风湿病及结缔组织病

012‘60小儿急性风湿热的实验空与心脏器械检查及分析/马沛然…//中国实用儿科杂志一2001,16(4)一215一217 检查分析结果示:114例急性风湿热(ARF)患儿p溶血性链球菌快速鉴定(GABHSRA)阳性率为90%,显著高于ASO阳性率65.8%;CK一MB升高者46.5%;Q一轻丁酸脱氢酶升高者32.7%;超声心动图左房增大者23.7%,左室扩大者16.7%,右室扩大者7.1%,二尖瓣增厚者13.2%,主动脉瓣增厚者8.8%;多普勒超声心动图检查二尖瓣返流者29.8%,主动脉瓣返流者9.6%。34例有二尖瓣返流患儿中29例二尖瓣返流流速时间积分增大。表3参7(李瑛) 012“1幼年型类风湿性关节…     

风湿性心脏炎心脏超声改变的研究

目的　了解风湿性心脏炎患儿心脏受累情况 ,探讨超声心动图对本病的价值。方法　总结分析2 2 8例风湿性心脏炎患儿的心电图及超声心动图特点 ,比较两者诊断房室肥大的敏感性 ,观察治疗前后心脏超声改变。结果　心电图异常者 1 66例 (73 .1 % ) ,以房室传导阻滞最多见 (67例 ,40 .4% ) ;治疗前 1 66例行心脏超声检查 ,1 62例 (97.6 % )显示不同程度的房室扩大 ,其中以左房大最多见 (1 0 4例 ,62 .7% ) ,1 66例患儿心脏瓣膜均有改变 ,主要表现为二尖瓣返流、增厚及狭窄和主动脉瓣返流 ,其中以二尖瓣返流最多见 (1 0 8例 ,65 .1 % ) ;超声诊断左房大、左室大的敏感性明显优于心电图 ;部分患儿左室收缩功能左室射血分数、心脏指数降低 ,舒张功能E/A <1 ;治疗后复查心脏超声二尖瓣返流、增厚可消失。结论　超声心动图对儿童风湿性心脏炎的诊断及治疗随访具有重要价值     

18例尿毒症患儿心脏彩色多普勒超声表现

目的　探讨尿毒症患儿彩色多普勒超声心动图表现。方法　采用彩色多普勒超声心动图仪观察 18例8岁～ 14岁尿毒症患儿心脏各腔室内径、室壁厚度及瓣膜返流。结果　尿毒症患儿心脏受到不同程度的损害 ,其中左心室扩大 8例 (44 .4% ) ,左心房扩大 9例 (5 0 .0 % ) ,左心室肥厚 3例 (16 .7% ) ,心包积液 10例 (5 5 .6 % ) ,二尖瓣返流 11例(6 1.1% ) ,三尖瓣返流 4例 (2 2 .2 % ) ,主动脉瓣返流 3例 (16 .7% ) ,左室舒张功能减退 8例 (44 .4% ) ,未见左室收缩功能减退。结论　彩色多普勒超声心动图能客观地反映尿毒症患儿心脏病变 ,对指导临床治疗具有重要意义。     

马方综合征24例

目的探讨儿童马方综合征的临床表现、实验室检查的特点。方法对24例马方综合征患儿的症状、体征及检查进行综合评估,分析各自阳性率。结果24例患儿中,胸廓、脊柱畸形19例(79%),其次为瘦长体形16例(67%),蜘蛛指(趾)15例(63%)。超声心动图主动脉窦增宽16例(67%),左心室增大及二尖瓣返流3例(13%)。结论早期诊断马方综合征,预防心血管并发症,是降低本病死亡率的关键。     

