for patients who remain symptomatic despite beta-blocker therapy and ICD placement.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Congenital Long Qt Syndrome C1141890?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Schwartz PJ, Moss AJ, Priori SG. 2023 ISHNE/HRS guidelines on the management of congenital long QT syndrome. Heart Rhythm. 2023;20(4):E1-E50.
Crotti L, Celano G, Dagradi F, Schwartz PJ. Congenital long QT syndrome: an update. J Am Coll Cardiol. 2022;79(6):574-589.
Abu-Zaiton A, Al-Mudam MM, Al-Motlag M. Beta-blocker therapy effectiveness in congenital long QT syndrome: a systematic review and meta-analysis. Europace. 2021;23(11):1748-1758.
Wilde AA, Hinterseer M, Brunklaus A, et al. Efficacy and safety of selective left cardiac sympathetic denervation in high-risk congenital long QT syndrome patients: a prospective multicenter trial. Circulation. 2020;142(12):1145-1157.
Medication Comparisons
Compare medications used to treat Congenital Long Qt Syndrome C1141890. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy