Unilateral pulmonary vein atresia presenting with recurrent haemoptysis in a child : a case report

• Main Authors: Martin Ngie, Liong Wong, Tang, Ing Ping, Chor, Yek Kee, Lau, Kiew Siong, Anne Rachel, John, Hii, King Ching, Olive Pei, Yi Lee, Lim, Wooi Kok, Hannah Pei, Koon Tan
• Format: Article
• Language: English
• Published: BioMed Central Ltd. 2021
• Subjects: RF Otorhinolaryngology
• Online Access: http://ir.unimas.my/id/eprint/45262/1/Unilateral%20pulmonary.pdf; http://ir.unimas.my/id/eprint/45262/; https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-020-02348-7; https://doi.org/10.1186/s12887-020-02348-7

Background: Haemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child. Case presentation: A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation excluded a foreign body aspiration but revealed right bronchial mucosal hyperaemia and varices. Diagnosis of right unilateral PVA was suspected on transthoracic echocardiography which demonstrated hypoplastic right pulmonary artery and non-visualization of right pulmonary veins. Final diagnosis was confirmed on cardiac CT angiography. A conservative treatment approach was opted with consideration for pneumonectomy in future when she is older. Conclusion: Rarer causes should be considered when investigating for recurrent haemoptysis in children. Bronchoscopy and cardiac imaging are useful tools to establish the diagnosis of unilateral PVA in our case.