Abstract

Background

Heart transplantation (HTx) in adults with congenital heart disease (ACHD) remains challenging because of structural anomalies and often previous procedure. The aim of this retrospective study was to describe the outcomes of heart transplantation (HTx) in a cohort of ACHD patients at our tertiary centre.

Patients and methods

Between January 1993 and December 2010, 223 consecutive adult patients (age > 18 years) underwent HTx at our institution. Fifteen (6.7%) were ACHD patients. Outcomes were reviewed using our institution’s HTx database. We looked at 30-day, 1, 5 and 10-years survival, as well as post-transplantation complications.

Results

The mean age at HTx of the groups of ACHD was 42 ± 14.4 years, vs 54.2 ± 9.8 years for the non-CHD patients. Prior to transplant, thirteen of the fifteen ACHD had undergone one or more surgical procedures including palliative or corrective open-heart procedures in 66.6% of them. Seven of the fifteen ACHD (47%) required additional surgical procedures at transplantation. The mean follow-up was 95,44 ± 84.3 months. There was no significant difference in survival (ACHD vs non-CHD) at 30 days (87% vs. 90%), 1 year (73% vs. 74.5%) or 5 years (53% vs. 55%). Survival at 10 years was respectively 53% and 41% for ACHD patients and non-CHD patients.

Conclusion

Despite the surgical challenge, HTx in ACHD has a good long-term result. However, the small sample size of our cohort limits any definitive conclusions.

中文翻译：

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先天性心脏病成人心脏移植：17 年单中心经验

摘要

背景

由于结构异常和通常之前的手术，患有先天性心脏病 (ACHD) 的成人的心脏移植 (HTx) 仍然具有挑战性。这项回顾性研究的目的是描述我们三级中心 ACHD 患者队列心脏移植 (HTx) 的结果。

患者和方法

1993 年 1 月至 2010 年 12 月期间，连续 223 名成年患者（年龄 > 18 岁）在我们机构接受了 HTx。15 名 (6.7%) 是 ACHD 患者。使用我们机构的 HTx 数据库对结果进行了审查。我们研究了 30 天、1 年、5 年和 10 年的存活率，以及移植后并发症。

结果

ACHD 组的 HTx 平均年龄为 42 ± 14.4 岁，而非 CHD 患者为 54.2 ± 9.8 岁。在移植之前，15 名 ACHD 患者中有 13 名接受了一次或多次外科手术，其中 66.6% 的患者接受了姑息性或矫正性心脏直视手术。15 例 ACHD 中有 7 例 (47%) 在移植时需要额外的外科手术。平均随访时间为 95.44 ± 84.3 个月。在 30 天（87% 对 90%）、1 年（73% 对 74.5%）或 5 年（53% 对 55%）时，存活率（ACHD 对非 CHD）没有显着差异。ACHD 患者和非 CHD 患者的 10 年生存率分别为 53% 和 41%。

结论

尽管面临手术挑战，但 HTx 治疗 ACHD 具有良好的长期效果。然而，我们队列的小样本量限制了任何明确的结论。