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自发性非肥厚限制性心肌病在宠物猫中的发生:人类疾病的一种新动物模型。

Spontaneously occurring restrictive nonhypertrophied cardiomyopathy in domestic cats: a new animal model of human disease.

机构信息

Caspary Research Institute, The Animal Medical Center, New York, NY.

出版信息

Cardiovasc Pathol. 2014 Jan-Feb;23(1):28-34. doi: 10.1016/j.carpath.2013.08.001. Epub 2013 Sep 12.

Abstract

BACKGROUND

Spontaneously occurring small animal models of myocardial disease, closely resembling the human condition, have been reported for hypertrophic cardiomyopathy (in cats) and arrhythmogenic right ventricular cardiomyopathy (in cats and boxer dogs). Nonhypertrophied restrictive cardiomyopathy (RCM) is a well-recognized but relatively uncommon primary heart muscle disease causing substantial morbidity in humans. We describe RCM occurring in felines here as a potential model of human disease.

METHODS

We used two-dimensional and Doppler echocardiography to define morphologic and functional features of RCM in 35 domestic cats (25 male; 10±4 years old) presenting to a subspecialty veterinary clinic. Ten underwent complete necropsy examination. Echocardiographic parameters of diastolic filling were compared to those in 41 normal controls.

RESULTS

The 35 cats presented with congestive heart failure (n=32), lethargy (n=2), or syncope (n=1), associated with thromboembolism in 5 and supraventricular tachyarrhythmias in 8. During an average 4.4-year follow-up period, 18 died or were euthanized due to profound heart failure, and 3 died suddenly; survival from clinical presentation to death was 0.1 to 52 months. Echocardiographic and necropsy examination showed biatrial enlargement, nondilated ventricular chambers, and normal wall thicknesses and atrioventricular valves. Histopathology demonstrated disorganized myocyte architecture and patchy replacement myocardial fibrosis. Pulsed Doppler demonstrated restrictive physiology with increased early (E) mitral filling velocity (1.1±0.3 m/s) and peak E to peak late (A) flow ratios (4.3±1.2), reduced A filling velocity (0.3±0.1 m/s), and shortened mitral deceleration time (40.7±9.3 ms; all P<.001 vs. controls), with preserved left ventricular systolic function.

CONCLUSIONS

A primary myocardial disease occurring spontaneously in domestic cats is remarkably similar to restrictive nondilated and nonhypertrophied cardiomyopathy in man and represents another potential animal model for human disease.

摘要

背景

已报道多种自发性小动物心肌疾病模型,与人类疾病情况非常相似,包括肥厚型心肌病(在猫中)和致心律失常性右室心肌病(在猫和拳师犬中)。非肥厚性限制型心肌病(RCM)是一种公认但相对少见的原发性心肌疾病,可导致人类发病率较高。我们在此描述猫中的 RCM,认为其是人类疾病的潜在模型。

方法

我们使用二维和多普勒超声心动图,来定义 35 只出现于专业兽医诊所的家猫(25 只为雄性;10±4 岁)的 RCM 的形态和功能特征。其中 10 只进行了完整的尸检检查。比较舒张期充盈的超声心动图参数与 41 例正常对照。

结果

35 只猫表现为充血性心力衰竭(n=32)、嗜睡(n=2)或晕厥(n=1),5 只伴有血栓栓塞,8 只伴有室上性心动过速。在平均 4.4 年的随访期间,18 只因严重心力衰竭而死亡或安乐死,3 只突然死亡;从临床出现到死亡的生存率为 0.1 至 52 个月。超声心动图和尸检检查显示双心房增大、心室腔不扩张、正常的室壁厚度和房室瓣。组织病理学显示心肌细胞结构紊乱和斑片状替代心肌纤维化。脉冲多普勒显示限制生理特性,早期(E)二尖瓣充盈速度增加(1.1±0.3 m/s),E 峰与晚期(A)峰值流速比值升高(4.3±1.2),A 充盈速度降低(0.3±0.1 m/s),二尖瓣减速时间缩短(40.7±9.3 ms;均 P<.001 与对照组相比),而左心室收缩功能正常。

结论

家猫中自发性发生的原发性心肌疾病与人类限制型非扩张性和非肥厚性心肌病非常相似,是人类疾病的另一种潜在动物模型。

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