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肥厚型心肌病的表型表达与心脏磁共振延迟钆增强

Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance.

作者信息

Caetano Francisca, Botelho Ana, Trigo Joana, Silva Joana, Almeida Inês, Venâncio Margarida, Pais João, Sanches Conceição, Leitão Marques António

机构信息

Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

出版信息

Rev Port Cardiol. 2014 May;33(5):261-7. doi: 10.1016/j.repc.2013.10.005. Epub 2014 Jun 6.

Abstract

INTRODUCTION AND AIM

The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients.

METHODS

From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed.

RESULTS

Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality.

CONCLUSIONS

The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events.

摘要

引言与目的

钆延迟增强(LGE)对肥厚型心肌病(HCM)患者进行危险分层的预后价值存在争议。我们旨在研究HCM患者的临床和形态学变量、心源性猝死危险因素与LGE之间的关联。

方法

在78例HCM患者中,我们对53例接受心脏磁共振检查的患者进行了研究。根据是否存在LGE将他们分为两组。分析随访期间的室性心律失常以及发病率和死亡率。

结果

LGE患者诊断时年龄较小(p = 0.046),更常具有心源性猝死家族史(p = 0.008)和已知冠状动脉疾病(p = 0.086)。超声心动图显示,他们有更大的最大壁厚(p = 0.007)、左心房面积(p = 0.037)和容积(p = 0.035),更常表现为舒张功能障碍的限制性模式(p = 0.011),E/A比值更高(p = 0.003)以及左心室收缩功能障碍(p = 0.038)。心脏磁共振支持LGE与先前超声心动图结果之间的关联:更大的左心房面积(p = 0.029)、最大壁厚(p < 0.001)以及更低的左心室射血分数(p = 0.056)。LGE患者更常植入植入式心律转复除颤器(ICD)(p = 0.015)。随访期间,室性心律失常的频率、ICD的适当治疗或死亡率均未发现差异。

结论

LGE的存在成为一种风险标志物,与该人群心源性猝死的经典预测因素相关。然而,需要更大规模的研究来证实其与临床事件的独立关联。

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