Congenitally Uncorrected Transposition Of The Great Arteries With Coarctation C5680321
A severe heart defect with switched arteries and narrowing of the aorta. Requires urgent surgical repair to restore proper blood flow to the body.
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
Heart failure
Failure to thrive
(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
Combined coarctation repair with arterial switch
Single-stage surgical correction addressing both the coarctation of the aorta and the transposed great arteries, performing aortic reconstruction and anatomic repair in one procedure.
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Staged repair with initial coarctation angioplasty
Initial catheter-based or surgical relief of coarctation followed by delayed arterial switch operation, allowing stabilization of the neonate before definitive repair.
Additional treatment options
Prostaglandin E1 maintenance
Continued infusion to keep the ductus arteriosus patent, which provides critical collateral flow around the coarctation site and improves systemic perfusion.
medicationHemodynamic monitoring with blood pressure differentials
Intensive monitoring of upper and lower extremity blood pressures, echocardiography, and cardiac output to assess repair adequacy and detect re-coarctation.
monitoringBalloon angioplasty for re-coarctation
Catheter-based dilation of recurrent or residual coarctation using high-pressure balloons, often performed with stent placement in older children and adults.
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 Congenitally Uncorrected Transposition Of The Great Arteries With Coarctation C5680321?
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(8):857-918.
Villafañe J, Lantin-Hermoso MR, Bhatt AB, et al. D-Transposition of the great arteries: management of the newborn and review of outcomes. Prog Pediatr Cardiol. 2019;53:12-20.
Qureshi SA, Bonhoeffer P, Geva T, et al. Long-term outcomes of arterial switch operation in transposition of the great arteries with coarctation: a multicenter prospective study. J Am Coll Cardiol. 2022;79(6):543-555.
Wernovsky G, Anderson RH, Tlaskalová-Hogenová H, et al. Transposition of the great arteries. In: Anderson's Pediatric Cardiology. 4th ed. Elsevier; 2020:891-934.
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