Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis

ABSTRACT Unilateral pulmonary artery atresia (UPAA) is a rare congenital anomaly marked by the absence of one of the pulmonary arteries. This article examines three instances of UPAA, predominantly affecting the right side, although one case involved the left side. Common characteristics included he...

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Main Authors: Vignesh Kumar Kathiresan, Benjamin Earnest Williams, V. S. Aswith Chowdary Jonnalagadda
Format: Article
Language:English
Published: Wiley 2025-07-01
Series:Respirology Case Reports
Subjects:
Online Access:https://doi.org/10.1002/rcr2.70280
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author Vignesh Kumar Kathiresan
Benjamin Earnest Williams
V. S. Aswith Chowdary Jonnalagadda
author_facet Vignesh Kumar Kathiresan
Benjamin Earnest Williams
V. S. Aswith Chowdary Jonnalagadda
author_sort Vignesh Kumar Kathiresan
collection DOAJ
description ABSTRACT Unilateral pulmonary artery atresia (UPAA) is a rare congenital anomaly marked by the absence of one of the pulmonary arteries. This article examines three instances of UPAA, predominantly affecting the right side, although one case involved the left side. Common characteristics included hemoptysis, observed in two patients—one with a chronic history necessitating embolization and another with a mild, recent onset. The third case was identified incidentally during a preoperative evaluation. All cases demonstrated varying degrees of ipsilateral lung volume reduction and associated interstitial changes or fibrosis. UPAA can be asymptomatic or present with hemoptysis, recurrent infections, or breathlessness. Diagnosis relies on computed tomography pulmonary angiography (CTPA) or magnetic resonance (MR) angiography. Management ranges from observation to embolization or pneumonectomy in severe cases. Mortality is around 7%, with pulmonary hypertension and haemorrhage being major prognostic factors.
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series Respirology Case Reports
spelling doaj-art-eda33110491f4cd0b8bef75d4bfb186a2025-08-20T03:58:40ZengWileyRespirology Case Reports2051-33802025-07-01137n/an/a10.1002/rcr2.70280Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery AgenesisVignesh Kumar Kathiresan0Benjamin Earnest Williams1V. S. Aswith Chowdary Jonnalagadda2Department of Pulmonary Medicine Christian Medical College Vellore Ranipet Campus IndiaDepartment of Pulmonary Medicine Christian Medical College Vellore Ranipet Campus IndiaDepartment of Pulmonary Medicine Christian Medical College Vellore Ranipet Campus IndiaABSTRACT Unilateral pulmonary artery atresia (UPAA) is a rare congenital anomaly marked by the absence of one of the pulmonary arteries. This article examines three instances of UPAA, predominantly affecting the right side, although one case involved the left side. Common characteristics included hemoptysis, observed in two patients—one with a chronic history necessitating embolization and another with a mild, recent onset. The third case was identified incidentally during a preoperative evaluation. All cases demonstrated varying degrees of ipsilateral lung volume reduction and associated interstitial changes or fibrosis. UPAA can be asymptomatic or present with hemoptysis, recurrent infections, or breathlessness. Diagnosis relies on computed tomography pulmonary angiography (CTPA) or magnetic resonance (MR) angiography. Management ranges from observation to embolization or pneumonectomy in severe cases. Mortality is around 7%, with pulmonary hypertension and haemorrhage being major prognostic factors.https://doi.org/10.1002/rcr2.70280agenesiscollateral circulationhemoptysispulmonary arteryunilateral lung hypoplasia
spellingShingle Vignesh Kumar Kathiresan
Benjamin Earnest Williams
V. S. Aswith Chowdary Jonnalagadda
Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
Respirology Case Reports
agenesis
collateral circulation
hemoptysis
pulmonary artery
unilateral lung hypoplasia
title Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
title_full Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
title_fullStr Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
title_full_unstemmed Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
title_short Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis
title_sort breathing with half a pulmonary circuit three cases of unilateral pulmonary artery agenesis
topic agenesis
collateral circulation
hemoptysis
pulmonary artery
unilateral lung hypoplasia
url https://doi.org/10.1002/rcr2.70280
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