儿童扩张型心肌病心脏无创性检查及其在鉴别诊断中的价值

目的　明确心脏无创性检查在儿童扩张型心肌病 (DCM )的变化及其在DCM与肥厚型心肌病 (HCM )、心内膜弹力纤维增生症 (EFE)鉴别中的价值。方法　对 14 6例DCM、4 1例HCM、5 9例EFE患儿 ,用心电图(EKG)、多普勒超声心动图 (ECHO)、彩色多普勒组织显像 (DTI)检查 ,并与 6 117名正常儿童EKG、2 86名正常儿童多普勒ECHO、14 3名正常儿童DTI检查对比。结果　EKG左室面电压增高者HCM、EFE显著多于DCM。多普勒ECHO检查 :DCM患儿心腔增大 ,左心室后壁 (LVPW )、室间隔 (IVS)不增厚 ;HCM患儿心腔不增大 ,LVPW、IVS增厚 ;EFE患儿心腔扩大 ,LVPW、IVS增厚。多普勒ECHO检查 :DCM和EFE患儿心脏收缩功能降低大于HCM患儿 ;DCM、HCM、EFE患儿心脏舒张功能下降差异无显著性。DTI检查 :HCM、DCM、EFE三者差异无显著性 ,DCM患儿心脏舒张功能降低重于收缩功能降低。DCM患儿MVRDeV <4cm/s者 ,预后极差。结论　EKG、多普勒ECHO、DTI对DCM的诊断和DCM与HCM、EFE的鉴别有重要价值。DTI对估计DCM预后有重要价值。     

小儿生理性瓣膜返流

自近10年来多普勒超声心动图广泛应用以来,发现正常的小儿心脏亦可有生理性瓣膜返流,其发生机理尚无定论。生理性瓣膜返流以三尖瓣和肺动脉瓣返流最多,二尖瓣返流比较少,多数学者认为无生理性主动脉瓣返流。生理性瓣膜返流的速度较慢,持续时间较短,面积较小,可与病理性瓣膜返流相鉴别。     

室间隔缺损并二尖瓣返流的病因及预后分析

目的：探讨小儿室间隔缺损（VSD）并二尖瓣返流（MR）的病因及预后。方法：应用HP－500或2000型超声心动图常规探查，明确有无VSD。确诊后应用二维超声、脉冲多普勒叠加彩色血液多切面探查二尖瓣口有无异常返流信号。结果：1787例单纯VSD患儿中233例并MR，其中82．0％有以复呼吸道感染，且感染次数越多，MR程度越重；9．9％VSD患儿二尖瓣存在先天性发育异常；SVD越大，重度MR者越多；手术者MR消失率（78．3％）较非手术者（37．4％）明显提高（P＜0．001）。结论：反复感染、瓣膜先天性病变、大的左向右分流与VSD和并发MR密切相关；适时关闭VSD或修补瓣膜可改善患儿预后。     

泌尿系统疾病

530701 超声心动图改变与儿童急性肾炎预后判断陈荫庭等浙江医学8(1):52～54,1986对52例急性肾炎患儿(年龄6～15岁)于入院第2天作超声心动图检查,动态观察房、室、隔情况并作左心功能测定,以后每月检查一次,结果:4例正常,48例异常,其中左房扩大29例,左房左室扩大12例,左室扩大6例,室间隔增厚1例。52例左心功能测定基本正常。48例房室增大的患儿随着临床症状和实验室检查好转,超声心动图的改变亦逐渐恢复正常,一般恢复时间在3～8个月,以4～6     

左冠状动脉起源于肺动脉6例患儿的诊断与治疗

目的分析左冠状动脉起源于肺动脉(ALCAPA)的诊断特点,评估其手术效果。方法本院2006年3月-2007年10月共收治ALCAPA患儿6例。男4例,女2例;年龄6个月～10岁,平均43.5个月。对这6例患儿的临床表现、心电图(ECG)特征、心脏超声、64排螺旋CT及心导管检查资料、手术治疗及转归进行分析。结果ALCAPA5例患儿有不同程度的多汗、烦躁、气促、拒食等临床表现,临床检查示心功能不全。ECG特异表现:4例患儿ECG存在前侧壁异常Q波及ST段改变,1例患儿存在ST段改变,1例患儿ECG大致正常。典型彩色超声心动图表现为:4例患儿出现左室扩大伴左室收缩功能减低;2例患儿存在二尖瓣轻-中量返流,2例患儿为大量二尖瓣返流;2例患儿表现为右冠状动脉扩张。增强CT及心导管检查有特征性改变。其中5例患儿行手术治疗:2例直接移植,3例行左冠状动脉重建术;2例重度二尖瓣返流患儿同时行二尖瓣成形术。1例无症状患儿未行手术治疗。术后患儿症状改善,无死亡病例。结论了解ALCAPA的病变特点及相关辅助检查,可提高ALCAPA的检出率,尽早手术治疗,改善预后。     

