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MAPCAs following D-TGA repair and pulmonary stenosis

Case contributed by Dr Issac Yang

Presentation

Patient with D-TGA post arterial switch, hypoplastic right ventricle and prior right pulmonary artery stenosis.

Patient Data

Age: 35 years
Gender: Male
  • Anterior position of the pulmonary artery relative to the aorta indicating prior arterial switch procedure.
  • There is asymmetric narrowing of the right main pulmonary artery compared to the left.
  • The right ventricle is diminished in size relative to the left ventricle.
  • There are numerous large (>2mm) right-sided aorto-pulmonary collateral arteries arising from: right subclavian artery (origin not in field of view), right internal mammary artery, left common carotid artery, right internal mammary artery, right coronary artery, left circumflex coronary artery, and right inferior phrenic artery (origin not in field of view). In addition, there are numerous small (<1mm) and medium (1-2mm) collateral arteries arising from vertebral and intercostal arteries, as well as likely the right superior epigastric artery.
  • Two Amplatzer vascular plugs are present in the prevertebral region at and slightly above the level of the aortic arch from prior collateral embolization.

Volume rendered MRA depicting the origins of collateral arteries arising from the right subclavian artery, left common carotid artery and right inferior phrenic artery (arising from the celiac trunk). The origin of the right subclavian artery collateral demonstrates aneurysmal dilation.

Case Discussion

This patient is known for D-TGA and right ventricular hypoplasia, who underwent arterial switch and right sided unidirectional Glenn procedure (SVC to right pulmonary artery anastamosis).  He later developed stenosis of the right pulmonary artery, requiring surgical reconstruction with Glenn take down. Multiple (right-sided) major aortopulmonary collateral arteries (MAPCAs) developed as a result of the right-sided pulmonary artery stenosis.

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Case information

rID: 76218
Published: 4th May 2020
Last edited: 22nd May 2020
System: Vascular, Cardiac
Inclusion in quiz mode: Included
Institution: McGill University

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