PDF(Pubmed)
Abstract：
UNASSIGNED: Fragmented QRS (f-QRS) complex on the surface ECG is a cardiac conduction abnormality that indicates myocardial scarring. The relationship between the f-QRS complex and cardiac status in patients with Danon disease (DD) remains unclear and will be explored in this study.
UNASSIGNED: Patients with genetically confirmed DD and cardiac magnetic resonance imaging (CMR) examinations were recruited from multiple centers. The number of leads, pattern, score, and segmental distribution of the f-QRS complex were assessed by surface 12-lead ECG. Cardiac status, such as left ventricular (LV) volume, function, and extent of late gadolinium enhancement (LGE), was demonstrated by CMR. The segmental distribution of LGE was also assessed. Correlations between the f-QRS and cardiac status were assessed.
UNASSIGNED: Fifteen patients (14 men) with DD who underwent 12-lead ECG and CMR imaging were included. The f-QRS complex was documented in all patients (n = 15, 100%). Three patterns of f-QRS were found, with the notched R/S pattern (74%) being the most common, followed by fragmented QRS (16%) and various RSR\' (11%). The fragmented QRS pattern showed an association with a higher level of myocardial fibrosis (LGE > 35%). The burden of f-QRS in each patient was assessed by the number of leads with f-QRS (median 7, range 2-12) and the f-QRS score (median 9, range 2-33). In the correlation analysis, the f-QRS score was positively correlated with LGE% (r = 0.726, p = 0.002), negatively correlated with LV ejection function (LVEF; r = -0.617, p = 0.014) as evaluated by CMR. In the local distribution, f-QRS score and LGE% were both predominant in the LV free wall but did not correlate well among the anterior, lateral, and inferior segments.
UNASSIGNED: In this DD cohort, the quantitative f-QRS was correlated well with myocardial fibrosis burden and LV dysfunction in general. This finding suggests that f-QRS can be used as a simple screening tool to assess cardiac status in patients with DD.
UNASSIGNED: Patients with genetically confirmed DD and cardiac magnetic resonance imaging (CMR) examinations were recruited from multiple centers. The number of leads, pattern, score, and segmental distribution of the f-QRS complex were assessed by surface 12-lead ECG. Cardiac status, such as left ventricular (LV) volume, function, and extent of late gadolinium enhancement (LGE), was demonstrated by CMR. The segmental distribution of LGE was also assessed. Correlations between the f-QRS and cardiac status were assessed.
UNASSIGNED: Fifteen patients (14 men) with DD who underwent 12-lead ECG and CMR imaging were included. The f-QRS complex was documented in all patients (n = 15, 100%). Three patterns of f-QRS were found, with the notched R/S pattern (74%) being the most common, followed by fragmented QRS (16%) and various RSR\' (11%). The fragmented QRS pattern showed an association with a higher level of myocardial fibrosis (LGE > 35%). The burden of f-QRS in each patient was assessed by the number of leads with f-QRS (median 7, range 2-12) and the f-QRS score (median 9, range 2-33). In the correlation analysis, the f-QRS score was positively correlated with LGE% (r = 0.726, p = 0.002), negatively correlated with LV ejection function (LVEF; r = -0.617, p = 0.014) as evaluated by CMR. In the local distribution, f-QRS score and LGE% were both predominant in the LV free wall but did not correlate well among the anterior, lateral, and inferior segments.
UNASSIGNED: In this DD cohort, the quantitative f-QRS was correlated well with myocardial fibrosis burden and LV dysfunction in general. This finding suggests that f-QRS can be used as a simple screening tool to assess cardiac status in patients with DD.
摘要：
未经证实：体表心电图上的碎裂QRS波（f-QRS波）是一种心脏传导异常，表明心肌瘢痕形成。Danon病（DD）患者的f-QRS波与心脏状态之间的关系尚不清楚，将在本研究中进行探讨。
UNASSIGNED：从多个中心招募了基因证实的DD和心脏磁共振成像（CMR）检查的患者。线索的数量，模式，得分,通过表面12导联心电图评估f-QRS波的节段分布。心脏状态,如左心室（LV）容积，函数,以及钆晚期增强(LGE)的程度，由CMR证明。还评估了LGE的节段分布。评估f-QRS与心脏状态之间的相关性。
UNASSIGNED：15例（14例男性）DD患者接受了12导联ECG和CMR成像。所有患者均记录了f-QRS波（n=15，100％）。发现了三种f-QRS模式，缺口R/S模式(74%)是最常见的，其次是零散的QRS（16%）和各种RSR'（11%）。碎裂QRS图显示与较高水平的心肌纤维化相关(LGE>35%)。通过具有f-QRS的导联数量（中位数7，范围2-12）和f-QRS评分（中位数9，范围2-33）评估每位患者的f-QRS负担。在相关性分析中，f-QRS评分与LGE%呈正相关(r=0.726,p=0.002),通过CMR评估，与LV射血功能呈负相关（LVEF；r=-0.617，p=0.014）。在本地分布中，f-QRS评分和LGE%均在左心室游离壁中占主导地位，但在前段之间没有很好的相关性。横向,和劣势。
未经评估：在此DD队列中，定量f-QRS与心肌纤维化负荷和左心室功能障碍相关。这一发现表明f-QRS可以用作评估DD患者心脏状态的简单筛查工具。
UNASSIGNED：从多个中心招募了基因证实的DD和心脏磁共振成像（CMR）检查的患者。线索的数量，模式，得分,通过表面12导联心电图评估f-QRS波的节段分布。心脏状态,如左心室（LV）容积，函数,以及钆晚期增强(LGE)的程度，由CMR证明。还评估了LGE的节段分布。评估f-QRS与心脏状态之间的相关性。
UNASSIGNED：15例（14例男性）DD患者接受了12导联ECG和CMR成像。所有患者均记录了f-QRS波（n=15，100％）。发现了三种f-QRS模式，缺口R/S模式(74%)是最常见的，其次是零散的QRS（16%）和各种RSR'（11%）。碎裂QRS图显示与较高水平的心肌纤维化相关(LGE>35%)。通过具有f-QRS的导联数量（中位数7，范围2-12）和f-QRS评分（中位数9，范围2-33）评估每位患者的f-QRS负担。在相关性分析中，f-QRS评分与LGE%呈正相关(r=0.726,p=0.002),通过CMR评估，与LV射血功能呈负相关（LVEF；r=-0.617，p=0.014）。在本地分布中，f-QRS评分和LGE%均在左心室游离壁中占主导地位，但在前段之间没有很好的相关性。横向,和劣势。
未经评估：在此DD队列中，定量f-QRS与心肌纤维化负荷和左心室功能障碍相关。这一发现表明f-QRS可以用作评估DD患者心脏状态的简单筛查工具。
