1型强直性肌营养不良患者右心腔广泛的电解剖改变

Widespread electroanatomic alterations of right cardiac chambers in patients with myotonic dystrophy type 1.

作者信息

Dello Russo Antonio, Pelargonio Gemma, Parisi Quintino, Santamaria Matteo, Messano Loredana, Sanna Tommaso, Casella Michela, De Martino Giuseppe, De Ponti Roberto, Pace Manuela, Giglio Vincenzo, Ierardi Carolina, Zecchi Paolo, Crea Filippo, Bellocci Fulvio

机构信息

Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy.

出版信息

J Cardiovasc Electrophysiol. 2006 Jan;17(1):34-40. doi: 10.1111/j.1540-8167.2005.00277.x.

DOI:10.1111/j.1540-8167.2005.00277.x

PMID:16426397

Abstract

INTRODUCTION

Conduction disturbances and arrhythmias characterize the cardiac feature of myotonic dystrophy type 1 (MD1); a myocardial involvement has been suggested as part of the cardiac disease. The aim of our study was to investigate the underlying myocardial alterations using electroanatomic mapping (CARTO) and their possible correlation with genetic and neurological findings.

METHODS AND RESULTS

Right atrial and ventricular CARTO maps were obtained in 13 MD1 patients. Thirteen age-matched patients with paroxysmal supraventricular tachycardia and normal heart served as controls. Unipolar voltage (UNI-v), bipolar voltage (BI-v) amplitudes, bipolar potential duration (Bi-dur), and atrial propagation time (A-pt) were measured. UNI-v and BI-v in interatrial septum, anterolateral atrial wall, and right ventricle outflow tract were lower in MD1 patients than controls (P < 0.001). Bi-dur and A-pt were longer in MD1 patients than controls (P < 0.001, P = 0.046, respectively). A significant relationship was documented between CTG triplets and the percentage of Bi-v <0.5 mV in the atrial anteroseptal region (r = 0.6, P = 0.02).

CONCLUSIONS

Altered electroanatomic patterns are present in the right cardiac chambers in MD1 patients. Widespread myocardial alterations, not necessarily limited to the conduction system, may support the presence of a cardiac myopathy as part of the disease.

摘要

引言

传导障碍和心律失常是1型强直性肌营养不良（MD1）的心脏特征；心肌受累被认为是心脏病的一部分。我们研究的目的是使用电解剖标测（CARTO）来研究潜在的心肌改变及其与遗传和神经学发现的可能相关性。

方法与结果

对13例MD1患者进行了右心房和心室的CARTO标测。13例年龄匹配的阵发性室上性心动过速且心脏正常的患者作为对照。测量了单极电压（UNI-v）、双极电压（BI-v）幅度、双极电位持续时间（Bi-dur）和心房传导时间（A-pt）。MD1患者房间隔、心房前外侧壁和右心室流出道的UNI-v和BI-v低于对照组（P < 0.001）。MD1患者的Bi-dur和A-pt比对照组更长（分别为P < 0.001，P = 0.046）。CTG三联体与心房前间隔区域Bi-v < 0.5 mV的百分比之间存在显著关系（r = 0.6，P = 0.02）。