Zhao Junfei, Si Ming-Sing, Wang Xiaoyan, Chen Siyao, Yan Qian, Chen Jimei
Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, P.R. China.
Department of Cardiac Surgery, University of Michigan, Ann Arbor, Michigan.
J Card Surg. 2020 Feb;35(2):437-440. doi: 10.1111/jocs.14322. Epub 2019 Nov 6.
Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital cardiac anomaly characterized by progressive pulmonary arterial hypertension (PAH) and pulmonary vascular obstructive disease (PVOD) in early childhood. This condition is assumed to be inoperable in older children and adults. However, pulmonary vascular resistance of the isolated lung is difficult to measure by catheterization due to its unique anatomical features, and thus we have used lung biopsy as an alternative method to evaluate candidacy for repair. Here, we report our experience with two patients whose lung biopsy demonstrated reversible PVOD, leading us to perform a surgical repair.
一条肺动脉起源于升主动脉的异常是一种罕见的先天性心脏异常，其特征为在儿童早期出现进行性肺动脉高压（PAH）和肺血管阻塞性疾病（PVOD）。这种情况在大龄儿童和成人中被认为无法手术治疗。然而，由于其独特的解剖特征，通过导管插入术很难测量孤立肺的肺血管阻力，因此我们采用肺活检作为评估修复手术候选资格的替代方法。在此，我们报告两例患者的经验，他们的肺活检显示PVOD可逆，这使我们得以进行手术修复。
