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法洛四联症患者区域性心功能的异质性及其与心室形态的关系。

Heterogeneity of regional function and relation to ventricular morphology in patients with fontan circulation.

机构信息

Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Department of Congenital Heart Disease, German Heart Institute, Berlin, Germany.

出版信息

Am J Cardiol. 2013 Oct 15;112(8):1207-13. doi: 10.1016/j.amjcard.2013.06.016. Epub 2013 Aug 5.

DOI:10.1016/j.amjcard.2013.06.016
PMID:23927787
Abstract

The relation between underlying ventricular morphology and regional function in patients with Fontan circulation remains unclear. The aim of this study was to compare regional function and its heterogeneity in patients with tricuspid atresia (TA), biventricular apex-forming morphology (BiV), and controls. Nineteen patients (median age 12 years) with Fontan circulation who presented consecutively were prospectively enrolled and compared with age- and heart rate-matched controls. Most patients were in New York Heart Association class I (63%). Longitudinal systolic strain (S), systolic strain rate (SRsys), and early diastolic strain rate (SRdia) peaks were obtained from 6 ventricular segments, and a coefficient of variation by segment was calculated as a measure of regional heterogeneity. Systolic S, SRsys and SRdia peaks were decreased at the right and left lateral walls in both patient groups compared with controls (p ≤0.001 for all). Patients with TA had higher systolic S and SRsys in the middle of the right lateral wall than those with BiV morphology (p = 0.009 and p = 0.001, respectively). The mean coefficients of variation assessed by S and SRsys were similar in controls and patients with TA but lower in those with BiV than in controls and patients with TA (p <0.001 and p = 0.01, respectively). The mean coefficient of variation assessed by SRdia was greater only in patients with BiV than in controls (p = 0.001). In conclusion, patients with Fontan circulation have more heterogeneous systolic and early diastolic regional function than healthy control subjects, and patients with TA have better systolic regional function in the middle of the right lateral wall and less systolic heterogeneity than patients with BiV morphology.

摘要

在 Fontan 循环患者中,潜在心室形态与局部功能之间的关系尚不清楚。本研究旨在比较三尖瓣闭锁(TA)、双心室心尖形成形态(BiV)和对照组患者的局部功能及其异质性。连续入选了 19 例 Fontan 循环患者,并与年龄和心率匹配的对照组进行了前瞻性比较。大多数患者心功能分级为纽约心脏协会(NYHA)Ⅰ级(63%)。从 6 个心室节段获得纵向收缩应变(S)、收缩应变率(SRsys)和早期舒张应变率(SRdia)峰值,并计算节段变异系数作为局部异质性的衡量指标。与对照组相比,两组患者的右、左侧壁的收缩 S、SRsys 和 SRdia 峰值均降低(所有 p 值均≤0.001)。与 BiV 形态患者相比,TA 患者右外侧壁中部的收缩 S 和 SRsys 更高(p = 0.009 和 p = 0.001)。通过 S 和 SRsys 评估的平均变异系数在对照组和 TA 患者之间相似,但在 BiV 患者中低于对照组和 TA 患者(均 p <0.001 和 p = 0.01)。仅在 BiV 患者中,通过 SRdia 评估的平均变异系数更大(p = 0.001)。总之,Fontan 循环患者的收缩期和早期舒张期局部功能比健康对照组更具异质性,而 TA 患者的右外侧壁中部收缩期局部功能更好,收缩期异质性较 BiV 形态患者更小。

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