Patel Chinky,  Maller Vijetha

Final Pr. ID: Poster #: EDU-097

An isolated anomalous systemic arterial supply to a segment of the lung without associated sequestration is a rare congenital pulmonary abnormality. This anomaly was initially believed to be a type of pulmonary sequestration (Pryce type 1). However, it was later found to have different characteristics than a true sequestration. The affected pulmonary segment is supplied by an aberrant systemic artery, however, there is no abnormality of lung parenchyma such as a mass or cystic change to suggest sequestration. In addition, there is a normal pulmonary venous drainage into the left atrium and a normal bronchial tree. However, there is a fistulous communication between the abdominal aortic branch supplying the lung segment and the respective pulmonary vein which can cause an elevated systemic-to-pulmonary flow ratio due to left-to-left shunting. Sade and associates introduced a sequestration spectrum for the classification of various complexes of bronchovascular anomalies. Clements and Warner presented this anomaly as a “pulmonary malinosculation spectrum.”

Patients with this condition are usually asymptomatic. They may present with hemoptysis and exertional dyspnea with a continuous heart murmur. A large shunt can lead to left-sided overload and congestive heart failure.

On chest radiographs, the appearance of a tubular opacity from the upper abdomen to the right lower lobe (abdominal aortic supply to the lung) may mimic the appearance seen in Scimitar syndrome (a type of partial anomalous pulmonary venous return with hypoplasia of the right lung, which is drained by an anomalous pulmonary vein into the systemic venous system). CT angiography chest is required to make an accurate diagnosis to distinguish this anomaly from Scimitar syndrome as well as exclude the possibility of sequestration, as management is different in these conditions.

In this educational poster, we will review the clinical presentation, describe the imaging findings of anomalous systemic arterial supply to normal lung using examples from our own institution, and discuss the treatment options as well as the natural progression of the disease. Recognition of the imaging features in anomalous systemic arterial supply to normal segments of the lung is important to identify patients at risk of developing left-to-left shunt. Endovascular coil embolization or surgical ligation of the aberrant systemic artery to the lung segment is usually the treatment of choice in symptomatic patients. Read More

Authors:  Patel Chinky , Maller Vijetha

Keywords:  malinosculation, sequestration, bronchovascular