Abstract
Disconnected unilateral pulmonary arteries are frequently misdiagnosed as "absent". They typically arise from the base of the innominate artery and are fed by an aberrant arterial duct. If diagnosed early enough, they can be reconnected with catheter techniques even after closure of this aberrant duct. Consecutive surgical anatomical correction at a later stage is possible. Four cases illustrate the anatomical findings on computed tomography and angiography, all show an outpouching at the base of the brachiocephalic artery. The therapeutic approach consisted of stenting of the aberrant ductus and consecutive surgery. In the oldest patient, 13 years, such an approach was impossible. If identified early in life, disconnected pulmonary arteries can be recruited with catheter techniques, and reconnected surgically at a later stage. It is not yet known if this approach prevents pulmonary damage, which is frequently seen in older untreated patients.
Highlights
Disconnected pulmonary arteries are frequently misdiagnosed as “absent” [1]
Pulmonary venous wedge angiography can help delineate the anatomy of the disconnected pulmonary artery, and determine the distance between the origin of the ductal ampulla and the hilus of the pulmonary artery (PA) [3]
Treatment may consist of primary surgery to reconnect the disconnected PA, or of initial catheterization to recanalise the arterial duct followed by surgery at a later stage [2,4]
Summary
Disconnected pulmonary arteries are frequently misdiagnosed as “absent” [1]. Frequently these are initially fed by an aberrant ductus from the origin of the innominate artery, and become disconnected once the ductus closes [2]. Treatment may consist of primary surgery to reconnect the disconnected PA, or of initial catheterization to recanalise the arterial duct followed by surgery at a later stage [2,4]. Both strategies can achieve growth of the PA. In older children with this entity, abnormal lungs with bronchiectasis, recurrent pulmonary infections, hemoptysis and respiratory distress have been reported as early as 1982 [8] It is not yet known if these changes can be prevented by early reconnection of the PA. We present four cases of this entity, which illustrate anatomical and technical considerations
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