Miani D, Pinamonti B, Bussani R, Silvestri F, Sinagra G, Camerini F
Divisione di Cardiologia, Ospedale Maggiore, Trieste, Italy.
Br Heart J. 1993 Feb;69(2):151-7. doi: 10.1136/hrt.69.2.151.
Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle.
To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy.
Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients.
Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy.
右心室发育不良是一种病因不明的心肌疾病，常具有家族性，其解剖学特征为右心室心肌出现脂肪或纤维脂肪浸润。它通常被视为右心室的一种选择性疾病。
调查两个家族中左心室受累的患病率及特征，这两个家族中至少有一名成员经尸检确诊患有右心室发育不良。
发现8例患者患有右心室发育不良。其中3例经尸检确诊。超声心动图或血管造影或两者均显示7例存在左心室受累。范围从局部室壁运动异常到中度或重度左心室功能障碍。4例病情呈进行性发展。尸检时，左心室心肌显示主要为纤维化及心肌细胞退行性改变。2例患者心尖部存在心肌变薄并伴有脂肪浸润区域。
家族性右心室发育不良可能是一种累及左心室的进行性疾病。晚期疾病在临床上可能与扩张型心肌病相混淆。