病毒性心肌炎的心功能改变及相关因素分析

目的了解病毒性心肌炎(VM)的心功能改变及相关因素.方法对152例病毒学确诊的心肌炎患儿测定其心功能,并分析心功能改变与抗心肌线粒体和心肌酶之间的关系.结果超声心动图检查,VM患儿每搏射血指数显著低于健康儿童;左室侧壁射血分数、室间隔射血分数和左室射血率显著低于健康儿童;二尖瓣环收缩运动和舒张早期运动速度显著低于健康儿童.心功能下降与抗心肌线粒体抗体阳性与否无关,肌酸激酶同功酶(CK-MB)增高者心功能降低者多于CK-MB正常者.结论部分VM患儿心功能降低,心功能降低与CK-MB增高与否有关,与抗心肌线粒体抗体阳性与否无关.     

Gaucher's disease with mitral and aortic involvement: Echocardiographic findings

Summary Cardiac involvement in Gaucher's disease has been reported in only a few patients, mostly adults with pericardial changes. We describe findings in two siblings with Gaucher's disease, aged 15 and 9 years respectively, in whom mitral and aortic valve lesions were evaluated by auscultation and echocardiography. In both cases the mitral and aortic valves were thick and restricted in motion. Continuous Doppler echocardiography revealed significant mitral regurgitation and mitral stenosis. At the aortic valve level there was a systolic pressure gradient. Echocardiographic investigation of patients with suspected cardiac involvement with Gaucher's disease is recommended.     

Marfanoid children. Etiologic heterogeneity and cardiac findings

The clinical, cardiac, and echocardiographic test results of 20 children with marfanoid features are reviewed. Fifteen were diagnosed as having Marfan syndrome, two had "possible" Marfan syndrome, and three had other diagnoses. On first evaluation, eight patients with Marfan syndrome (53%) had mitral regurgitation and none had aortic regurgitation. Echocardiography showed aortic root enlargement in 12 (80%) of 15 patients and mitral valve prolapse in 12 (80%) of 15. None had a normal echocardiogram. At follow-up examination, one patient had developed aortic root enlargement, and one patient, mitral valve prolapse. Thus, although aortic root enlargement is usually present in early childhood in patients with Marfan syndrome, it is not considered specific because in this study it also occurred in one child with Alport's syndrome and in one with marfanoid features. Four patients with aortic root enlargement were treated with propranolol and their echocardiograms showed no further increase in the aortic root diameter for several years. We recommend echocardiography in the diagnosis and routine management of children in whom Marfan syndrome is suspected.     

Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses

In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%–90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respectively, with peak velocities exceeding 2.5 m/s. Of the 84 patients with satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our patients. None of the control persons showed mitral or aortic regurgitation.     

Cardiac involvement in Sydenham's chorea: clinical and Doppler echocardiographic findings

Sydenham's chorea is often encountered in pure form, but it may also occur in association with carditis. Recently, it has been reported that silent, mild valvular regurgitation has been encountered in patients with rheumatic fever manifested by isolated polyarthritis and pure chorea. We studied 22 children with Sydenham's chorea to determine clinical and subclinical heart involvement at the time of the initial diagnosis. Ages of the patients ranged from 6-19 y (mean 13.14 ± 3.0), and the female/male ratio was 3.4:1. All patients were evaluated by clinical and echocardiographic examination with respect to heart involvement. Carditis was detected in only 5 of 22 patients (23%) by auscultation. Clinical valvular involvement was determined: mitral regurgitation in three and combined mitral and aortic regurgitation in two. The frequency of valvular involvement increased significantly to 63% (14/22) after adding silent mitral regurgitation detected by colour Doppler echocardiography (χ² = 7.50, p = 0.0062). We found that clinically inaudible, but echocardiographically significant, mild valvular regurgitation was frequent in patients with pure chorea. It is concluded that colour Doppler echocardiography may be useful in detecting silent valvular regurgitation and in deciding the duration of prophylaxis.     

