Overview
Pulmonary veno-occlusive disease (PVOD) is a rare and serious lung condition that affects the small veins inside the lungs. It is sometimes called pulmonary capillary hemangiomatosis (PCH) when it also involves the tiny blood vessels called capillaries, and the two conditions are now often grouped together because they share many features. In PVOD, the small veins that carry blood away from the lungs become blocked or narrowed over time. This makes it very hard for blood to flow through the lungs normally, which causes pressure to build up in the lung blood vessels — a condition called pulmonary arterial hypertension (PAH). As the disease gets worse, the heart has to work much harder to pump blood through the lungs. Over time, this extra strain can weaken the right side of the heart. People with PVOD often feel very short of breath, especially during activity, and may feel extremely tired. They may also develop a persistent cough, sometimes coughing up blood, and their lips or fingertips may turn bluish (a sign called cyanosis) because not enough oxygen is getting into the blood. Treatment for PVOD is very challenging. Unlike other forms of pulmonary hypertension, the medications commonly used for PAH can actually make PVOD worse by causing fluid to build up in the lungs. The only known cure is a lung transplant. Supportive treatments like oxygen therapy and careful use of certain medications can help manage symptoms, but the disease tends to progress. Early diagnosis and referral to a specialized center are very important for the best possible outcomes.
Key symptoms:
Shortness of breath, especially during physical activityExtreme tiredness and low energyPersistent dry coughCoughing up bloodBluish color of the lips or fingertips (cyanosis)Swelling in the legs or anklesFainting or near-fainting, especially with exertionRapid or irregular heartbeatChest pain or pressureReduced ability to exercise or do daily activitiesCrackling sounds in the lungs heard by a doctorLow oxygen levels in the blood
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Pulmonary venoocclusive disease.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Pulmonary venoocclusive disease at this time.
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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 Pulmonary venoocclusive disease.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
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.Is my diagnosis confirmed as PVOD, and have other causes of pulmonary hypertension been ruled out?,Should I have genetic testing for the EIF2AK4 gene, and should my family members be tested too?,Am I a candidate for lung transplantation, and how do I get referred to a transplant center?,Which medications are safe for me to take, and which ones should I avoid?,What warning signs should make me go to the emergency room right away?,Are there any clinical trials I might be eligible for?,What support services or patient organizations can help me and my family cope with this diagnosis?
Common questions about Pulmonary venoocclusive disease
What is Pulmonary venoocclusive disease?
Pulmonary veno-occlusive disease (PVOD) is a rare and serious lung condition that affects the small veins inside the lungs. It is sometimes called pulmonary capillary hemangiomatosis (PCH) when it also involves the tiny blood vessels called capillaries, and the two conditions are now often grouped together because they share many features. In PVOD, the small veins that carry blood away from the lungs become blocked or narrowed over time. This makes it very hard for blood to flow through the lungs normally, which causes pressure to build up in the lung blood vessels — a condition called pulmona
Which specialists treat Pulmonary venoocclusive disease?
3 specialists and care centers treating Pulmonary venoocclusive disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.