Advertisement

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
  • 16 Downloads

Abstract

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.

Keywords

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

Notes

Funding

None.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interests.

References

  1. 1.
    Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia—ventricular septal defect. Ann Thorac Surg. 2000;69:97–105.CrossRefGoogle Scholar
  2. 2.
    Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. 2000;101:1826–32.CrossRefGoogle Scholar
  3. 3.
    Liava’a M, Brizard CP, Konstantinov IE, et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization. Ann Thorac Surg. 2012;93:185–91.CrossRefGoogle Scholar
  4. 4.
    Reddy VM, Petrossian E, McElhinney DB, Moore P, Teitel DF, Hanley FL. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed? J Thorac Cardiovasc Surg. 1997;113:858–66 (discussion 866–8).CrossRefGoogle Scholar
  5. 5.
    Brawn WJ, Jones T, Davies B, Barron D. How we manage patients with major aorta pulmonary collaterals. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12:152–7.CrossRefGoogle Scholar
  6. 6.
    Mainwaring RD, Patrick WL, Roth SJ, et al. Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 2018;156:1194–204.CrossRefGoogle Scholar
  7. 7.
    Mainwaring RD, Sheikh AY, Punn R, Reddy VM, Hanley FL. Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome. Eur J Cardiothorac Surg. 2012;42:235–40 (discussion 40–1).CrossRefGoogle Scholar

Copyright information

© The Japanese Association for Thoracic Surgery 2019

Authors and Affiliations

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

Personalised recommendations