Role of prenatal echocardiography in the study of hypertrophic cardiomyopathy in the fetus

The increased incidence of hypertrophic cardiomyopathy in children of diabetic mothers has already been demonstrated, but its prenatal diagnosis has not yet been extensively studied. The purpose of this prospective study was to evaluate the frequency, severity, and echocardiographic features of fetal hypertrophic cardiomyopathy in a population with several indications for prenatal echocardiography. From March 1987 to April 1991, 283 fetuses were submitted to comprehensive prenatal echocardiography, including M-mode measurements, cross-sectional imaging, Doppler studies, and color flow mapping. One hundred seventy-six were pregnancies complicated by previous or gestational diabetes. The diagnosis of disproportionate septal hypertrophy was made in 39 fetuses (mean septal thickness 7.12 +/- 1.6 mm), at a mean gestational age of 32 weeks. Diabetes mellitus was present in 36 of these pregnancies (92.3%). In four cases, nonimmune hydrops was detected. A systolic anterior motion of the mitral valve was present in three fetuses, but only one showed a gradient across the left ventricular outflow tract. Postnatal echocardiographic examination in 27 babies did not show false positivity. In ten cases, spontaneous regression of the septal hypertrophy was shown. There were three neonatal deaths, unrelated to the myocardial disease. We concluded that transient hypertrophic cardiomyopathy is a frequent entity, especially when associated with diabetes during gestation, being a potential cause for nonimmune hydrops. Fetal echocardiography is the method of choice for its prenatal diagnosis and should always be indicated in diabetic mothers.     

An infant with Costelio syndrome complicated with fatal hypertrophic obstructive cardiomyopathy

We report a 3-month-old girl with Costelio syndrome complicating fatal hypertrophic obstructive cardio-myopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costelio syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.     

回心草化学成分研究

目的:研究回心草的化学成分。方法:回心草95%乙醇提取物依次用石油醚、醋酸乙酯、正丁醇进行充分萃取,用硅胶柱色谱、葡聚糖凝胶柱色谱反复对醋酸乙酯部位进行成分分离、纯化,通过波谱方法鉴定结构。结果:从回心草中共分离鉴定了8个化合物,分别为胡椒碱(1),咖啡酸甲酯(2),尿嘧啶苷(3),熊果酸(4),3β-羟基-5α,8α-氧桥-6,22-二烯(5),3β-羟基-5α,8α-氧桥-6,9(11),22-三烯(6),β-谷甾醇(7),胡萝卜苷(8)。结论:化合物1-6和8为首次从该植物分离得到。     

螺内酯对扩张型心肌病心力衰竭的治疗作用

目的 探讨螺内酯对扩张型心肌病心力衰竭的治疗作用.方法 将119例患者按入选顺序随机分编入治疗组和对照组.对照组接受常规治疗（β受体阻滞剂＋ACEI＋地高辛＋速尿）,治疗组在对照组的基础上加安体舒通（螺内酯）（20mg BID）.结果 治疗1a后,左房、左室内经明显小于对照组,左室射血分数,左室短轴缩短率明显高于对照组.结论 螺内酯对扩张型心肌病心力衰竭有效,可改善心功能和改善心室重构,改善预后.     

Relation between myocardial response to dobutamine stress and sympathetic nerve activation in patients with idiopathic dilated cardiomyopathy: A comparison of123I-MIBG scintigraphic and echocardiographic data

It is likely that a close association exists between findings obtained by two methods: dobutamine stress echocardiography and 123I-MIBG scintigraphy. Both of these methods are associated with beta-adrenergic receptor mechanisms. This study was conducted to demonstrate the relation between myocardial response to dobutamine stress and sympathetic nerve release of norepinephrine in the failing heart. In 12 patients with heart failure due to idiopathic dilated cardiomyopathy, the myocardial effects of dobutamine stress were evaluated by low-dose dobutamine stress echocardiography: and sympathetic nerve function was evaluated by scintigraphic imaging with iodine-123 [123I] meta-iodobenzylguanidine (MIBG), an analogue of norepinephrine. Echocardiography provided quantitative assessment of wall motion and left ventricular dilation; radiotracer studies with 123I-MIBG provided quantitative assessment of the heart-to-mediastinum (H/M) uptake ratio and washout rate. Results showed that H/M correlated with baseline wall motion (r = 0.682, p = 0.0146), wall motion after dobutamine stress (r = 0.758, p = 0.0043), the change in wall motion (r = 0.667, p = 0.0178), and with left ventricular diastolic diameter (r = 0.837, p = 0.0007). In addition, the 123I-MIBG washout rate correlated with baseline wall motion (r = 0.608, p = 0.0360), wall motion after dobutamine stress (r = 0.703, p = 0.0107), and with the change in wall motion (r = 0.664, p = 0.0185). Wall motion, especially in the myocardial response to dobutamine stress, is related to sympathetic nerve activity in heart failure.     

氨基胍对糖尿病大鼠心脏功能及心肌超微结构的影响

目的 :探讨非酶糖化抑制剂 氨基胍 (amin oguanidine ,AG)对链尿佐菌素 (streptozotocin ,STZ)糖尿病大鼠心肌的保护作用 ,为糖尿病心肌病的防治提供参考。方法 :建立STZ糖尿病大鼠模型 ,随机分为对照组、糖尿病组和氨基胍治疗组 ,AG剂量为15 0mg·kg-1·d-1。 12周时测定大鼠血清果糖胺含量、心脏重量指数、左室内压最大上升和下降率 (±dp dtmax)值 ,电镜观察心肌超微结构 ,测量心肌毛细血管基底膜厚度。结果 :糖尿病组大鼠血清果糖胺含量、心脏重量指数明显增高 ,±dp dtmax降低 ;超微结构显示心肌肌原纤维排列紊乱 ,线粒体肿胀变性 ,间质胶原增生 ,微血管内皮细胞肿胀、基底膜明显增厚。氨基胍治疗组血清果糖胺含量降低、心脏重量指数明显下降、±dp dtmax 值上升 ,微血管基底膜增厚减轻 ,间质胶原减少 ,心肌细胞超微结构异常减轻。结论 :糖尿病时存在心脏功能异常、心肌肥厚和超微结构的改变 ,早期应用AG在一定程度上可阻抑糖尿病心肌病变的发展。     

儿童先天代谢异常伴发的肥厚型心肌病

儿童先天代谢异常是导致肥厚型心肌病（HCM）的重要病因, 掌握每种疾病的特征性表现有助于正确的诊断及鉴别诊断。多数先天代谢异常伴发HCM属于常染色体隐性遗传,少数为常染色体显性遗传X连锁遗传方式,部分线粒体病呈母系遗传。常规心电图、超声心动图等心脏检查即可以为部分疾病的基础病因找到诊断线索。随着酶替代疗法等新的治疗方法的不断进步,加强心脏评估,恰当地针对原发病治疗,多学科协同合作将为越来越多的患者生存提供可能。