Zeng Qi-Xian, Liu Zhihong, Xi Qun-Ying, Xiong Changming
State Key Laboratory of Cardiovascular Disease, Center of Pulmonary Vascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Center for Pulmonary Vascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences, Shenzhen, China.
Eur Heart J Case Rep. 2022 Mar 22;6(4):ytac127. doi: 10.1093/ehjcr/ytac127. eCollection 2022 Apr.
Patent ductus arteriosus (PDA) can close on its own during childhood. Patent ductus arteriosus with left pulmonary artery (LPA) occlusion is rare. Here, we describe possible aetiologies of this condition and treatment strategies based on recent guidelines.
A 35-year-old man experienced shortness of breath for 20 years. Physical examination revealed pitting oedema, digital clubbing, and bi-phasic murmur along the left sternal border at the 2nd and 3rd intercostal space. Congenital heart disease was suspected. Echocardiography revealed a severely dilated pulmonary trunk and PDA; however, the LPA was not visible. The patient has undergone PDA ligation surgery >30 years ago, which may have caused accidental LPA ligation; however, extreme elevation of pulmonary pressure led to increased difficulties in performing LPA reconstruction and PDA division. Therefore, pulmonary arterial hypertension (PAH) initial combination therapy with parenteral prostanoids was prescribed. The patient's condition improved gradually.
Inadvertent ligation of the LPA instead of PDA is a rare and usually fatal complication during ductal ligation. Patients who survive this catastrophic complication usually develop the progressive pulmonary vascular disease with increased pulmonary pressure and impaired lung parenchyma resulting in right heart and respiratory failure. Early and regular follow-up with cardiac imaging studies is important to diagnose this complication. Reconstruction of the ligated LPA and PDA late in the disease course is difficult due to the development of pulmonary arterial hypertension. Initial PAH combination therapy may be valuable for relieving the patients' symptoms at that stage. Heart and lung transplantation can also be considered in suitable patients.
动脉导管未闭（PDA）在儿童期可自行闭合。合并左肺动脉（LPA）闭塞的动脉导管未闭较为罕见。在此，我们根据近期指南描述该病症可能的病因及治疗策略。
一名35岁男性有20年呼吸急促病史。体格检查发现有凹陷性水肿、杵状指，以及在胸骨左缘第2和第3肋间有双期杂音。怀疑患有先天性心脏病。超声心动图显示肺动脉主干严重扩张及PDA；然而，未见到LPA。该患者30多年前接受过PDA结扎手术，可能意外结扎了LPA；然而，肺动脉压力极度升高导致LPA重建和PDA分离的难度增加。因此，给予了肺动脉高压（PAH）初始联合治疗，采用肠外前列腺素类药物。患者病情逐渐好转。
在导管结扎过程中意外结扎LPA而非PDA是一种罕见且通常致命的并发症。在这种灾难性并发症中存活下来的患者通常会发展为进行性肺血管疾病，肺动脉压力升高，肺实质受损，导致右心和呼吸衰竭。早期并定期进行心脏影像学检查随访对于诊断该并发症很重要。由于肺动脉高压的发展，在疾病后期重建结扎的LPA和PDA很困难。初始PAH联合治疗在该阶段可能对缓解患者症状有价值。对于合适的患者也可考虑心肺移植。
