Vetter U, Mayerhofer R, Lang D, von Bernuth G, Ranke M B, Schmaltz A A
Department of Pediatrics, University of Ulm, Federal Republic of Germany.
Eur J Pediatr. 1990 Apr;149(7):452-6. doi: 10.1007/BF01959393.
Forty-eight children and adolescents (mean age 10.5 years, range 1.25-18 years) with clinical evidence of Marfan syndrome were studied. Height and weight percentiles were established. Cardiac dimensions and morphology were studied by M-mode and 2D-echocardiography. At diagnosis left atrial and left ventricular end-diastolic diameter and left ventricular posterior wall thickness were within normal limits except in a few adolescent patients. Interventricular septum was thickened in about 20% and aortic diameter increased in 56% of the patients. An additional 13% of patients developed aortic dilation during the study period. At diagnosis regression analysis revealed a significant (P less than 0.05) correlation of the aortic diameter, septal thickness and the posterior left ventricular wall thickness and body surface area. Follow up studies of 19 patients allowed documentation of the development of aortic root dilation.
对48名有马凡氏综合征临床证据的儿童和青少年（平均年龄10.5岁，范围1.25 - 18岁）进行了研究。确定了身高和体重百分位数。通过M型和二维超声心动图研究心脏大小和形态。诊断时，除少数青少年患者外，左心房和左心室舒张末期直径以及左心室后壁厚度均在正常范围内。约20%的患者室间隔增厚，56%的患者主动脉直径增加。在研究期间，另有13%的患者出现主动脉扩张。诊断时回归分析显示主动脉直径、室间隔厚度、左心室后壁厚度与体表面积之间存在显著（P < 0.05）相关性。对19名患者的随访研究记录了主动脉根部扩张的发展情况。
