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

  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...

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Veröffentlicht in:Indian journal of thoracic and cardiovascular surgery 2022-11, Vol.38 (6), p.637-643
Hauptverfasser: Katewa, Ashish, Saxena, Shivang, Malhotra, Pulkit, Rajesh, Venuthurupalli S. P., Champaneri, Bhavik, Surti, Jigar
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container_end_page 643
container_issue 6
container_start_page 637
container_title Indian journal of thoracic and cardiovascular surgery
container_volume 38
creator Katewa, Ashish
Saxena, Shivang
Malhotra, Pulkit
Rajesh, Venuthurupalli S. P.
Champaneri, Bhavik
Surti, Jigar
description   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.
doi_str_mv 10.1007/s12055-022-01393-4
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subjects Cardiac Surgery
Medicine
Medicine & Public Health
Mini Review
Mini Review Article
Surgery
Thoracic Surgery
Vascular Surgery
title Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot
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