Pulmonary Atresia with Intact Ventricular Septum (PA IVS)

  • In Sook Park
  • Hyun Woo Goo
  • Hye-Sung Won


This chapter includes types, morphology, clinical manifestations, diagnosis, natural and unnatural course, treatment, and fetal diagnosis of pulmonary atresia with intact ventricular septum, including one-and-half ventricle repair and Fontan operation.


Coronary fistula Balloon dilatation Valve perforation Radiofrequency energy Bidirectional cavopulmonary shunt Blalock-Taussig shunt Fontan operation 

Suggested Reading

  1. 1.
    Dyamenahalli U, McCrindle BW, McDonald C, et al. Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients. Cardiol Young. 2004;14:299–308.CrossRefGoogle Scholar
  2. 2.
    Guleserian KJ, Armsby LB, Thiagarajan RR, et al. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg. 2006;81:2250–8.CrossRefGoogle Scholar
  3. 3.
    Justo RN, Nykanen DG, Williams WG, et al. Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn. Catheter Cardiovasc Diagn. 1997;40:408–13.CrossRefGoogle Scholar
  4. 4.
    Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle dependent coronary circulation. Am J Cardiol. 2000;86:1272–4.CrossRefGoogle Scholar
  5. 5.
    Reddy VM, McElhinney DB, Silverman NH, et al. Partial biventricular repair for complex congenital heart defect: an intermediate option for complicated anatomy or functionally borderline right complex heart. J Thorac Cardiovasc Surg. 1998;116:21–7.CrossRefGoogle Scholar
  6. 6.
    Schneider AW, Blom NA, Bruggemans EF, et al. More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 2014;98:1680–6.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • In Sook Park
    • 1
  • Hyun Woo Goo
    • 2
  • Hye-Sung Won
    • 3
  1. 1.Department of Pediatric CardiologyUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea
  2. 2.Department of RadiologyUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea
  3. 3.Department of Obstetrics and GynecologyUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea

Personalised recommendations