Other Pulmonary Vascular Anomalies: Anatomy, Epidemiology, Diagnosis, and Treatment

Pulmonary vascular malformations consist of a spectrum of anomalies, ranging from abnormal vascular connection to normal lung tissue to vascular malformations that connect to abnormal lung tissue. These are rare lesions, the most common being bronchopulmonary sequestration, hybrid lesions containing both congenital cystic pulmonary airway malformation and bronchopulmonary sequestration features, lesions of aberrant venous or arterial systemic anastomoses and pulmonary arteriovenous malformations. Various classifications have been proposed using different terminologies, such as "congenital bronchopulmonary vascular malformation" and "congenital bronchopulmonary foregut malformation"; however, a proper classification is not defined yet, because none of the proposed theories is able to explain the origin of the various malformations, which share common elements. Only a multidisciplinary approach may allow a better understanding of their etiology, natural history, and associated anomalies, leading to the more appropriate treatment.