Sakamoto Takahiko, Nagashima Mitsugi, Umezu Kentarou, Houki Ryogo, Ikarashi Jin, Katagiri Junko, Yamazaki Kenji
Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
Interact Cardiovasc Thorac Surg. 2018 Jul 1;27(1):20-26. doi: 10.1093/icvts/ivy034.
Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity.
A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach.
There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively).
Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.
孤立性完全性肺静脉异位连接（TAPVC）是一种相对罕见的先天性心脏缺陷，而肺静脉梗阻（PVO）与预后不良相关。我们回顾了我院孤立性TAPVC完全矫正术的长期结果，并分析了死亡和发病的危险因素。
1965年至2016年间评估的290例孤立性TAPVC患者分为两组：早期组（n = 151）于1989年前接受手术；晚期组（n = 139）于1990年后接受手术。手术时的平均年龄为10.4±30.2个月（范围0天至23岁），平均体重为5.5±6.0 kg（范围1.6 - 48 kg）。晚期组包括更多心内型TAPVC患者，且更倾向于采用后入路。
有53例院内死亡和16例晚期死亡。28例患者术后出现PVO。平均随访时间为18.2±9.7年（范围2个月至42.4年）。晚期组20年时的精算生存率提高到87.8%。多变量分析显示，早期组患者、体重<2 kg且术后出现PVO的患者死亡率显著增加（分别为P < 0.0001、P = 0.0041、P = 0.0003）。22例患者进行了27次再次手术（PVO修复11例；分期修复4例，其他12例）。PVO修复平均在2.5±1.6个月后进行。20年和30年时再次手术的精算无复发生存率分别为88.8%和83.2%。多变量分析显示，再次手术的风险与混合型TAPVC和术后PVO相关（分别为P = 0.
