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pulmonary venoocclusive disease 2
Description
Pulmonary Veno-Occlusive Disease (PVOD): A Rare and Complex Condition
Pulmonary veno-occlusive disease (PVOD) is a rare and complex condition that affects the lungs. It is characterized by the blockage of the pulmonary veins, which carry oxygen-rich blood from the lungs to the heart.
Key Features:
- PVOD is a rare form of pulmonary hypertension, accounting for 5-20% of cases classified as idiopathic pulmonary arterial hypertension (PAH) [1].
- The condition leads to high blood pressure in the lung arteries (pulmonary hypertension) [3].
- PVOD is often mistaken with pulmonary arterial hypertension (PAH), but it is considered a distinct class I′ of PAH [5].
Symptoms and Characteristics:
- PVOD is characterized by the blockage (occlusion) of the blood vessels that carry oxygen-rich blood from the lungs to the heart (the pulmonary veins) [6].
- Symptoms include fatigue, breathlessness, and symptoms of right heart failure [9].
- The condition can lead to disastrous outcomes (severe pulmonary edema) in patients with PVOD when treated with certain medications, such as prostacyclin 6 [7].
Importance of Accurate Diagnosis:
- Accurate diagnosis is crucial to distinguish PVOD from PAH and other forms of pulmonary hypertension.
- Misdiagnosis can lead to inappropriate treatment and poor outcomes.
References:
[1] September 11, 2024 - Pulmonary veno-occlusive disease (PVOD) is one of the less commonly encountered causes of pulmonary hypertension. Some reports suggest that PVOD accounts for 5-20% of cases classified as idiopathic pulmonary arterial hypertension (PAH).
[3] Pulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (pulmonary hypertension).
[5] April 22, 2019 - Pulmonary veno-occlusive disease (PVOD) is a rare entity that is usually mistaken with pulmonary arterial hypertension (PAH) but is considered class I′ of PAH.
[6] August 18, 2020 - Pulmonary veno-occlusive disease (PVOD) is characterized by the blockage (occlusion) of the blood vessels that carry oxygen-rich (oxygenated) blood from the lungs to the heart (the pulmonary veins).
[7] April 4, 2024 - It is important to note that ... prostacyclin 6 can lead to disastrous outcomes (severe pulmonary edema) in patients with pulmonary veno-occlusive disease.
[9] by D Montani · 2016 · Cited by 287 — PVOD and PAH share a common clinical presentation and are characterised by insidious onset of fatigue and breathlessness progressing to symptoms of right heart failure.
Additional Characteristics
- PVOD is characterized by the blockage (occlusion) of the blood vessels that carry oxygen-rich blood from the lungs to the heart.
- Symptoms include fatigue, breathlessness, and symptoms of right heart failure.
- The condition can lead to disastrous outcomes (severe pulmonary edema) in patients with PVOD when treated with certain medications.
- Accurate diagnosis is crucial to distinguish PVOD from PAH and other forms of pulmonary hypertension.
- PVOD is a rare form of pulmonary hypertension, accounting for 5-20% of cases classified as idiopathic pulmonary arterial hypertension (PAH).
- The condition leads to high blood pressure in the lung arteries (pulmonary hypertension).
Signs and Symptoms
Common Signs and Symptoms of Pulmonary Veno-Occlusive Disease (PVOD)
Pulmonary veno-occlusive disease (PVOD) is a rare condition characterized by the blockage of small veins in the lungs, leading to high blood pressure in the pulmonary arteries. The symptoms of PVOD can be non-specific and may mimic other pulmonary and cardiac conditions.
Common Symptoms:
- Shortness of breath (dyspnea) [7][9]
- Fatigue during exertion [7][9]
- Cough [5]
- Swelling in the legs [3]
These symptoms are often nonspecific and can be similar to those experienced by patients with other pulmonary or cardiac conditions. In some cases, a respiratory tract infection-like illness may precede the diagnosis of PVOD.
Other Possible Symptoms:
- Increased pressure in the neck veins [9]
- Clubbing of the fingers (a sign of long-standing lung disease) [9]
It's essential to note that the symptoms of PVOD can be similar to those experienced by patients with other conditions, making it challenging to diagnose. A thorough medical examination and diagnostic tests are necessary to confirm the diagnosis.
References: [3] - The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. [5] - Sometimes, a respiratory tract infection–like illness may be identifiable preceding the diagnosis. [7] - Shortness of breath (dyspnea) and tiredness (fatigue) during exertion are the most common symptoms. [9] - The exam may reveal: Increased pressure in the neck veins; Clubbing of the fingers.
