Pan Jun-Yen, Lin Chu-Chuan, Chang Jen-Ping
Department of Cardiovascular Surgery.
Department of Pediatrics, Kaohsiung Veterans General Hospital.
Acta Cardiol Sin. 2016 Sep;32(5):612-615. doi: 10.6515/acs20160105a.
Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging. We reported a 17-year-old girl with a single ventricle and heterotaxy syndrome and only her left lung functioning, who underwent one-lung Fontan operation with a satisfactory result.
评估肺循环状态,包括压力、阻力、大小以及有无解剖结构畸形,对于成功实施Fontan手术至关重要。大多数患者在先前的姑息治疗后被发现肺动脉状况尚可,尽管一定程度的畸形并不罕见。然而,在极少数情况下,一些患者只有一个功能正常的肺,另一条肺动脉严重发育不全或闭锁。对于这些患者,完成Fontan手术将具有挑战性。我们报告了一名17岁患有单心室和内脏异位综合征且仅左肺有功能的女孩,她接受了单肺Fontan手术,结果令人满意。