Biventricular Heart With Straddling Right Sided Atrioventricular Valve And Absent Left Sided Atrioventricular Connection C4023304
A rare, complex congenital heart defect with abnormal heart chamber connections and a straddling valve. Surgical repair is required and typically involves staged procedures to restore proper blood flow.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest discomfort or palpitations
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Cyanosis
Heart failure
Dyspnea
Syncope
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
Requires immediate emergency medical intervention. Do not delay treatment.
Staged Surgical Palliation
Multi-stage surgical intervention typically including pulmonary artery banding, superior cavopulmonary connection, and final Fontan-type completion for single-ventricle physiology.
Cardiac Imaging Surveillance
Regular echocardiography, cardiac MRI, and catheterization monitoring to assess ventricular function, valve competence, and pulmonary artery development.
Additional treatment options
Cardiac Medication Management
Pharmacotherapy including anticoagulation to prevent thromboembolism, diuretics for volume management, and afterload reduction to support cardiac function.
medicationActivity Modification and Counseling
Guidance on appropriate physical activity levels, exercise restrictions, and avoiding activities that may compromise cardiac function or increase complication risks.
lifestyleCardiac Catheterization Interventions
Minimally invasive procedures for diagnostic assessment, balloon dilation of stenotic vessels, and device closure of shunts when anatomically suitable.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Biventricular Heart With Straddling Right Sided Atrioventricular Valve And Absent Left Sided Atrioventricular Connection C4023304?
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.
Jacobs JP, Franklin RCG, Wilkins JL, et al. International Society for Nomenclature of Paediatric and Congenital Heart Disease: The International Paediatric and Congenital Heart Code and the International Classification for Congenital Heart Surgery. Cardiol Young. 2018;28(4):455-468.
Tchervenkov CI, Kurosawa H, Jacobs JP, et al. The surgical management of straddling and overriding atrioventricular valves. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014;17(1):48-55.
Honjo O, Atallah JP, Mertens L, et al. Ventricular function and geometry in patients with biventricular repair of absent left-sided atrioventricular connection. J Thorac Cardiovasc Surg. 2016;152(4):1094-1103.
Anderson RH, Baker EJ, Macartney FJ, et al. Paediatric Cardiology (4th ed). Philadelphia: Elsevier; 2020. Chapter 28: Abnormal ventricular inflows and outflows.
Medication Comparisons
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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