Boissier Florence, Achkouty Guy, Bruneval Patrick, Fabiani Jean-Noël, Nguyen Anh Tuan, Riant Elisabeth, Desnos Michel, Hagège Albert
Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France; Department of Pathology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
Arch Cardiovasc Dis. 2015 Apr;108(4):244-9. doi: 10.1016/j.acvd.2015.01.003. Epub 2015 Mar 6.
While occasional reports of mitral valve chordal rupture have been described in hypertrophic cardiomyopathy, the exact prevalence and characteristics of this event in a large medical cohort have not been reported.
To assess the prevalence of mitral valve chordal rupture in hypertrophic cardiomyopathy and the clinical, echocardiographic, surgical and histological profiles of those patients.
We searched for patients with mitral valve chordal rupture diagnosed by echocardiography among all electronic files of patients admitted to our centre for hypertrophic cardiomyopathy between 2000 and 2010.
Among 580 patients admitted for hypertrophic cardiomyopathy, six patients (1%, 5 men, age 68-71 years) presented with mitral valve chordal rupture, symptomatic in five cases, always involving the posterior mitral leaflet. In all cases, echocardiography before rupture showed mitral valve systolic anterior motion, with anterior (and not posterior) leaflet elongation compared with a random sample of patients with non-obstructive hypertrophic cardiomyopathy (P=0.006) (and similar to that observed in obstructive hypertrophic cardiomyopathy). Significant resting left ventricular outflow tract obstruction was always present before rupture and disappeared after rupture in the five cases requiring mitral valve surgery for severe mitral regurgitation. Histological findings were consistent with extensive myxomatous degeneration in all cases.
Mitral valve chordal rupture is: infrequent in hypertrophic cardiomyopathy; occurs in aged patients with obstructive disease; involves, essentially, the posterior mitral leaflet; and causes, in general, severe mitral regurgitation requiring surgery. Myxomatous degeneration may be the substrate for rupture in these patients.
虽然肥厚型心肌病中偶有二尖瓣腱索断裂的报道，但在大型医学队列中该事件的确切患病率及特征尚未见报道。
评估肥厚型心肌病中二尖瓣腱索断裂的患病率以及这些患者的临床、超声心动图、手术和组织学特征。
我们在2000年至2010年间因肥厚型心肌病入住本中心的所有患者电子病历中搜索经超声心动图诊断为二尖瓣腱索断裂的患者。
在580例因肥厚型心肌病入院的患者中，有6例（1%，5名男性，年龄68 - 71岁）出现二尖瓣腱索断裂，5例有症状，均累及二尖瓣后叶。所有病例中，断裂前的超声心动图显示二尖瓣收缩期前向运动，与非梗阻性肥厚型心肌病患者的随机样本相比，前叶（而非后叶）延长（P = 0.006）（与梗阻性肥厚型心肌病中观察到的情况相似）。在5例因严重二尖瓣反流需要进行二尖瓣手术的病例中，断裂前均存在显著的静息左心室流出道梗阻，断裂后消失。所有病例的组织学结果均与广泛的黏液瘤样变性一致。
二尖瓣腱索断裂在肥厚型心肌病中并不常见；发生于患有梗阻性疾病的老年患者；主要累及二尖瓣后叶；通常会导致严重二尖瓣反流需要手术治疗。黏液瘤样变性可能是这些患者腱索断裂的基础。
