Clinical Operational Research Unit, University College London, London, UK.
Charles West division, Great Ormond Street Hospital NHS Foundation Trust, London, UK.
Heart. 2018 Sep;104(18):1500-1507. doi: 10.1136/heartjnl-2017-312448. Epub 2018 Jan 25.
To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.
The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.
There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive.
Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.
描述在英格兰和威尔士被诊断为左心发育不全综合征（HLHS）的患者的长期结果、治疗途径和风险因素。
英国国家审计数据库记录了为先天性心脏病患者进行的每一次手术以及在英格兰和威尔士居住的患者的最新生存状况。使用国际儿科和先天性心脏病代码，识别出 2000 年至 2015 年期间出生的 HLHS 患者。
共有 976 例 HLHS 患者。其中，9.6%的患者接受了预途径干预，89.5%的患者接受了传统的分阶段姑息治疗途径，6.4%的婴儿接受了杂交途径。接受预途径手术或杂交途径的患者更为复杂，表现出更高的早产率和获得性合并症发生率。预途径干预与最高的院内死亡率（34.0%）相关。44.6%的患者在初次手术后进行了非途径手术，最常见的是支架置入术或残余狭窄或再狭窄的扩张，最常见于第 1 阶段和第 2 阶段之间。1 年和 5 年的生存率分别为 60.7%（95%CI 57.5%至 63.7%）和 56.3%（95%CI 53.0%至 59.5%）。有产前诊断（多变量 HR（MHR）1.63（95%CI 1.12 至 2.38））、低体重（<2.5kg）（MHR 1.49（95%CI 1.05 至 2.11））或获得性合并症（MHR 2.04（95%CI 1.30 至 3.19））的患者更不可能存活。
HLHS 患者的治疗途径复杂且多样化。对于像 HLHS 这样需要连续干预的疾病的长期结果进行研究至关重要，以便提供更全面的情况，并为质量保证和改进提供信息。
