Congenitally Uncorrected Transposition Of The Great Arteries With Cardiac Malformation C5679811
A critical heart defect where the two main arteries are switched, combined with another cardiac abnormality. Requires surgery to improve blood flow and oxygen delivery.
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.
Severe cyanosis
Respiratory distress
Failure to thrive
(cardiovascular)Cardiomegaly
(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
Arterial switch operation
Definitive surgical correction that detaches the aorta and pulmonary artery from their respective ventricles and reconnects them to the correct chambers, along with coronary artery transfer.
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Prostaglandin E1 infusion
Continuous infusion to maintain ductal patency in newborns before definitive surgery, ensuring adequate mixing of oxygenated and deoxygenated blood.
Additional treatment options
Cardiac catheterization with balloon atrial septostomy
Catheter-based procedure to enlarge the atrial communication, improving oxygen saturation by allowing better mixing of blood between atria in critically ill newborns.
monitoringPalliative pulmonary artery banding
Temporary banding of the pulmonary artery to restrict pulmonary blood flow, used when definitive repair must be delayed or as part of staged reconstruction.
surgicalContinuous oxygen saturation monitoring
Ongoing monitoring of oxygen levels to detect hypoxemia and guide intervention timing, particularly important in pre-operative period and early post-operative recovery.
monitoringWhen 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 Congenitally Uncorrected Transposition Of The Great Arteries With Cardiac Malformation C5679811?
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.
Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease. J Am Coll Cardiol. 2008.
Villafañe J, Lantin-Hermoso MR, Bhatt AB, et al. D-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation. J Am Coll Cardiol. 2014.
Brawn WJ, Barron DJ, Jones TJJ, et al. The fate of the arterial switch operation in adults with transposition of the great arteries. Eur J Cardiothorac Surg. 2020.
Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography. J Am Soc Echocardiogr. 2016.
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