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Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot

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Indian Journal of Thoracic and Cardiovascular Surgery Aims and scope Submit manuscript

Abstract 

The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1–3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.

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Authors and Affiliations

  1. Department of Cardio-Thoracic & Vascular Surgery, U N Mehta Institute of Cardiology and Research Center, Medicity, Asarwa, Ahmedabad, Gujarat, 3800016, India

    Ashish Katewa, Shivang Saxena & Pulkit Malhotra

  2. Department of Cardiac Anaesthesia, U N Mehta Institute of Cardiology and Research Center, Medicity, Asarwa, Ahmedabad, Gujarat, 3800016, India

    Venuthurupalli S. P. Rajesh & Jigar Surti

  3. Department of Pediatric Cardiology, U N Mehta Institute of Cardiology and Research Center, Medicity, Asarwa, Ahmedabad, Gujarat, 3800016, India

    Bhavik Champaneri

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  1. Ashish Katewa
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  2. Shivang Saxena
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  3. Pulkit Malhotra
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Correspondence to Ashish Katewa.

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Ethical approval was waived by the local Ethics Committee of U N Mehta Institute of Cardiology and Research Centre in view of the retrospective nature of the study and all the procedures being performed were part of the routine care.

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The authors have no relevant financial or non-financial interests to disclose.

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Informed consent was obtained from the parents for submission of the case report to the journal.

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Katewa, A., Saxena, S., Malhotra, P. et al. Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot. Indian J Thorac Cardiovasc Surg 38, 637–643 (2022). https://doi.org/10.1007/s12055-022-01393-4

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  • DOI: https://doi.org/10.1007/s12055-022-01393-4

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