Pulmonary Artery Agenesis: Causes, Symptoms, and Treatment
Written by Sumiran Racherla, MD. Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Pulmonary Artery Agenesis?
A pulmonologist, a lung specialist, usually manages this condition. Some patients also see a cardiologist. Children are often cared for by specialists in birth defects of the heart and lungs. A primary care doctor can coordinate referrals. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What it is
Pulmonary artery agenesis is a rare condition where one of the two pulmonary arteries never forms before birth. When one artery is missing, that lung gets less blood flow, and the other often carries more blood than usual. Some people never know they have it.
Symptoms
Symptoms vary a lot. Some people have none and learn about it by accident during a scan. Others may have shortness of breath, especially during exercise. Common signs include repeated lung infections, coughing, coughing up blood, and feeling very tired. Some develop pulmonary hypertension, or high blood pressure in the lung arteries.
Causes and who gets it
This condition happens in the womb when the blood vessel that should become a pulmonary artery fails to form. Most cases happen by chance, though some are linked to genetic changes or other heart birth defects.
How doctors diagnose it
Doctors often first spot the condition on an unusual chest X-ray. A CT scan with contrast dye is the main test used to confirm it. An echocardiogram can check the heart, and other tests measure blood flow and pressure in the lungs.
Treatment
Treatment depends on severity. People with mild symptoms may only need regular checkups. Medicines can help manage pulmonary hypertension, prevent infections, and control coughing or coughing up blood. Surgery is considered mainly when other heart defects are present.
When to see a doctor
See a doctor for ongoing shortness of breath, a cough that will not go away, or repeated chest infections. Coughing up blood always needs prompt attention. Sudden worsening of breathing or chest pain needs urgent care.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance; a concierge doctor usually charges more and often works alongside insurance. Either type can handle routine checkups, treat simple lung infections, and order basic imaging. When specialized care is needed, they refer patients to a pulmonologist or cardiologist.
Common questions
Can you live a normal life with one pulmonary artery?
Many people do, especially if the condition is mild and there are no other heart defects. Some people need regular monitoring and medicines. Outcomes differ from person to person. Regular follow-up with a doctor matters.
Is pulmonary artery agenesis hereditary?
Most cases happen by chance and are not passed down in families. Some cases are linked to genetic changes or occur with other conditions present at birth. A doctor may suggest genetic testing in certain situations.
How is it found if there are no symptoms?
It is sometimes discovered on a chest X-ray or CT scan done for another reason. The missing artery changes the normal look of the chest on imaging. A doctor can confirm the finding with more detailed scans.
Can the missing artery be replaced or fixed?
In most isolated cases, the missing artery cannot be recreated. Treatment focuses on managing symptoms and protecting the lungs. Surgery is considered mainly when other heart defects are present.
Sources
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
