Overview
Idiopathic pulmonary artery dilatation (also called idiopathic dilatation of the pulmonary artery, or IDPA) is a rare condition where the main pulmonary artery — the large blood vessel that carries blood from the heart to the lungs — becomes abnormally widened or enlarged. The word 'idiopathic' means that no clear underlying cause, such as high blood pressure in the lungs or a heart defect, can be found to explain the enlargement. This sets it apart from pulmonary artery dilatation that happens as a result of other heart or lung diseases. Most people with this condition have no symptoms at all, and it is often discovered by accident during a chest X-ray or heart scan done for another reason. When symptoms do occur, they can include mild shortness of breath, a feeling of fluttering or pounding in the chest, or occasional chest discomfort. The heart and lung function usually remain normal or near-normal in most cases. Because the condition is generally benign (not harmful) in most people, treatment is often not needed. Instead, doctors recommend regular monitoring with imaging tests to make sure the artery is not growing larger over time. There are no specific FDA-approved drugs for this condition. Management focuses on ruling out other causes and watching for any changes that might suggest a complication is developing.
Key symptoms:
Shortness of breath, especially during physical activityFeeling of fluttering, pounding, or racing heartbeat (palpitations)Mild chest discomfort or pressureFatigue or feeling unusually tiredOccasional dizziness or lightheadednessA heart murmur heard by a doctor during examinationNo symptoms at all in many cases (found by accident on imaging)
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Idiopathic pulmonary artery dilatation.
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Specialists
View all specialists →No specialists are currently listed for Idiopathic pulmonary artery dilatation.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Idiopathic pulmonary artery dilatation.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How large is my pulmonary artery, and at what size would you consider it dangerous?,How often do I need imaging tests to monitor the artery, and what type of scan is best?,Are there any activities or exercises I should avoid?,What symptoms should prompt me to go to the emergency room immediately?,Have other causes of pulmonary artery enlargement, such as Marfan syndrome or pulmonary hypertension, been fully ruled out?,At what point would you recommend surgery, and what does that surgery involve?,Should my family members be screened for this condition?
Common questions about Idiopathic pulmonary artery dilatation
What is Idiopathic pulmonary artery dilatation?
Idiopathic pulmonary artery dilatation (also called idiopathic dilatation of the pulmonary artery, or IDPA) is a rare condition where the main pulmonary artery — the large blood vessel that carries blood from the heart to the lungs — becomes abnormally widened or enlarged. The word 'idiopathic' means that no clear underlying cause, such as high blood pressure in the lungs or a heart defect, can be found to explain the enlargement. This sets it apart from pulmonary artery dilatation that happens as a result of other heart or lung diseases. Most people with this condition have no symptoms at al
How is Idiopathic pulmonary artery dilatation inherited?
Idiopathic pulmonary artery dilatation follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.