Pulmonary veno occlusive disease

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterized by the blockage of small veins in the lungs. This disease is often misdiagnosed as pulmonary arterial hypertension due to the similarities in clinical presentation. However, the treatment and prognosis for these two conditions are significantly different.

Etiology

The exact cause of PVOD is unknown. However, it is believed to be associated with several factors including exposure to certain toxins such as chemotherapy drugs, organic solvents, and tobacco smoke. Genetic factors may also play a role, as mutations in the EIF2AK4 gene have been identified in some patients with familial PVOD.

Clinical Presentation

Patients with PVOD typically present with symptoms of progressive dyspnea (shortness of breath), fatigue, and signs of right heart failure such as edema (swelling) and ascites (fluid accumulation in the abdomen). On physical examination, patients may have a loud pulmonary component of the second heart sound, jugular venous distension, and hepatomegaly (enlarged liver).

Diagnosis

Diagnosis of PVOD can be challenging due to its rarity and the nonspecific nature of its symptoms. It is often misdiagnosed as other forms of pulmonary hypertension. Diagnostic tests may include echocardiography, pulmonary function tests, and right heart catheterization. However, the definitive diagnosis of PVOD is made by lung biopsy, which shows characteristic changes in the small veins of the lungs.

Treatment

Treatment of PVOD is primarily supportive, as there is currently no cure for the disease. Management strategies may include oxygen therapy, diuretics to manage fluid overload, and anticoagulation to prevent blood clots. Lung transplantation may be considered in severe cases.

Prognosis

The prognosis of PVOD is generally poor, with a median survival of 2 years from the time of diagnosis. However, survival can vary widely depending on the severity of the disease and the patient's response to treatment.

NIH genetic and rare disease info

• NIH info on Pulmonary veno occlusive disease

Pulmonary veno occlusive disease is a rare disease.

Template:Pulmonary hypertension

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