Pulmonary hypertension secondary to veno-occlusive disease in a 15-years old boy: a case report.

Abstract

Pulmonary hypertension (PH) is a pathology characterized by a mean arterial pressure of the pulmonary artery greater than 20 mmHg and with a classification system based on its etiologies. Among the etiologies of pulmonary hypertension, there is the veno-occlusive disease, a rare pathology characterized by a continuous process of occlusion of pulmonary venules and veins leading to a progressive increase in pulmonary vascular resistance, whose pharmacological treatment results with traditionally used drugs for HP is still undetermined while pulmonary transplantation shows as the only definitive treatment. We report the case of a young patient complaining of dyspnea on exertion with rapid evolution to an acute case of hydropneumothorax, whose diagnosis of veno-occlusive disease was established after imaging studies associated with pulmonary catheterization. Combined treatment with vasodilator drugs of different classes was initiated, and the patient presented sustained remission of symptoms and improved quality of life for five years, while his medium expectative of life was 3 years long after the diagnosis.

Keywords

• pulmonary hypertension; pulmonary veno-occlusive disease; cardiac insufficiency; pneumology