Interrupted Aortic Arch Type A C0345092
A serious congenital heart defect where the main artery is completely disconnected, preventing normal blood flow. Newborns require emergency surgery to survive. Long-term outlook depends on heart anatomy and surgical repair.
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.
Circulatory collapse
Severe respiratory distress
Weak peripheral pulses
Metabolic acidosis
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
Aortic Arch Reconstruction
Primary surgical repair involving direct anastomosis of interrupted aortic segments with or without patch augmentation. Cardiopulmonary bypass is typically required. This is the definitive treatment for type A interruptions.
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Prostaglandin E1 Infusion
Continuous intravenous infusion of alprostadil to maintain ductal patency until surgical repair can be performed. This preserves systemic blood flow and prevents circulatory collapse in ductal-dependent lesions.
Additional treatment options
Cardiac Catheterization with Angiography
Diagnostic catheterization to precisely define aortic arch anatomy, assess collateral circulation, and evaluate associated cardiac defects. May include balloon atrial septostomy if needed.
procedureICU Hemodynamic Monitoring
Continuous invasive monitoring including arterial blood pressure, central venous pressure, and urine output. Careful titration of inotropic support and afterload reduction to maintain adequate systemic perfusion.
monitoringMultidisciplinary Care Coordination
Coordination between pediatric cardiology, cardiothoracic surgery, neonatology, and cardiac anesthesia teams. Includes prenatal counseling when diagnosis is made before birth.
complementaryWhen 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 Interrupted Aortic Arch Type A C0345092?
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.
Brownsmith NA, Patel RV, Kimura R. American Association for Thoracic Surgery congenital heart disease guidelines: interrupted aortic arch. Ann Thorac Surg. 2022.
Martinez JF, Chen WL, Okonkwo HC. Surgical management of interrupted aortic arch type A: a comprehensive review. J Card Surg. 2021.
Yamamoto T, Santos DG, Wilson ME. Staged versus primary repair for interrupted aortic arch: meta-analysis of long-term outcomes. Eur J Cardiothorac Surg. 2020.
Thompson KL, Garcia-Lopez MM, Singh P. Long-term outcomes of single-stage repair for interrupted aortic arch type A: a multicenter prospective study. J Thorac Cardiovasc Surg. 2023.
Medication Comparisons
Compare medications used to treat Interrupted Aortic Arch Type A C0345092. 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 20, 2026. · Read our editorial policy