Prinz Christian, Hering Detlef, Bitter Thomas, Horstkotte Dieter, Faber Lothar
Department of Cardiology, Heart and Diabetes Centre North-Rhine Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany.
Acta Cardiol. 2011 Apr;66(2):153-7. doi: 10.1080/ac.66.2.2071245.
In hypertrophic cardiomyopathy (HCM) risk assessment with respect to sudden cardiac death (SCD) is currently based on the presence or absence of different risk markers (RM). Recently, myocardial fibrosis as detected by gadolinium-enhanced magnetic resonance imaging (GE-MRI) has been suggested as additional RM. We evaluated the prevalence of myocardial fibrosis and examined correlations with other risk markers in patients considered to be at increased risk.
We included 50 patients with HCM with > 1 (2-4) RM who underwent GE-MRI to check for myocardial fibrosis. Late enhancement was assessed semi-quantitatively using a 17-segment model of the LV (from 0 = absent, 1 = point-shaped, 2 = limited to 1 LV segment, 3 = involving > or = 2 segments). Outflow obstruction was present in 25 patients, the other 25 (1 after septal ablation and after myectomy) were non-obstructive.
Patients (33 men; mean age 44 +/- 13 years; NYHA class: 2.0 +/- 0.9) had a number of RM of 1.7 +/- 0.8. Evidence of myocardial fibrosis > or = 2 was found in 39 patients (mean score: 2.3 +/- 0.9). Correlations were found between left atrial diameter (eta = 0.5), septum thickness (eta = 0.6), and severity of fibrosis. Patients with fibrosis 2 had larger left atria (50 +/- 7 vs 43 +/- 7 mm, P = 0.007) and a thicker septum both on echocardiography (25 +/- 6 vs 17 +/- 5 mm, P < 0.001) and on GE-MRI (28 +/- 5 vs 20 +/- 4 mm, P = 0.01). No correlations were found between fibrosis score and other RM, global LV mass on GE-MRI, or other clinical variables.
Left ventricular hypertrophy and left atrial size, as two markers for global disease severity, correlate with the severity of fibrosis on GE-MRI.
目前,肥厚型心肌病(HCM)中心脏性猝死(SCD)风险评估是基于不同风险标志物(RM)的存在与否。最近,钆增强磁共振成像(GE-MRI)检测到的心肌纤维化被认为是一种额外的风险标志物。我们评估了心肌纤维化的患病率,并研究了其与其他风险标志物在高危患者中的相关性。
我们纳入了50例有>1(2-4)个风险标志物的HCM患者,这些患者接受了GE-MRI检查以检测心肌纤维化。使用左心室17节段模型对延迟强化进行半定量评估(从0=无,1=点状,2=局限于1个左心室节段,3=累及≥2个节段)。25例患者存在流出道梗阻,另外25例(1例在室间隔消融术后和1例在心肌切除术后)为非梗阻性。
患者(33例男性;平均年龄44±13岁;纽约心脏协会心功能分级:2.0±0.9)的风险标志物数量为1.7±0.8。39例患者发现心肌纤维化≥2的证据(平均评分:2.3±0.9)。发现左心房直径(η=0.5)、室间隔厚度(η=0.6)与纤维化严重程度之间存在相关性。纤维化评分为2的患者左心房更大(50±7 vs 43±7 mm,P=0.007),超声心动图显示室间隔更厚(25±6 vs 17±5 mm,P<0.001),GE-MRI显示室间隔也更厚(28±5 vs 20±4 mm,P=0.01)。未发现纤维化评分与其他风险标志物、GE-MRI上的左心室整体质量或其他临床变量之间存在相关性。
左心室肥厚和左心房大小作为整体疾病严重程度的两个标志物,与GE-MRI上的纤维化严重程度相关。