Bu'Lock F A, Stümper O, Jagtap R, Silove E D, De Giovanni J V, Wright J G, Sethia B, Brawn W J
Heart Unit, Birmingham Children's Hospital.
Br Heart J. 1995 May;73(5):456-61. doi: 10.1136/hrt.73.5.456.
Prospective audit of the first year of implementation of a modified approach to palliation for infants with hypoplastic systemic ventricle and severe systemic outflow obstruction.
Tertiary referral centre for neonatal and infant cardiac surgery.
17 of 19 infants (aged < 35 days) presenting to Birmingham Children's Hospital in 1993 with hypoplastic systemic ventricle and severe outflow obstruction underwent surgery. This was performed using a new modification of the Norwood-type arch repair, without the use of exogenous material, and a 3.5 mm Gore-tex shunt between the innominate and right pulmonary arteries. The Gore-tex shunt was replaced by a cavopulmonary shunt between 3 and 5 months later. Clinical, morphological, and functional determinants of outcome were examined.
10 (59%) infants survived initial surgery. All proceeded to cavopulmonary shunt without further loss. Significant atrioventricular valve regurgitation seemed to be the main risk factor for poor outcome. If this was excluded, the morphology of the dominant ventricle seemed to have little effect on the outcome of initial surgery.
Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.
对采用改良姑息治疗方法治疗肺发育不全性体循环心室和严重体循环流出道梗阻婴儿的第一年进行前瞻性审计。
新生儿和婴儿心脏外科三级转诊中心。
1993年，19名年龄小于35天、患有肺发育不全性体循环心室和严重流出道梗阻的婴儿前往伯明翰儿童医院就诊，其中17名接受了手术。手术采用诺伍德式主动脉弓修复术的新改良方法，不使用外源材料，并在无名动脉和右肺动脉之间植入3.5毫米的戈尔特斯分流管。3至5个月后，将戈尔特斯分流管替换为腔肺分流管。检查了结果的临床、形态学和功能决定因素。
10名（59%）婴儿在初次手术后存活。所有患儿均接受了腔肺分流术，没有进一步的损失。明显的房室瓣反流似乎是预后不良的主要危险因素。如果排除这一因素，优势心室的形态似乎对初次手术的结果影响不大。
在实施针对肺发育不全性体循环心室和严重流出道梗阻婴儿的手术方案的第一年，59%的患者实现了早期存活。不使用外源材料构建新主动脉可能会改善新主动脉后期的生长。早期腔肺分流术可以安全进行，并且应该减少因紫绀和体循环心室容量负荷导致的中期并发症。
