Atrioventricular Septal Defect And Common Atrioventricular Junction C0344783
Atrioventricular septal defect with common atrioventricular junction is a severe congenital heart defect where the walls between heart chambers do not form properly. This complex condition leads to heart failure symptoms in infancy and requires early surgical repair. Without treatment, it can cause significant health problems and shortened survival.
Key facts at a glance
- ICD-10
- Q21.0
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Dyspnea
Palpitations
Syncope
Heart block
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Complete Surgical Repair
Early surgical intervention involves patch closure of atrial and ventricular septal defects with valve repair or replacement as needed. This corrective procedure addresses the anatomical defects causing left-to-right shunting and valve insufficiency in AVSD.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Heart Failure Management
Pharmacologic management includes diuretics for volume overload, ACE inhibitors or ARBs for afterload reduction, and beta-blockers for rate control and remodeling. These medications support cardiac function while awaiting or following surgical correction.
Additional treatment options
Cardiac Surveillance Program
Lifelong echocardiographic monitoring and periodic cardiac MRI assess valve function, ventricular dimensions, and detect residual defects or arrhythmias. Regular follow-up intervals are tailored to individual risk profiles and surgical outcomes.
monitoringActivity Modification and Cardiac Rehabilitation
Structured exercise programs with appropriate activity restrictions promote cardiovascular fitness while preventing overexertion. Phase II cardiac rehabilitation provides supervised exercise training, education, and psychosocial support for optimal long-term outcomes.
lifestyleInterventional Catheter Closure
Select cases of residual or secondary VSDs may be amenable to percutaneous device closure, offering a less invasive alternative to repeat surgery. This approach requires careful patient selection and anatomic suitability assessment.
procedureWhen 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 Atrioventricular Septal Defect And Common Atrioventricular Junction C0344783?
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.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645.
Calkoen EE, Hazekamp MG, Blom NA, et al. Atrioventricular septal defect: from fetus to adult. Heart. 2016;102(16):1341-1349.
Ooi A, Koth A, Nakamura M, et al. Long-term outcomes of surgical repair for complete atrioventricular septal defect: a multicenter study. Cardiol Young. 2020;30(8):1123-1131.
Miller SG, Campbell MJ, Jones TK, et al. Left atrioventricular valve regurgitation after repair of atrioventricular septal defect: systematic review and meta-analysis. J Thorac Cardiovasc Surg. 2022;164(3):847-859.
Medication Comparisons
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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