Pulmonary Veno Occlusive Disease (PVOD) is a rare and severe form of pulmonary hypertension (PH) that affects the small veins (venules) and pulmonary capillaries within the lungs. It is characterized by the narrowing, inflammation, and eventual obstruction of these blood vessels, leading to increased resistance and elevated blood pressure in the pulmonary circulation.
PVOD is often considered an idiopathic disease, meaning its exact cause is unknown. However, it has been associated with genetic mutations related to certain proteins involved in the structure and function of blood vessels. In some cases, PVOD can occur as a complication of other medical conditions like connective tissue disorders, bone marrow transplantation, or exposure to certain toxins.
The progressive narrowing of the pulmonary veins and capillaries leads to compromised blood flow and reduced oxygen exchange within the lungs. Consequently, individuals with PVOD may experience symptoms such as shortness of breath, fatigue, cough, chest pain, and in advanced stages, right-sided heart failure.
Diagnosis of PVOD requires a combination of clinical evaluation, imaging studies (such as chest X-rays, computed tomography scans, or echocardiography), and pulmonary function tests. The definitive diagnosis may be confirmed through invasive procedures like a lung biopsy or pulmonary angiography.
Currently, there is no cure for PVOD, and treatment mainly focuses on managing symptoms and slowing the progression of the disease. Medications such as pulmonary vasodilators and diuretics are often prescribed to alleviate symptoms and reduce fluid buildup. In severe cases, lung transplantation may be considered as a viable treatment option.
Overall, PVOD is a complex and debilitating condition that requires specialized medical care to ensure proper management and improve the quality of life for affected individuals.
