Congenital anomalies of the pulmonary vasculature include a variety of malformations that arise due to errors in embryological development. These anomalies may remain asymptomatic or present with nonspecific symptoms such as respiratory distress, frequent infections, hemoptysis, or pulmonary hypertension, making diagnosis challenging. Early detection is crucial for effective management, as these conditions can lead to serious complications, including heart failure, inadequate blood oxygenation, and pulmonary hypertension.
The prevalence of pulmonary vascular malformations varies depending on the specific type and the population studied. They are more commonly observed in patients with genetic syndromes or unexplained pulmonary issues. In pediatric patients, chest radiography and echocardiography are often the initial diagnostic modalities, while computed tomography (CT) is the modality of choice in adults for evaluating pulmonary arteries, veins, and associated structures.
Advanced imaging techniques, such as CT angiography (CTA), play a pivotal role in delineating the anatomy and nature of these anomalies. Understanding the precise characteristics of malformations guides treatment decisions, including the need for surgical intervention or other therapies, ultimately improving patient outcomes.