This term comprises a heterogeneity of uncommon abnormalities that may occur singly or in combinations involving anomalous connections of the lung parenchyma, the pulmonary and systemic vasculature, and, rarely, the gastrointestinal tract. Most of the patients present as asymptomatic. In cases of concurrent cardiac anomalies or severe forms of pulmonary atresia or hypoplasia clinical symptoms may occur at an early age. Surgical therapy may be required in selected cases of intractable infections, hemoptysis, and congestive heart failure or pulmonary hypertension due to excessive shunting. Precise anatomic identification of abnormal pulmonary and systemic vessels as well as tracheobronchial anomalies is mandatory for preoperative evaluation of affected patients [18,19].
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