THE ART BEHIND LINES, OPACITIES, ADENOPATHY & EDEMA: PULMONARY VENO-OCCLUSIVE DISEASE

Pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that mainly affects children and young adults. Its cause is unknown, although viral infections and drugs have been implicated. Patients with PVOD present with symptoms of right-sided heart failure. Radiologic examination shows prominent pulmonary arteries with Kerley B lines, pleural effusion, and mediastinal aden...

Pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease has recently been recognized as a distinct pathological entity and a cause of pulmonary arterial hypertension. Twenty previously reported cases and a new patient are here reviewed. The majority presented with breathlessness and in the early stages of the disease, when the abnormal signs were not striking, some patients were wrongly diagnosed as suffering from an...

Pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterised by preferential remodelling of the pulmonary venules. In the current PH classification, PVOD and pulmonary capillary haemangiomatosis (PCH) are considered to be a common entity and represent varied expressions of the same disease. The recent discovery of biallelic mutations in the EIF2AK4 gene as...

Pulmonary veno-occlusive disease. Entity or syndrome?

Pulmonary veno-occlusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins and venules. Without a lung biopsy the clinical diagnosis of this disease is difficult. If there is pulmonary hypertension with roentgenographic signs of pulmonary edema and of congestion in the absence of signs of increased left atrial pressure, the diagnosis mus...