Complete repair with unifocalization of the only unilateral lung

  • Motonori IshidouEmail author
  • Keisuke Ota
  • Kazuyoshi Kanno
  • Masaya Murata
  • Keiichi Hirose
  • Hisao Nagato
  • Kisaburo Sakamoto
  • Akio Ikai
Case Report


A boy with 22q11.2 deletion was diagnosed with pulmonary atresia with ventricular septal defect (PAVSD) and major aortopulmonary collateral arteries (MAPCAs). At 8 months, unifocalization of left MAPCAs and BT shunt was performed at another hospital. However, they occluded directly after surgery. An angiography revealed 2 MAPCAs supplying all segments of the right lung. Qp:Qs was 2.24. At 1 year 6 months, we performed VSD closure with unifocalization of the only right lung vascularity, using intraoperative PA flow study. We report a successful case of complete repair with unilateral lung in PAVSD and MAPCAs.


Pulmonary atresia with ventricular septal defect Major aortopulmonary collateral arteries Intraoperative pulmonary flow study Unifocalization Unilateral lung 




Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interests.


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Copyright information

© The Japanese Association for Thoracic Surgery 2019

Authors and Affiliations

  1. 1.Department of Cardiovascular SurgeryMt. Fuji Shizuoka Children’s HospitalShizuokaJapan

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