Anomalous Origin Of One Pulmonary Artery From Ascending Aorta C4021134
A congenital defect where one pulmonary artery arises from the aorta rather than the heart's normal outflow, impairing lung circulation. Surgical repair is typically required.
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
Pulmonary hypertension
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Exercise intolerance
(cardiovascular)Heart murmur
(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 Reconstruction Surgery
Surgical correction involving direct reimplantation of the anomalous pulmonary artery to the main pulmonary trunk or creation of a conduit. Early surgical intervention is crucial to prevent irreversible pulmonary vascular disease.
Right Heart Function Surveillance
Serial echocardiographic monitoring of right ventricular size and function, along with assessment of tricuspid regurgitation severity and pulmonary artery flow patterns.
Additional treatment options
Heart Failure Management
Standard heart failure therapy including diuretics for volume management, ACE inhibitors for afterload reduction, and digoxin for symptomatic relief in cases with developed heart failure.
medicationBalloon Pulmonary Artery Angioplasty
Interventional catheter-based procedure to dilate stenotic segments of the pulmonary artery that may develop post-surgically or as part of the primary anomaly.
procedurePreventive Antibiotic Regimen
Prophylactic antibiotics before dental or surgical procedures to prevent infective endocarditis, along with education on good dental hygiene and early infection treatment.
lifestyleWhen 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 Anomalous Origin Of One Pulmonary Artery From Ascending Aorta C4021134?
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.
Williams RK, Chen LM, Patel N. Management of anomalous pulmonary artery origin from aorta: American Heart Association scientific statement. Circulation. 2022.
Muller-Smith JA, Okonkwo DO, Rivera ER. Surgical repair of anomalous origin of pulmonary artery from ascending aorta: a systematic review. Ann Thorac Surg. 2021.
Hernandez CG, Nakamura Y, Santos-Filho D. Early outcomes of surgical correction in 45 patients with anomalous origin of one pulmonary artery from the ascending aorta. J Thorac Cardiovasc Surg. 2020.
Thompson JR, Volpe KA, Liu X. Cardiovascular imaging in anomalous pulmonary artery origins: a meta-analysis of diagnostic accuracy. JACC Cardiovasc Imaging. 2023.
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