Bjørnstad P G, Semb B K, Trygstad O, Seip M
Eur J Pediatr. 1985 Nov;144(4):355-9. doi: 10.1007/BF00441777.
Because cardiomegaly has been observed in lipodystrophic patients we studied cardiac morphology and function with one- and two-dimensional echocardiography in addition to general cardiologic examination in a series of seven patients. Muscular hypertrophy with increased chamber size and myocardial indentations were found. Two patients had asymmetrical septal hypertrophy (ASH), and two patients demonstrated systolic anterior movement (SAM) of the mitral valve. Wall motion analysis showed anomalities in four patients during contraction, in three during the early relaxation phase. Since pathological findings, probably increasing with age, were made in the majority of the patients, these findings add an additional unfavourable aspect to the syndrome.
由于在脂肪营养不良患者中观察到心脏肥大，我们对一系列7例患者除进行常规心脏检查外，还用一维及二维超声心动图研究了心脏形态和功能。发现有心肌肥厚伴心腔扩大及心肌凹陷。2例患者有不对称性室间隔肥厚（ASH），2例患者显示二尖瓣收缩期前向运动（SAM）。室壁运动分析显示4例患者在收缩期、3例患者在舒张早期有异常。由于大多数患者有随年龄可能加重的病理表现，这些发现给该综合征增加了另一个不利方面。
