Long Qt Syndrome 1 Recessive C4017089
A rare inherited heart rhythm condition where both gene copies are altered. This can cause more severe symptoms. Careful management and treatment are essential.
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
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpitations
(cardiovascular)Dizziness
(cardiovascular)Ventricular arrhythmia
(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 the cornerstone of Long QT Syndrome treatment, helping to reduce heart rate and prevent potentially life-threatening arrhythmias. They work by blocking the effects of adrenaline on the heart, decreasing the risk of cardiac events.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Implantable Cardioverter-Defibrillator (ICD)
For high-risk patients with recurrent syncope or cardiac arrest survivors, ICD implantation provides automatic detection and correction of dangerous arrhythmias. This device continuously monitors heart rhythm and delivers shocks when needed.
Additional treatment options
Avoidance of QT-prolonging triggers
Patients must strictly avoid medications that prolong the QT interval, electrolyte imbalances, and known environmental triggers. Education about safe medications and sudden temperature changes is essential for preventing events.
lifestyleContinuous cardiac monitoring
Regular Holter monitoring and event recorders help track QT interval changes and detect silent arrhythmias. Remote monitoring technology allows healthcare providers to review heart rhythms without clinic visits.
monitoringGenetic counseling
Genetic counseling helps patients and families understand inheritance patterns, family screening implications, and reproductive options. Cascade testing of family members is recommended to identify at-risk relatives.
complementaryWhen 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 Long Qt Syndrome 1 Recessive C4017089?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Long Qt Syndrome 1 Recessive C4017089. 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