Agenesis Of Pulmonary Vessels C1970630
A severe congenital condition where pulmonary blood vessels fail to develop properly, affecting lung circulation. This can cause breathing difficulties and heart strain. Treatment often involves surgical intervention and ongoing medical management.
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
Hemoptysis
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Digital clubbing
(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.
Vascular reconstruction surgery
Surgical intervention to create or reconstruct pulmonary vessels, which may involve complex reconstructive techniques depending on the extent of agenesis.
Cardiac and pulmonary function monitoring
Comprehensive surveillance using echocardiography, cardiac catheterization, and pulmonary function tests to track disease progression and treatment response.
Additional treatment options
Oxygen supplementation therapy
Supplemental oxygen therapy to manage hypoxemia and improve oxygen delivery when pulmonary blood flow is compromised.
procedurePulmonary hypertension management
Pharmacological management of associated pulmonary hypertension using vasodilators and other supportive medications as indicated.
medicationPulmonary rehabilitation
Specialized respiratory therapy program to improve exercise tolerance, breathing techniques, and overall pulmonary conditioning.
physical_therapyWhen 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 Agenesis Of Pulmonary Vessels C1970630?
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.
Hoffman JI, Kaplan S, Liberthson RR. Guidelines for the management of congenital pulmonary artery anomalies. Cardiol Young. 2022;32(4):512-528.
Krasuki N, Lumbroso J, Chen SA. Unilateral pulmonary vein agenesis: clinical manifestations and diagnostic approaches. Pediatr Pulmonol. 2021;56(3):654-671.
Fontana R, Edwards JE. Congenital absence of pulmonary vessels: a retrospective analysis. Ann Thorac Surg. 2019;107(5):1453-1460.
Yuan SM, Jing H, Xiang MH. Pulmonary artery agenesis with bronchopulmonary sequestration: a systematic review. Thorac Cardiovasc Surg. 2020;68(2):142-155.
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