Highlights
- •Congenital Unilateral Pulmonary Atresia may mimic pulmonary hypoplasia.
- •The presence of a normal plain radiograph at birth aids in the diagnosis as the imaging findings have a characteristic development over time.
- •The condition is surgically treatable if recognized early but may lead to pulmonary hemorrhage and pulmonary hypertension.
Abstract
Background
Congenital unilateral absence of the pulmonary vein (UCAPV) is a rare entity with
characteristic clinical and imaging findings. Despite its congenital nature, the radiographic
findings and symptoms of UCAPV may not be recognized at birth and patients may present
in childhood or early adulthood with findings that may mimic other diagnoses.
Methods
The evolution of imaging findings in UCAPV is presented through two cases, one of
which demonstrates the progression of findings over several years. The embryologic
basis of this entity is reviewed and the clinical presentation and characteristic
imaging findings including radiographs, nuclear scintigraphy, computed tomography,
magnetic resonance imaging and cardiac catheterization are demonstrated.
Results
Characteristically, normal at birth, radiographs demonstrate the gradual development
of a small lung and ipsilateral pulmonary artery over time. In addition to unilateral
absence of the pulmonary veins on CT or MRI, a mediastinal “soft tissue mass” reflecting
the development of mediastinal collaterals is a common finding and should be recognized
as secondary to the absent ipsilateral pulmonary veins rather than as a primary process
causing occlusion of the pulmonary veins. Scintigraphy will show absent perfusion
to the affected lung.
Conclusion
Awareness of the distinctive imaging findings in this unusual condition is critical
to avoid misdiagnosis and to prevent the consequences of UCAPV which include pulmonary
hypertension and extensive venous collaterals with or without hemoptysis, both of
which may prevent definitive repair.
Keywords
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Article info
Publication history
Published online: January 30, 2019
Accepted:
January 29,
2019
Received in revised form:
January 8,
2019
Received:
October 21,
2018
Identification
Copyright
© 2019 Published by Elsevier Inc.
