Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation

Authors

  • Abul Kalam Shamsuddin Associate Professor, Pediatric Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Prodip Kumar Biswas Registrar, Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Muhammad Ishtiaque Sayeed Al Manzoo Registrar, Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Md Abul Kalam Azad Registrar, Pediatric Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Md Nurul Akhtar Hasan Associate professor, pediatric cardiac ICU National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Jasmin Hosain Assistant professor, pediatric cardiology National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh
  • Mohammad Sharifuzzaman Shamsuddin Professor, Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh

DOI:

https://doi.org/10.3329/bhj.v35i1.49148

Keywords:

Tetralogy of Fallot, Absent Left pulmonary artery, absent pulmonary valve syndrome

Abstract

Absent left pulmonary artery with Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS), is a rare congenital cardiac anomaly. Here we present such a case of A 2 year 11 month old girl with cyanosis, exertional dyspnoea. Her diagnosis is confirmed by echocardiography and CT angiogram. There are very few cases have been reported till date with high postoperative mortality. Although per operative decision making was challenging regarding pulmonary valve and size of the RPA, we performed ICR with RPA reductionplasty and creation of monocuspid pulmonary valve with success. As it is a rare association and we have overcome the hindrance we came across per operatively, we are reporting this case.

Bangladesh Heart Journal 2020; 35(1) : 74-77

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Published

2020-09-15

How to Cite

Shamsuddin, A. K., Biswas, P. K., Manzoo, M. I. S. A., Azad, M. A. K., Hasan, M. N. A., Hosain, J., & Shamsuddin, M. S. (2020). Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation. Bangladesh Heart Journal, 35(1), 74–77. https://doi.org/10.3329/bhj.v35i1.49148

Issue

Vol. 35 No. 1 (2020)

Section

Case Reports

License

© Bangladesh Cardiac Society.  

Creative Commons Licence
Articles in the Bangladesh Heart Journal are Open Access articles published under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC).  This license permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.