Zhang Liang-Ze, Ma Wei-Guo, Gao Shu-Geng, He Jie
Department of Thoracic Surgery, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, China.
Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China;; Aortic Institute at Yale-New Haven, Yale University School of Medicine, New Haven 06519, Connecticut, USA.
J Thorac Dis. 2016 Sep;8(9):E942-E946. doi: 10.21037/jtd.2016.08.97.
Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiac malformation that is often associated with other cardiovascular deformities. Surgical repair of this rare condition is usually performed only on the abnormal lung. The occurrence of lung cancer in association with UAPA is even rarer and clinical experience is very limited. This report aims to describe a case of unilateral absence of right pulmonary artery that was complicated by primary carcinoma of the contralateral lung. A left lower lobectomy was performed despite the absence of the right pulmonary artery and repeated decreases in the arterial oxygen saturation (SaO) were encountered intraoperatively. The current case provides insights into the operative tolerability and the foreseeable ominous prognosis after excision of the normal lung in patients with UAPA and highlights the importance of the clinical awareness of this potentially lethal congenital anomaly in light of its extreme rarity, which may facilitate better diagnosis and treatment of such patients.
单侧肺动脉缺如(UAPA)是一种罕见的先天性心脏畸形,常与其他心血管畸形相关。这种罕见病症的手术修复通常仅针对异常肺进行。与UAPA相关的肺癌发生更为罕见,临床经验非常有限。本报告旨在描述一例右侧肺动脉缺如并伴有对侧肺原发性癌的病例。尽管右侧肺动脉缺如,仍进行了左下肺叶切除术,术中动脉血氧饱和度(SaO)反复下降。本病例为了解UAPA患者切除正常肺后的手术耐受性和可预见的不良预后提供了见解,并鉴于这种潜在致命性先天性异常极为罕见,强调了临床认识其重要性,这可能有助于对此类患者进行更好的诊断和治疗。