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CC BY-NC-ND 4.0 · Indian J Radiol Imaging
DOI: 10.1055/s-0045-1802669
Case Reports

Type 2 Congenital Pulmonary Airway Malformation in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Damandeep Singh 
1   Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
,
Vineeta Ojha 
1   Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
,
Aprateem Mukherjee
1   Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
,
Lamak Kadiyani
2   Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
,
Sanjeev Kumar
1   Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
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Abstract

Tetralogy of Fallot with absent pulmonary valve syndrome is a unique congenital condition often accompanied with dilated pulmonary arteries causing varied degree of airway compression. Frequently, abnormal pulmonary arterial branching at the segmental level and compression at the level of intrapulmonary bronchi can be seen. The occurrence of isolated bronchopulmonary malinosculation in the form of congenital pulmonary airway malformation is exceedingly rare. This report highlights a case of such rarity, involving a young child with recurrent lower respiratory tract infections.

Keywords

absent pulmonary valve syndrome - CPAM - CT angiography - tetralogy of Fallot

Patient's Consent

Informed consent was taken from the concerned patient.




Publication History

Article published online:
27 March 2025

© 2025. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Cheevers N. Raaetraaecissement congenital de l” orifice pulmonaire. Arch Med Fourth Series 1847; 15: 488
    Search in Google Scholar
  • 2 Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol 1977; 8 (02) 155-171
    CrossrefPubMedSearch in Google Scholar
  • 3 Keivanidou A, Gogou M, Giannopoulos A. Tetralogy of Fallot with absent pulmonary valve syndrome; an imaging challenge. Images Paediatr Cardiol 2015; 17 (02) 1-2
    PubMedSearch in Google Scholar
  • 4 Verma M, Pandey NN, Ojha V. et al. Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of Fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography. J Card Surg 2021; 36 (08) 2697-2704
    CrossrefPubMedSearch in Google Scholar