Khadir Mohammed Abdul, Narayana Ganesh, Ramagopal Ganavi, Nayar Pradeep G
Registrar, Department of Cardiology, Chettinad Hospital and Research Institute , Chennai, Tamil Nadu, India .
Associate Professor, Department of Cardiology, Chettinad Hospital and Research Institute , Chennai, Tamil Nadu, India .
J Clin Diagn Res. 2016 Dec;10(12):SD04-SD05. doi: 10.7860/JCDR/2016/23224.9093. Epub 2016 Dec 1.
Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis. Early diagnosis and early surgical indication avoids the evolution of pulmonary hypertension to unfavourble state of more severe and progressive degrees and also prevents the development of pulmonary systemic collateral circulation, which is mainly responsible for subsequent haemoptysis in the adulthood. We hereby, report the case of an infant who presented with features of lower respiratory tract infection and later diagnosed as isolated congenital hypoplasia of left pulmonary artery and hence planned for proper follow-up for early surgery thereby preventing complications in the future.
孤立性单侧肺动脉分支发育不全或缺如非常罕见。它通常与先天性心脏病相关,如法洛四联症、房间隔缺损、主动脉缩窄、右位主动脉弓、动脉干、动脉导管未闭和肺动脉闭锁。它是由于左或右第六主动脉弓胚胎发育缺失所致,已发现其有多种临床表现,在儿童期表现为对侧肺动脉高压,在成人期表现为咯血。早期诊断和早期手术指征可避免肺动脉高压发展到更严重和进行性的不利状态,还可防止肺-体循环侧支循环的形成,而这主要是成年期后续咯血的原因。我们在此报告一例婴儿病例,该婴儿最初表现为下呼吸道感染症状,后来被诊断为孤立性先天性左肺动脉发育不全,因此计划进行适当的随访以便早期手术,从而预防未来的并发症。