Chiu Shuenn-Nan, Wang Jou-Kou, Chen Hui-Chi, Lin Ming-Tai, Wu En-Ting, Chen Chun-An, Huang Shu-Chien, Chang Chung-I, Chen Yih-Sharng, Chiu Ing-Sh, Chen Chi-Ling, Wu Mei-Hwan
Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
Circ Cardiovasc Qual Outcomes. 2012 Jan;5(1):120-5. doi: 10.1161/CIRCOUTCOMES.111.963603. Epub 2012 Jan 10.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in Taiwan. This study investigates the long-term survival and risks of TOF in an Asian cohort.
This study enrolled 819 consecutive patients with TOF (61.1% male), who received total correction between 1970 and 2002, as participants. Patient medical records were reviewed, and the survival status of those out of contact was confirmed by death records retrieved from the National Health database. The mean (±SD) patient age at cardiac repair was 6.5±7.6 years, and a prior shunt operation was performed in 119 (14.5%) of the patients. At cardiac repair, a transannular patch for right ventricle outlet reconstruction was required in 444 (54.2%) of the patients. After 13,808 patient-years of follow-up, the 30-year survival rate was 90.5%. The annual mortality rate increased from 0.123% in the initial 15 years after repair to 0.395% thereafter (P<0.05). The presence of major aortopulmonary collateral arteries, older operative age, and previous shunt operation are independent risks of late cardiac deaths. Secondary to cardiac mortality, unnatural deaths (accident and suicide) accounted for 27.6% of late deaths, significantly higher compared with that of the general population (odds ratio, 2.18; P=0.028).
In this Asian TOF cohort, except for a late decrease after 15 years, long-term survival after cardiac repair was satisfactory. Although cardiac death was the most common cause of late death, accidents or suicide may also be associated with late mortality, suggesting a potential role for psychosocial support.
法洛四联症（TOF）是台湾最常见的青紫型先天性心脏病。本研究调查了亚洲队列中TOF患者的长期生存情况及风险。
本研究纳入了1970年至2002年间连续接受根治手术的819例TOF患者（男性占61.1%）作为研究对象。回顾患者病历，并通过从国民健康数据库检索到的死亡记录确认失访患者的生存状态。心脏修复时患者的平均（±标准差）年龄为6.5±7.6岁，119例（14.5%）患者曾接受过分流手术。心脏修复时，444例（54.2%）患者需要使用跨环补片进行右心室流出道重建。经过13808患者年的随访，30年生存率为90.5%。年死亡率从修复后的最初15年的0.123%增加到此后的0.395%（P<0.05）。存在主要的主肺动脉侧支血管、手术年龄较大和既往分流手术是晚期心脏死亡的独立危险因素。除心脏原因导致的死亡外，非自然死亡（意外和自杀）占晚期死亡的27.6%，显著高于一般人群（优势比，2.18；P=0.028）。
在这个亚洲TOF队列中，除了15年后生存率有所下降外，心脏修复后的长期生存情况令人满意。虽然心脏死亡是晚期死亡最常见的原因，但意外或自杀也可能与晚期死亡率相关，提示心理社会支持可能发挥潜在作用。
