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通过组织多普勒早期检测强直性肌营养不良中的双心室受累情况。

Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler.

作者信息

Parisi Michele, Galderisi Maurizio, Sidiropulos Milena, Fiorillo Chiara, Lanzillo Roberta, D'Errico Arcangelo, Grieco Michela, Innelli Pasquale, Santoro Lucio, de Divitiis Oreste

机构信息

Divisione di Cardioangiologia con UTIC, Dipartimento di Medicina e Clinica Sperimentale, Italy.

出版信息

Int J Cardiol. 2007 May 31;118(2):227-32. doi: 10.1016/j.ijcard.2006.06.056. Epub 2006 Oct 12.

Abstract

BACKGROUND

Myotonic dystrophy is associated with arrhythmias and risk of sudden death but also with symptoms of heart failure. Our study aimed to identify early biventricular dysfunction in asymptomatic patients with myotonic dystrophy by tissue Doppler.

METHODS

Thirty-six patients with myotonic dystrophy (M/F=20/16, mean age=36.4 years), asymptomatic for heart failure, and 36 age- and sex-matched healthy controls underwent Doppler echocardiography and pulsed tissue Doppler of lateral mitral annulus and of tricuspid annulus.

RESULTS

The two groups had similar body mass index, blood pressure, heart rate, cardiac mass and endocardial shortening. Standard Doppler showed significantly lower transmitral early (E) diastolic peak velocity, longer transmitral deceleration and isovolumic relaxation times and higher tricuspid inflow atrial peak velocity in myotonic dystrophy than in controls. Tissue Doppler of mitral annulus showed lower myocardial systolic velocity (p<0.02), lower early diastolic velocity (E(m)) (p<0.05) and atrial velocity (A(m)) (p<0.005), but no difference of E(m)/A(m) ratio. At tricuspid annulus, E(m) and E(m)/A(m) ratio were lower (p<0.02 and p<0.005, respectively). The ratio between tricuspid inflow E velocity and E(m), index of the degree of right ventricular filling pressure, was higher (p<0.001) than in controls. Tissue Doppler derived left ventricular and right ventricular measurements were all associated with the disease condition, independent of age and heart rate.

CONCLUSIONS

Tissue Doppler identifies subclinical biventricular involvement in myotonic dystrophy. Early left ventricular myocardial systolic and diastolic changes are evident. Right ventricular dysfunction, involving myocardial relaxation and right ventricular filling pressure, might be the arrhythmogenic substratum of these patients.

摘要

背景

强直性肌营养不良与心律失常及猝死风险相关,也与心力衰竭症状有关。我们的研究旨在通过组织多普勒成像识别无症状强直性肌营养不良患者的早期双心室功能障碍。

方法

36例强直性肌营养不良患者(男/女=20/16,平均年龄=36.4岁),无心力衰竭症状,以及36例年龄和性别匹配的健康对照者接受了多普勒超声心动图检查以及二尖瓣环和三尖瓣环的脉冲组织多普勒检查。

结果

两组的体重指数、血压、心率、心脏质量和心内膜缩短情况相似。标准多普勒检查显示,强直性肌营养不良患者的二尖瓣舒张早期(E)峰值流速显著降低,二尖瓣减速时间和等容舒张时间延长,三尖瓣流入心房峰值流速高于对照组。二尖瓣环的组织多普勒检查显示心肌收缩速度较低(p<0.02),舒张早期速度(E(m))较低(p<0.05),心房速度(A(m))较低(p<0.005),但E(m)/A(m)比值无差异。在三尖瓣环处,E(m)和E(m)/A(m)比值较低(分别为p<0.02和p<0.005)。三尖瓣流入E速度与E(m)的比值(右心室充盈压程度指标)高于对照组(p<0.001)。组织多普勒得出的左心室和右心室测量值均与疾病状况相关,独立于年龄和心率。

结论

组织多普勒成像可识别强直性肌营养不良患者亚临床双心室受累情况。左心室心肌早期收缩和舒张变化明显。右心室功能障碍,涉及心肌松弛和右心室充盈压,可能是这些患者心律失常的基础。

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