Vignesh Kumar Kathiresan, Benjamin Earnest Williams, V S Aswith Chowdary Jonnalagadda
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Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis.
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.
期刊介绍:
Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.