Pulmonary Venoocclusive Disease 1 C3887658
A rare lung condition where small veins become blocked, raising blood pressure in the lungs. It affects both children and adults, making breathing difficult. Treatment options are limited.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest discomfort or palpitations
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Hemoptysis
Progressive exertional dyspnea
(cardiovascular)Nonproductive cough
(cardiovascular)Fatigue
(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
Requires immediate emergency medical intervention. Do not delay treatment.
Pulmonary vasodilator therapy
Calcium channel blockers or other pulmonary vasodilators may be trialed cautiously, as patients with PVOD can develop pulmonary edema. Careful titration and close monitoring are essential.
Lung transplantation
Definitive treatment for eligible patients with end-stage PVOD who have failed medical management. Requires comprehensive pre-transplant evaluation and lifelong immunosuppression.
Additional treatment options
Anticoagulation therapy
Chronic anticoagulation with warfarin or DOACs may be considered to prevent in-situ thrombosis, which is common in PVOD. Requires regular INR monitoring.
procedureSupplemental oxygen therapy
Long-term oxygen supplementation to maintain adequate oxygen saturation, particularly during exertion and sleep. Helps reduce pulmonary vascular resistance.
lifestyleRegular right heart catheterization
Serial hemodynamic assessments to track disease progression and response to therapy. Essential for treatment decisions and transplant listing.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Pulmonary Venoocclusive Disease 1 C3887658?
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.
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016.
Montaillier M, Toper C, Weatherald J, Jutant EM. Pulmonary veno-occlusive disease: an updated review. Respir Med. 2022.
K不用了Montani D, Price LC, Dorfmüller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2019.
Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2018.
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