孤立的左心室上角缺血症:迄今为止报告的37例病例的系统审查和分析
Pier Paolo Bassareo1, Sophie Duignan2, Adam James2
1University College of Dublin, School of Medicine and Department of Cardiology at Mater Misericordiae University Hospital, Dublin D07 R2WY, Ireland. piercard@inwind.it.
World journal of clinical cases
|August 28, 2023
概括
孤立左心室上垂体缺血症 (ILVAH) 是一种罕见的心肌病,更常影响男性. 虽然经常出现呼吸困难,但大多数患者都有良好的结果,尽管过程可能是不可预测的.
科学领域:
- 心脏病学和心血管疾病.
- 罕见的遗传疾病 罕见的遗传疾病
- 遗传性心脏异常 遗传性心脏异常
背景情况:
- 隔离左心室上缺血症 (ILVAH),或截断左心室 (LV),是一种非常罕见的心肌病.
- 它的特点是没有顶点的球形LV,右心室占据了这个空间.
- 由于它的稀有性,仅存在有限的病例报告和系列.
研究的目的:
- 综合分析全球所有报告的ILVAH病例.
- 了解这种罕见疾病的人口,临床和诊断特征.
主要方法:
- 对PubMed和Scopus数据库的系统文献审查.
- 数据收集截至2022年12月13日,包括37起报告的病例.
主要成果:
- 大多数病例 (52.7%) 是男性,诊断时平均年龄为26.1岁.
- 常见的症状包括喘息 (40.5%);T波异常和右轴偏差是常见的心电图发现.
- 减少排泄分数 (56.7%) 和相关的先天性心脏病 (16.2%) 被注意到;大多数患者经历了良好的结果.
结论:
- ILVAH是一种复杂的疾病,具有可变的临床轨迹.
- 疾病的发展过程是不可预测的,从良性表现到青春期的突然死亡.
- 需要进一步的研究,以更好地了解和管理ILVAH.
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