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"Absent" unilateral pulmonary arteries aren't absent, but disconnected. Review of the current literature based on 4
Thomas Krasemann1, Michiel Dalinghaus1, Ingrid van Beynum1
1Department of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, 3015CN Rotterdam, Netherlands.
Background:
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.
Methods:
Four cases illustrate the anatomical findings on computed tomography and angiography, all show an outpouching at the base of the brachiocephalic artery.
Results:
The therapeutic approach consisted of stenting of the aberrant ductus and consecutive surgery. In the oldest patient, 13 years, such an approach was impossible.
Conclusion:
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.
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