摘要
目的通过分析致心律失常性右室心肌病（ARVC）心力衰竭期的病理改变，以进一步了解其临床分期与病理表型的关系。方法从2004—2007年在阜外心血管病医院接受心脏移植的心力衰竭病例中，收集病理诊断为ARVC的受体心脏8例，测量心脏重量，评价左右心室心腔扩张、心肌细胞肥大、脂肪浸润、纤维化、附壁血栓和伴发心肌炎等指标，注意左心室受累情况，并进行病理分型。结果8例中的7例为经典型（即右心室改变为主），1例为左优势型（左心室改变为主），未见双室型病例。组织学均为纤维脂肪型，未见单纯脂肪型病例。经典型病例的右心室中、重度扩张，少数有室壁瘤形成，其中6例伴左心室受累，受累左心室轻、中度扩张，心肌广泛间质纤维化，部分病例伴替代性疤痕，而脂肪浸润量小，多局限于心外膜下。左心室心肌细胞肥大普遍。而左优势型的左心室重度扩张，弥漫间质纤维化和局部透壁性脂肪浸润。8例中3例左心室明显肥厚，3例查见双室附壁血栓，1例伴局灶性心肌炎。结论ARVC心力衰竭期的左心室受累多见而严重，左心室间质纤维化突出，心肌细胞肥大明显，但脂肪替代少见和局限。左、右心室多扩张，可见附壁血栓，应注意与扩张型心肌病等鉴别。
Objectives To study the pathologic features of arrhythmogenic right ventricular cardiomyopathy （ARVC） in the phase of heart failure. Methods Eight cases underwent heart transplantation in Fuwai Hospital during the period from May, 2004 to July, 2007 with pathologic diagnosis of ARVC were studied. The age of patients ranged from 15 to 54 years. They had history of palpitation and syncope for 1 to 22 years. Severe heart failure was diagnosed according to the New York Heart Association Classification System. The recipient hearts were examined and the following parameters were evaluated： weight of heart, presence of cardiac dilatation, myocardial hypertrophy, fatty infiltration, fibrosis, parietal thrombosis and myocarditis. The degree of left ventricular involvement was also analyzed. Results Of the 8 cases studied, 7 cases with prominent right ventricular lesion （fibrofatty replacement） were classified as classic type. One case with prominent left ventricle lesion and mild right ventricle involvement was classified as left predominant type. No biventricular type and no pure fatty infiltration were found. The cases of classic type showed moderate to severe dilatation of right ventricle, sometimes with aneurysm formation. Left ventricle was involved in 6 cases, which showed diffuse interstitial fibrosis, patchy fibrous replacement and subepicardial fatty infiltration. Mild to moderate dilatation of left ventricle, myocardial hypertrophy and vacuolation were also observed in these cases. The case of left predominant type had severe hypertrophy and dilatation of left ventricle, with prominent diffuse interstitial fibrosis and transmural fatty infiltration. Besides, 3 cases showed left ventricular hypertrophy and parietal thrombosis in both ventricles. Focal lymphocytic myocarditis was noted in 1 case. Conclusions Left ventricular involvement is common in the heart failure phase of ARVC. Extensive interstitial fibrosis, marked hypertrophy and degeneration of myocardial fibers, as well as severe cardiac dilatation with organized thrombi, represent the major pathologic changes which resembles dilated cardiomyopathy.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2008年第3期169-173,共5页
Chinese Journal of Pathology
关键词
心肌疾病
心律失常性右心室发育不良
心力衰竭
充血性
Cardiomyopathies
Arrhythmogenic right ventricular dysplasia
Heart failure, congestive
