Frustaci A, Perrone G A, Gentiloni N, Russo M A
Institute of Cardiology, Catholic University, Rome, Italy.
Am J Clin Pathol. 1992 Apr;97(4):503-11. doi: 10.1093/ajcp/97.4.503.
An unusual case of severe dilated cardiomyopathy is described in a woman with postpartum hypopituitary function, which was characterized by marked decrease of cardiac myofibrils (decreased ratio of myofibrillar volume:cell volume) and somatomedin C deficiency. The patient was treated with a daily intramuscular dose of 4 UI of recombinant human growth hormone for 3 months. Clinical and laboratory assessment and follow-up consisted of noninvasive (echocardiograms, Doppler study, and clinical laboratory tests) and invasive procedures (angiography and endomyocardial biopsy). Morphologic study included optical and electron microscopic examinations and morphometric analysis. The patient exhibited a relevant improvement of cardiac function after recombinant human growth hormone administration. The clinical improvement was confirmed by normalization of electrocardiographic voltages, increased myocardial mass, angiography, and optical and electron microscopic examination, which showed a dramatic recovery of myofibrillar content in myocardiocytes. Furthermore, withdrawal of growth hormone was followed by reduction of electrocardiographic voltages and myocardial mass as well as by impairment of left ventricular function. The authors suggest that there are forms of dilated cardiomyopathy that may benefit dramatically by growth hormone administration. These forms should be identified by growth hormone/somatomedin C axis deficiency and by decrease in myofibrillar content at endomyocardial biopsy.
本文描述了一例患有产后垂体功能减退的严重扩张型心肌病的不寻常病例,其特征为心肌肌原纤维显著减少(肌原纤维体积与细胞体积之比降低)以及生长调节素C缺乏。该患者接受了为期3个月的每日4单位重组人生长激素的肌肉注射治疗。临床和实验室评估及随访包括非侵入性检查(超声心动图、多普勒研究和临床实验室检查)和侵入性检查(血管造影和心内膜心肌活检)。形态学研究包括光学显微镜和电子显微镜检查以及形态计量分析。给予重组人生长激素后,患者的心功能有了显著改善。心电图电压恢复正常、心肌质量增加、血管造影以及光学显微镜和电子显微镜检查均证实了临床改善,这些检查显示心肌细胞中的肌原纤维含量有显著恢复。此外,停用生长激素后,心电图电压和心肌质量降低,左心室功能受损。作者认为,存在某些形式的扩张型心肌病,生长激素治疗可能会使其显著受益。这些形式应通过生长激素/生长调节素C轴缺乏以及心内膜心肌活检时肌原纤维含量减少来识别。