Interrupted Aortic Arch Type B C0345093
A critical heart defect where the aorta is completely separated, blocking normal blood circulation. Affected newborns need urgent surgical intervention. Outcomes depend on associated heart abnormalities and timely treatment.
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
Tachypnea
(cardiovascular)Feeding difficulty
(cardiovascular)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
Aortic Arch Repair with VSD Closure
Surgical repair involving arch reconstruction often through left thoracotomy approach, with definitive repair of associated ventricular septal defect. Procedure may be staged depending on patient stability and anatomy.
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Prostaglandin E1 Therapy
Continuous alprostadil infusion to maintain ductal patency ensuring adequate systemic perfusion. Essential bridge therapy until surgical correction can be safely performed.
Additional treatment options
Echocardiographic Assessment
Comprehensive transthoracic and transesophageal echocardiography to define arch anatomy, locate interruption site relative to subclavian arteries, and assess associated defects. Essential for surgical planning.
procedurePreoperative Stabilization and Monitoring
Aggressive management in NICU setting with attention to metabolic acidosis prevention, adequate oxygen delivery, and organ perfusion preservation. Mechanical ventilation often required.
monitoringFamily Education and Support
Comprehensive parental education regarding congenital heart defect, surgical intervention expectations, and long-term outcomes. Psychosocial support to address anxiety and facilitate informed decision-making.
psychologicalWhen 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 B C0345093?
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.
Stayer SA, Friesen RH, Vener DF. Guidelines for the perioperative management of patients with congenital heart disease. J Cardiothorac Vasc Anesth. 2022.
Kantor JL, Lodge AJ, Kelleher MD. Surgical repair of interrupted aortic arch: a comprehensive review. Ann Thorac Surg. 2021.
Huang MH, Chen CC, Yang KY. Long-term outcomes of single-stage repair for interrupted aortic arch. J Thorac Cardiovasc Surg. 2020.
Nakamura R, Williams WG, Barker MN. Neonatal cardiac surgery outcomes: a meta-analysis of interrupted aortic arch repair techniques. Cardiol Young. 2023.
Medication Comparisons
Compare medications used to treat Interrupted Aortic Arch Type B C0345093. 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