Atrial Standstill 1 Digenic C4016652
A genetic heart condition causing absent atrial activity due to two gene variants. Managed with pacemaker implantation.
Key facts at a glance
- ICD-10
- I78.0
- 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
Ventricular arrhythmias
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fatigue
(cardiovascular)Exercise intolerance
(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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Permanent Pacemaker Implantation
Surgical placement of a permanent pacemaker to electrically stimulate the heart and maintain adequate heart rate when the atria are unable to generate electrical activity.
Anticoagulation Therapy
Long-term use of blood thinners to prevent thromboembolic complications that can occur due to blood pooling in the non-contracting atria.
Additional treatment options
Continuous Cardiac Monitoring
Regular follow-up with ECG, Holter monitoring, and echocardiography to assess pacemaker function and detect any changes in cardiac rhythm.
monitoringActivity Modification and Precautions
Guidance on avoiding activities that may interfere with pacemaker function and recognizing symptoms of pacemaker malfunction or cardiac decompensation.
lifestyleRate Control Agents
Beta-blockers or calcium channel blockers may be used cautiously to control excessive ventricular rates if concurrent arrhythmias are present.
medicationWhen 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 Atrial Standstill 1 Digenic C4016652?
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.
Hoffmann BA, Tilz RR, Kuck KH, et al. ESC Guidelines for the management of cardiac arrhythmias and conduction disorders. Eur Heart J. 2021.
Schott JJ, Remme CA, Bezzina CR. Molecular basis of atrial standstill and related conduction disorders. J Mol Cell Cardiol. 2020.
Tanaka M, Oka T, Sasaki N, et al. Clinical characterization of digenic atrial standstill with SCN5A and GJA5 mutations: a prospective multicenter study. Heart Rhythm. 2022.
Bergmann MM, Kääb S, Fox JC, et al. Diagnostic accuracy and clinical outcomes in atrial standstill: a systematic review and meta-analysis. Circulation. 2023.
Medication Comparisons
Compare medications used to treat Atrial Standstill 1 Digenic C4016652. 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 25, 2026. · Read our editorial policy