Objective: The purpose of this article is to present serial clinical and imaging findings for the "absent" pulmonary artery. Data from six patients with this condition (five unilateral cases and one bilateral case) provide evidence concerning its embryology and illustrate the therapeutic implications of surgical intervention.

Conclusion: In our series, we found the anatomy of the absent pulmonary artery to be consistent with a distal ductal origin and involution of the proximal sixth aortic arch. The absent pulmonary artery is a distinct embryologic entity that requires early detection and detailed investigation. Early surgical intervention may be justified in selected patients.

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http://dx.doi.org/10.2214/ajr.179.5.1791253DOI Listing

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