Lung ventilation/perfusion scintigraphy in pulmonary capillary hemangiomatosis: a pattern to consider

Pulmonary capillary hemangiomatosis is a rare disease, characterized by small capillaries proliferation, leading to pulmonary hypertension. The authors report a case of pulmonary capillary hemangiomatosis, and discuss its diagnostic difficulties. Special attention is presented to ventilation/perfusion scintigraphy, given both its importance to the evaluation of pulmonary hypertension, and its referred limited usefulness in pulmonary capillary hemangiomatosis. The few published cases of ventilation/perfusion scintigraphy on this entity have showed different patterns, which are discussed. This case presents a pattern with augmented perfusion on lung bases and normal ventilation, which has been described by other authors as typical for pulmonary capillary hemangiomatosis. The authors consider important to retain this pattern, when evaluating pulmonary hypertensive patients, given not only its possible ability to help on pulmonary capillary hemangiomatosis diagnosis, but also mainly its risk of misinterpretation as a decreased perfusion on upper lung lobes, leading to erroneous diagnostic hypotheses.