Department of Paediatric Cardio-Thoracic Surgery, German Pediatric Heart Center ('Deutsches Kinderherzzentrum'), Asklepios Clinic, Sankt Augustin, Germany.
Eur J Cardiothorac Surg. 2013 Oct;44(4):648-54. doi: 10.1093/ejcts/ezt124. Epub 2013 Mar 12.
In symptomatic patients, performing a primary repair of tetralogy of Fallot (TOF), irrespective of age or placing a shunt, remains controversial. The aim of the study was to analyse the policy of primary correction.
Between May 2005 and May 2012, a total of 87 consecutive patients with TOF, younger than 6 months of age, underwent primary correction. All patients had one source of pulmonary blood flow, with or without a patent ductus arteriosus. The median age at surgery was 106 ± 52.3 days (8-180 days). Twelve patients (13.8%) were newborns. Two groups were analysed: group I, patients <1 month of age; group II, patients between 2-6 months of age.
There was no early or late death at 7 years of follow-up. There was no difference in bypass time or hospital stay between the two groups, but the Aristotle comprehensive score (P < 0.0001), ICU stay (P = 0.030) and the length of ventilation (P = 0.014) were significantly different. Freedom from reoperation was 87.3 ± 4.3% and freedom from reintervention was 85.9 ± 4.2% at 7 years, with no difference between the two groups. Neurological development was normal in all patients, but 1 patient in Group II had cerebral seizures and showed developmental delay. Growth was adequate in all patients, except those with additional severe non-cardiac malformations that caused developmental delay. Eighty-five per cent of the patients were without cardiac medication.
Even in symptomatic neonates and infants <6 months of age, primary repair of TOF can be performed safely and effectively. One hundred per cent survival at 7 years suggests that early primary repair causes no increase in mortality in the modern era. Shunting is not necessary, even in symptomatic newborns, thus avoiding the risk of shunt-related complications and repeated hospital stays associated with a staged approach.
在有症状的患者中，无论年龄大小，是否放置分流管，进行法洛四联症（TOF）的一期修复仍然存在争议。本研究的目的是分析一期矫正的策略。
2005 年 5 月至 2012 年 5 月，87 例年龄小于 6 个月的连续 TOF 患者接受了一期矫正。所有患者均有一条肺血来源，有或没有动脉导管未闭。手术时的中位年龄为 106±52.3 天（8-180 天）。12 例（13.8%）为新生儿。分析了两组：I 组，<1 个月龄患者；II 组，2-6 个月龄患者。
7 年随访无早期或晚期死亡。两组间体外循环时间和住院时间无差异，但 Aristotle 综合评分（P<0.0001）、ICU 住院时间（P=0.030）和通气时间（P=0.014）差异有统计学意义。7 年无再次手术率为 87.3±4.3%，无再次介入率为 85.9±4.2%，两组间无差异。所有患者神经发育正常，但 II 组 1 例患者出现脑性惊厥，表现为发育迟缓。所有患者生长发育良好，除伴有严重非心脏畸形导致发育迟缓的患者外。85%的患者无需心脏药物治疗。
即使在有症状的新生儿和<6 个月的婴儿中，也可以安全有效地进行 TOF 的一期修复。7 年时 100%的生存率表明，在现代，早期一期修复不会增加死亡率。即使在有症状的新生儿中也不需要分流，从而避免了分流相关并发症和与分期治疗相关的重复住院的风险。
