在患有唐氏综合征的患者中完整修复心房支柱缺陷,超过六个月-一个单一的中心体验
Kartik Patel1, Chandrasekaran Ananthanarayanan1, Trushar Gajjar1
1Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Centre, Ahmedabad, India.
World journal for pediatric & congenital heart surgery
|April 3, 2025
概括
在6个月以上的唐氏综合征患者中,完全修复心房隔膜缺陷是可行的. 尽管存在早期死亡风险,但长期存活率为85%,仔细监测门问题至关重要.
科学领域:
- 心脏病学 心脏病学
- 儿科手术 儿科手术
- 遗传学 遗传学 是一个
背景情况:
- 完整心房隔膜缺陷 (cAVSD) 是唐氏综合征 (DS) 中最常见的心脏缺陷.
- 建议在6个月前进行早期的手术修复,以预防肺动脉高血压 (PAH).
- 延迟修复在发展中国家很常见,需要对结果进行分析.
研究的目的:
- 为了回顾性地分析cAVSD的双心室修复结果,在六个月后出现的DS患者中.
- 在这个队列中确定死亡率和重新手术的风险因素.
- 评估治疗后的长期存活率和临床状态.
主要方法:
- 在6个月后对60名患有cAVSD的DS患者进行了回顾性审查,这些患者经历了双心室修复 (2015-2019).
- 与42名在6个月前修复的DS患者进行了比较.
- 分析实验室参数,心声回声数据,临床发现和随访结果.
主要成果:
- 修复时的中位数年龄为8个月;11.7%的早期死亡率 (n=7) 由于VR功能障碍/PAH和败血症.
- 死亡的独立风险因素包括右AVVR等级,术后胆红素和肌素.
- 平均随访时间为1658天,1,5年和10年生存率为85%;晚期重新手术率为5.6%.
结论:
- 在晚期呈现的DS患者中,手术修复cAVSD是可行的,具有良好的长期结果.
- 密切跟踪至关重要,以监测可能晚期发展的右心室回 (AVVR).
- 在长期随访时,患者仍然无症状,没有残留严重的PAH.
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