Additional Symptoms
- Swelling in the legs
- Increased pressure in the neck veins
- dyspnea
- clubbing of fingers
- cough
- fatigue
Diagnostic Tests
Pulmonary veno-occlusive disease (PVOD) can be challenging to diagnose, but various diagnostic tests can help confirm the condition. Here are some of the diagnostic tests used for PVOD:
- Arterial blood gases: This test measures the levels of oxygen and carbon dioxide in the blood to assess lung function.
- Blood oximetry: This test measures the amount of oxygen in the blood to determine if there is any issue with oxygenation.
- Chest x-ray: A chest x-ray can show signs of pulmonary hypertension, such as enlarged heart or fluid buildup in the lungs.
- Chest CT: A high-resolution computed tomography (CT) scan of the chest can help identify characteristic features of PVOD, such as dilated pulmonary veins and septal thickening [6].
- Cardiac catheterization: This test involves inserting a catheter into the heart to measure blood pressure and oxygen levels in the lungs.
- Lung function tests: These tests assess lung function by measuring parameters such as forced expiratory volume (FEV) and forced vital capacity (FVC).
- Echocardiogram: An echocardiogram can show signs of right ventricular dysfunction, which is common in PVOD patients.
It's worth noting that the absence or presence of only one diagnostic sign does not completely rule out the possibility of PVOD [6]. A comprehensive evaluation by a healthcare professional is necessary to confirm the diagnosis.
Additional Diagnostic Tests
- Lung function tests
- Echocardiogram
- Cardiac catheterization
- Chest x-ray
- Arterial blood gases
- Blood oximetry
- Chest CT
Treatment
Based on the search results, it appears that there are limited treatment options available for pulmonary veno-occlusive disease (PVOD). However, here are some potential drug treatments mentioned in the context:
- Vasodilators: While not specifically recommended for PVOD, vasodilators may be helpful for some people with this condition [3].
- Pulmonary arterial hypertension targeted therapy: The role of targeted therapy in PVOD remains controversial, and long-term effects have been rarely reported [5]. However, a study aims to retrospectively evaluate the role of targeted therapy in PVOD.
- No known effective medical treatment: Currently, there is no known effective medical treatment for PVOD [3, 9].
It's essential to note that lung transplantation is considered the only proven curative treatment for PVOD [1]. However, this option may not be feasible or recommended for everyone.
In terms of specific medications, none are specifically mentioned in the context as a first-line treatment for PVOD. The focus seems to be on the lack of effective medical treatments and the need for further research into targeted therapies.
References:
[1] Pulmonary veno-occlusive disease is characterized by significant venous and capillary involvement [1]. [3] Vasodilators may be helpful for some people with PVOD, but their effectiveness is not established [3]. [5] The role of targeted therapy in PVOD remains controversial, and long-term effects have been rarely reported [5]. [9] There is currently no known effective medical treatment for PVOD [9].
Recommended Medications
- Vasodilators
- Pulmonary arterial hypertension targeted therapy
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Pulmonary Veno-Occlusive Disease (PVOD) Differential Diagnosis
Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening condition that can be challenging to diagnose. A differential diagnosis, which involves ruling out other possible causes of symptoms, is essential in establishing the correct diagnosis.
Common Causes of Pulmonary Venous Hypertension
Before diagnosing PVOD, it's crucial to consider more common causes of pulmonary venous hypertension, such as:
- Systolic or diastolic left-sided heart failure
- Valvular heart disease (e.g., mitral stenosis)
These conditions can present with similar symptoms and should be ruled out before considering PVOD.
Differential Diagnosis Considerations
According to recent studies [1], the differential diagnosis of PVOD includes:
- Pulmonary capillary haemangiomatosis (PCH)
- Idiopathic pulmonary arterial hypertension (IPAH) or secondary PAH due to chronic lung diseases
- Chronic thromboembolic pulmonary hypertension
A thorough evaluation, including non-invasive investigations [9], is necessary to accurately diagnose PVOD and distinguish it from other conditions.
References
[1] More common causes of pulmonary venous hypertension should be considered as a part of the differential diagnosis before a diagnosis of pulmonary veno-occlusive disease (PVOD) is established. [2] May 1, 2016 - In clinical practice, direct ... demonstrated the clinical utility of this technique for the differential diagnosis of PVOD [79, 80]. [9] by D Montani · 2016 · Cited by 287 — An accurate diagnosis of PVOD based on noninvasive investigations is possible utilising oxygen parameters, low diffusing capacity for carbon monoxide and ...
Additional Differential Diagnoses
- Chronic thromboembolic pulmonary hypertension
- Pulmonary capillary haemangiomatosis (PCH)
- Idiopathic pulmonary arterial hypertension (IPAH) or secondary PAH due to chronic lung diseases
Additional Information
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- A pulmonary venoocclusive disease that has_material_basis_in homozygous or compound heterozygous mutation in the EIF2AK4 gene on chromosome 15q15 and that is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules, and is frequently associated with pulmonary capillary dilatation and proliferation.
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