关键词: Fontan palliation cardiac magnetic resonance imaging congenital heart disease torsion ventricular mechanics

来  源:   DOI:10.1016/j.jacadv.2023.100360   PDF(Pubmed)

Abstract:
UNASSIGNED: Abnormal left ventricular (LV) rotational mechanics in biventricular hearts are associated with adverse outcomes; however, these are less well characterized for hearts with functionally single ventricles.
UNASSIGNED: The purpose of this study was to characterize ventricular rotational mechanics in the Fontan circulation and their relationship to outcomes.
UNASSIGNED: Single-center, retrospective analysis of magnetic resonance examinations for 329 Fontan patients (15 [IQR: 10-21] years) and 42 controls. The ventricular cine short-axis stack was analyzed to derive torsion metrics. Torsion calculated as the difference between apical and basal rotation normalized to ventricular length.
UNASSIGNED: Fontan patients had higher indexed ventricular end-diastolic volume (97 mL/body surface area1.3 vs 72 mL/body surface area1.3), lower ejection fraction (53% vs 60%), and lower proportion of basal clockwise rotation (62% vs 93%), apical counterclockwise rotation (77% vs 95%), and positive torsion (82% vs 100%); P < 0.001 for all. A composite outcome of death or heart transplant-listing occurred in 31 (9%) patients at a median follow-up of 3.9 years. Torsion metrics were associated with the outcome; although, on multivariate analysis only right ventricular (RV) morphology and indexed ventricular end-diastolic volume were independently associated. LVs with negative torsion, and RVs regardless of torsional pattern, had worse outcomes compared to LVs with positive torsion (P = 0.020).
UNASSIGNED: Single ventricles in a Fontan circulation exhibit abnormal torsional mechanics, which are more pronounced for RV morphology. Abnormal torsion is associated with death or need for heart transplantation. Fontan patients with LV morphology and preserved torsion exhibit the highest transplant-free survival and torsion may offer incremental prognostic data in this group of patients.
摘要:
双心室心脏的左心室(LV)旋转力学异常与不良后果有关;然而,对于具有功能单心室的心脏,这些特征较差。
本研究的目的是表征Fontan循环中的心室旋转力学及其与结果的关系。
单中心,对329例Fontan患者(15[IQR:10-21]年)和42例对照者进行磁共振检查的回顾性分析.分析了心室电影短轴叠加以得出扭转指标。扭转计算为根尖和基底旋转之间的差异,标准化为心室长度。
Fontan患者的心室舒张末期容积指数较高(97mL/体表面积1.3vs72mL/体表面积1.3),降低射血分数(53%vs60%),基础顺时针旋转的比例较低(62%对93%),顶端逆时针旋转(77%对95%),和正扭转(82%vs100%);所有P<0.001。在中位3.9年的随访时间内,31例(9%)患者出现死亡或心脏移植的复合结局。扭转指标与结果相关;尽管,在多变量分析中,仅右心室(RV)形态和心室舒张末期容积指数是独立相关的.负扭转的LV,和RV,无论扭转模式如何,与具有正扭转的LV相比,结果更差(P=0.020)。
Fontan循环中的单个心室表现出异常的扭转力学,对于RV形态更明显。异常扭转与死亡或需要心脏移植有关。具有LV形态和保留的扭转的Fontan患者表现出最高的无移植存活率,并且扭转可能在该组患者中提供增量的预后数据。
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