Caruana Maryanne, Grech Victor
Department of Cardiology, Mater Dei Hospital, Msida, MSD 2090, Malta.
Department of Pediatrics, Mater Dei Hospital, Msida, MSD 2090, Malta.
Congenit Heart Dis. 2017 May;12(3):301-308. doi: 10.1111/chd.12439. Epub 2016 Nov 28.
To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan-Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95-51.21) years).
Patients born after 1985 were operated at a younger age (median 1.28 years) compared with patients born before 1985 (median 9.64 years) (P < .001). Transannular patch repair was the commonest operation among patients born after 1985 (43.90%), while repair without transannular patch use prevailed among those born before 1985 (66.13%). 90.24% of patients born after 1985 survived reparative surgery compared with 70.37% of those born before 1985. Of the 75 repair survivors, 7 (9.33%) died of cardiac causes and 22 (29.33%) needed reintervention during follow-up. Overall estimated mean survival was 45.56 years (95% CI 41.67-49.24) with estimated survival rate of 77.5% at 40 years from repair. Estimated mean reintervention-free survival was 37.71 years (95% CI 33.75-41.66) with estimated reintervention-free survival rate of 59.2% at 40 years. Patients with genetic syndromes had significantly lower overall survival after repair. Transannular patch repair was associated with significantly lower reintervention-free survival (median 32.37 years (95% CI 12.75-51.99)) compared with repair without transannular patch [median 44.21 years (95% CI 43.06-45.35); P = .03].
Although survival after tetralogy of Fallot repair in contemporary patients is very good, cardiac death can occur at any stage and structural reintervention is common. Regular follow-up with imaging and rhythm monitoring remains of utmost importance in all patients.
确定法洛四联症修复术后患者的总体生存率和无再次干预生存率，并研究性别、修复时年龄、遗传综合征、既往姑息治疗以及修复类型对这些结局的潜在影响。
提取本地数据库中1997年底前出生的所有130例法洛四联症患者。对103/130例接受修复手术的患者分析手术修复类型、修复时年龄和手术生存率。对75例有完整随访数据的修复术后存活者（平均随访26.37±9.27（范围9.95 - 51.21）年）进行Kaplan-Meier生存分析。
1985年后出生的患者修复手术时年龄较年轻（中位数1.28岁），而1985年前出生的患者为（中位数9.64岁）（P <.001）。跨环补片修复是1985年后出生患者中最常见的手术方式（43.90%），而未使用跨环补片的修复在1985年前出生的患者中占主导（66.13%）。1985年后出生的患者90.24%在修复手术后存活，而1985年前出生的患者为70.37%。在75例修复术后存活者中，7例（9.33%）死于心脏原因，22例（29.33%）在随访期间需要再次干预。总体估计平均生存时间为45.56年（95%可信区间41.67 - 49.24），修复后40年的估计生存率为77.5%。估计平均无再次干预生存时间为37.71年（95%可信区间33.75 - 41.66），修复后40年的估计无再次干预生存率为59.2%。患有遗传综合征的患者修复术后总体生存率显著较低。与未使用跨环补片的修复相比，跨环补片修复的无再次干预生存率显著降低（中位数32.37年（95%可信区间12.75 - 51.99））[中位数44.21年（95%可信区间43.06 - 45.35）；P = 0.03]。
尽管当代法洛四联症修复术后患者的生存率非常高，但心脏死亡可能在任何阶段发生，且结构再次干预很常见。对所有患者进行定期的影像学和心律监测随访仍然至关重要。
