马尔芬患者的右心室功能尽管经过多次心脏干预,但仍然稳定
Maks Mihalj1,2, Maria Nucera1, Cyril Ferro1
1Department of Cardiac Surgery, University Hospital Bern, University of Bern, Bern, Switzerland.
概括
在接受心脏手术的马方综合征患者中,右心室功能长期保持稳定. 严重的三管吐和需要修复三管的情况并不常见,即使在重新手术后也是如此.
科学领域:
- 心脏病学 心脏病学
- 心血管外科心血管外科
- 遗传学 是一个遗传学.
背景情况:
- 马方综合征通常表现为心心回和左心室功能障碍.
- 关于马尔凡综合征患者三管吐和右心室功能的数据有限.
研究的目的:
- 为了研究右心室功能的长期变化.
- 为了评估三管的反进展.
- 为了确定马方综合征患者心脏手术后的三管修复的自由性.
主要方法:
- 对1995年至2020年间接受心脏手术的Marfan患者进行了回顾性单中心分析.
- 对右心室功能和三腹吐的纵向心声分析.
- 综合终点:三环状平面缩外流 (TAPSE) ≤16毫米,严重的三反,或三修复.
主要成果:
- 135名患者接受了193次手术;中位数随访时间为8年.
- 综合终点发生在40名患者中,通常是作为复发事件.
- 复合终点的十年累积发病率为22.0%;新发的TAPSE ≤16毫米为9.0%. 三管吐显示每年进展,但在10年后仍然没有临床相关性.
- 精算10年生存率为91.1%.
结论:
- 在有心脏手术和重复手术史的马方综合征患者中,右心室功能保持稳定.
- 严重的三角管吐和需要三角管修复的发生率很低.
- 在这个队列中,对右心室功能和三门的长期结果是有利的.
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