Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Yap J, Sharma R, et al. Pulmonary atresia with intact interventricular septum. Reference article, Radiopaedia.org (Accessed on 28 Mar 2025) https://doi.org/10.53347/rID-31901
Pulmonary atresia with intact interventricular septum (PA-IVS) is a subtype of pulmonary atresia that presents as cyanotic congenital heart disease.
PA-IVS is the combination of obstruction of the pulmonary outflow tract from pulmonary valve atresia without a ventricular septal defect. Blood returns to the left heart via a patent foramen ovale, and a patent ductus arteriosus (PDA) is required for pulmonary blood flow.
The right ventricle is variably hypoplastic, and the right atrium is typically dilated.
Associations
Plain radiograph
The features on chest radiographs are variable depending on associated anomalies 8:
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cardiomegaly may be present with severe tricuspid regurgitation
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pulmonary plethora may be present with large left-to-right shunts (from PDA or aortopulmonary collaterals)
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Treatment and prognosis
The prognosis depends on the degree of atresia as well as whether it is unilateral versus bilateral. Percutaneous interventional perforation is an effective first-stage procedure 5.
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1. Daubeney PE, Sharland GK, Cook AC et-al. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation. 1998;98 (6): 562-6. doi:10.1161/01.CIR.98.6.562 - Pubmed citation
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2. Liava'a M, Brooks P, Konstantinov I et-al. Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardiothorac Surg. 2011;40 (6): 1406-11. doi:10.1016/j.ejcts.2011.02.036 - Pubmed citation
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3. Hung JH, Lu JH, Hung J et-al. Prenatal diagnosis of pulmonary atresia with an intact ventricular septum and Ebstein malformation. J Ultrasound Med. 2006;25 (8): 1069-73. J Ultrasound Med (full text) - Pubmed citation
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4. Shinebourne EA, Rigby ML, Carvalho JS. Pulmonary atresia with intact ventricular septum: from fetus to adult: congenital heart disease. Heart. 2007;94 (10): 1350-7. doi:10.1136/hrt.2006.108936 - Pubmed citation
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5. Marasini M, Gorrieri PF, Tuo G et-al. Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum. Heart. 2009;95 (18): 1520-4. doi:10.1136/hrt.2009.169078 - Pubmed citation
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6. Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv. 2006;67 (5): 679-86. doi:10.1002/ccd.20672 - Pubmed citation
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7. Peraira JR, Burgueros M, Esteban I et-al. Pulmonary atresia with intact ventricular septum associated with severe aortic stenosis. Rev Esp Cardiol. 2004;56 (12): 1235-8. Pubmed citation
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8. Heart Diseases in Children: A Pediatrician's Guide. Springer. ISBN:B00F5U1GS0. Read it at Google Books - Find it at Amazon
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