Purpose or Case Report: Pulmonary sequestration is a rare - but well-characterized - congenital malformation of the foregut, the hallmark of the diagnosis consists in the detection of an anomalous systemic arterial supply to a segment of the lung that is isolated from the tracheobronchial tree and therefore nonfunctional, with a pulmonary and/or systemic venous drainage. Depending on the relationship of the abnormal parenchyma with the pleura, it can be classified as intralobar or extralobar.
Nevertheless, the spectrum of congenital lung malformations includes lesions that are predominantly parenchymal, predominantly vascular or hybrid, with variable foregut or airway components intertwined. The exact physiopathological mechanisms for each one of these malformations are yet to be fully understood.
We present a 15-month-old girl with a history of dilated cardiomyopathy. Mild-to-moderate left ventricular (LV) enlargement and a small persistent ductus arteriosus (PDA) were seen on the echocardiogram. The size of the PDA did not explain the LV dilation. An angiography was done to assess the coronary arteries, which were normal. However, it showed an aberrant vessel from the descending aorta to the left lower lobe and severe pulmonary hypertension in the supplied lung, reactive to oxygen. A CT was performed to assess the airway, ruling out the possibility of a pulmonary sequestration and confirming the diagnosis of an isolated major aortopulmonary collateral artery (MAPCA). No evidence of structural heart disease was seen. Unifocalization and ductal ligation were planned.
MAPCA in the absence of an associated congenital heart disease is a rare entity, most seen in preterm babies. The isolated MAPCA tends to regress spontaneously in most cases. However, in a smaller group of patients, it can persist and lead to recurrent respiratory tract infection,
pulmonary hypertension, and lastly heart failure due to left-to-right shunt. Contrast-enhanced computed tomography (CT) is an excellent method for correct identification and treatment guidance.
The purpose of this presentation is to describe an unusual case of our practice, in which the imaging finding of an aberrant arterial supply to the lung is not always related to a pulmonary sequestration, and therefore to draw attention to the fact that in the spectrum of congenital lung
anomalies that are predominantly vascular, isolated MAPCA can be found as the only alteration.
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