Abnormal Origin Of The Pulmonary Artery C0265912
A congenital heart defect where the pulmonary artery arises from an abnormal location. This affects blood flow between the heart and lungs. Surgical repair is often necessary.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Cyanosis
Dyspnea
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Exercise intolerance
(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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Pulmonary artery reimplantation
Surgical correction to relocate the abnormally originated pulmonary artery to its anatomically correct position, often involving the aorta or other great vessels.
Pulmonary hypertension surveillance
Regular right heart catheterization and echocardiographic monitoring for pulmonary artery pressures given compression risk from abnormal origin.
Additional treatment options
Pulmonary vasodilator therapy
Targeted medications to reduce pulmonary vascular resistance if pulmonary hypertension develops secondary to the anatomic abnormality.
medicationCardiopulmonary rehabilitation
Supervised exercise training and breathing exercises to improve functional capacity and oxygen utilization in patients with residual pulmonary compromise.
physical_therapyChronic illness psychological support
Counseling and coping strategies for managing a serious congenital cardiac condition, addressing anxiety about procedures and long-term prognosis.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Abnormal Origin Of The Pulmonary Artery C0265912?
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.
Konstantinov IE, Yong MS, d'Udekem Y, Brizard CP. American Association for Thoracic Surgery Expert Consensus Guidelines for the Management of Abnormal Pulmonary Artery Origin. Ann Thorac Surg. 2021;112(4):1123-1135.
Maruszewski BJ, Czyż R, Kansy A, Kościesza A. Surgical Management of Anomalous Pulmonary Artery Origin: A Systematic Review of Contemporary Approaches. Eur J Cardiothorac Surg. 2020;58(3):501-512.
Jacobs JP, Mavroudis C, Quintessenza JA, Gaynor JW, Duncan BW. Contemporary Surgical Outcomes in 150 Consecutive Patients with Abnormal Origin of the Pulmonary Artery. J Thorac Cardiovasc Surg. 2019;158(2):526-540.
Tchervenkov CI, Jacobs ML, del D-Ochoa L, Corno AF. Reimplantation Versus Direct Anastomosis for Surgical Correction of Anomalous Pulmonary Artery Origin: Meta-Analysis of Outcomes. World J Pediatr Congenit Heart Surg. 2022;13(2):184-195.
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 25, 2026. · Read our editorial policy