Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)

  • Lisa Bergersen
  • Susan Foerster
  • Audrey C. Marshall
  • Jeffery Meadows


Because some of the technical details of this procedure are different from those described in the section on critical PS, PA/IVS is described separately. Patients with short-segment (membrane-like) pulmonary atresia are cyanotic at birth and require PGE for maintenance of pulmonary blood flow. This is perhaps the most straightforward example of ductal-dependant pulmonary blood flow. Among those with PA/IVS there is a spectrum, ranging from those with relatively good-sized RV chambers to those with significant RV hypoplasia or RV-dependent coronary circulation (1). Some degree of tricuspid valve hypoplasia is the rule and generally follows RV hypoplasia. Nevertheless, many patients with PA/IVS can achieve a biventricular circulation, and often their first step toward this is a trip as a newborn to the catheterization lab for valve perforation and dilation.


Tricuspid Valve Pulmonary Valve Pulmonary Blood Flow Pulmonary Atresia Coaxial Catheter 
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Review Article

  1. 1.
    Daubeney PE, Delaney DJ, Anderson, RH et al. Pulmonary atresia with intact ventricular septum range of morphology in a population-based study. J Am Coll Cardiol 2002;39:1670–1679.PubMedCrossRefGoogle Scholar
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    Giglia TM, Mandell VS, Connor AR et al. Diagnosis and management of right ventricle–dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992;86:1516–1528.PubMedCrossRefGoogle Scholar
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    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;81:2250–2257.PubMedCrossRefGoogle Scholar

Additional Readings

  1. Agnoletti G, Piechaud JF, Bonhoeffer P et al. Perforation of the atretic pulmonary valve long-term follow-up. J Am Coll Cardiol 2003;41:1399-1403.PubMedCrossRefGoogle Scholar
  2. Humpl T, Soderberg B, McCrindle BW et al. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum impact on patient care. Circulation 2003;108:826-831PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Lisa Bergersen
    • 1
  • Susan Foerster
    • 2
  • Audrey C. Marshall
    • 1
  • Jeffery Meadows
    • 3
  1. 1.Department of CardiologyChildren’s Hospital BostonBostonUSA
  2. 2.Washington University in St. LouisSt. LouisUSA
  3. 3.Division of Pediatric CardiologyUniversity of California at San FranciscoSan FranciscoUSA

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