室间隔缺损修补术后少见并发症的超声心动图诊断病例系列报告

目的： 探讨室间隔缺损(VSD)修补术后少见并发症的超声心动图诊断要点。 方法：通过查询北京安贞医院（我院）超声影像报告系统及病案管理系统获取病例信息,纳入2013年1月至2018年4月在我院诊断或随访的VSD修补术后少见并发症的连续病例,术后时间不限,使用Philips IE33彩色多普勒超声诊断仪,选择S5-1探头,频率1~5 MHz。VSD修补术后少见并发症由我院经验丰富的医生确诊,并经团队医生共同确认。 结果：符合本文纳入标准的VSD修补术后少见并发症34例,男19例,女15例,年龄4个月至25岁,平均（4.7±5.9）岁。28例为术后左室-右房通道（LVRAC）形成,3例为术后主动脉瓣穿孔并主动脉瓣反流,1例为术后LVRAC形成合并主动脉瓣穿孔,2例为术后主动脉-左房通道形成。34例患者中6例接受外科手术矫治,5例LVRAC患者在随诊过程中自行愈合,其余23例患者继续随访观察中。LVRAC超声心动图表现：①心尖及剑突下四腔心切面显示三尖瓣隔瓣与二尖瓣前瓣之间回声中断,缺损位于三尖瓣环上方;②彩色多普勒显示从左室至右房的异常分流信号;③连续多普勒记录到收缩期高速射流频谱。主动脉瓣穿孔超声心动图表现：①左心室长轴及大动脉短轴切面显示无冠瓣或右冠瓣局部回声中断;②彩色多普勒显示源自瓣叶裂孔处的主动脉瓣反流。主动脉-左房通道超声心动图表现：①彩色多普勒显示从主动脉至左房的异常分流信号,左心室长轴、大动脉短轴及五腔心切面均可显示;②连续多普勒记录到双期连续性高速射流频谱。 结论：多切面扫查及密切结合血流频谱特点,可以明确VSD修补术后异常分流信号的诊断。     

Subclinical Valvulitis in Children with Acute Rheumatic Fever

Doppler echocardiography facility is now available in most parts of the world and its routine use for the initial diagnosis of acute rheumatic fever (ARF) might enhance its early detection and, hence, prevention of rheumatic recurrences. To add to the existing evidence and to reemphasize the need of including echocardiography as a diagnostic criterion for ARF, we investigated the pattern and prevalence of subclinical valvulitis by Doppler echocardiography in patients with ARF manifesting as either pure chorea or isolated arthritis but without clinical signs of carditis. This prospective study was carried out in a single center over a period of 6 months (June to December 2006). Thirty patients with ARF, 16 males and 14 females, aged 4–15 years (mean: 10 ± 3.2) presenting with either chorea or isolated arthritis were included by convenience sampling. Evidence of carditis as detected by echocardiography was present in 21 patients (70%). Chorea was the presenting feature in 19 patients (63%), followed by migratory polyarthritis in 11 (37%). Among patients with chorea, 13 (68%) had evidence of carditis. Mitral regurgitation (MR) was present in all 13, being isolated in 11 and with aortic regurgitation (AR) in 2 patients. In patients with migratory polyarthritis, 8 (73%) had evidence of carditis, all with isolated MR. Echocardiography detected subclinical valvulitis in at least 70% of patients with ARF presenting with either rheumatic chorea or migratory arthritis but no clinical evidence of carditis. MR was the predominant lesion present in all patients either in isolation (90%) or in combination with AR (10%). We suggest that Doppler echocardiography be performed in all patients with suspected ARF and evidence of subclinical valvulitis be used as a diagnostic criterion.     

Acute rheumatic fever in Saudi Arabia: Mild pattern of initial attack

Summary Fifty-one children with the initial attack of acute rheumatic fever (ARF) were studied prospectively to verify the sociodemographic and clinical profile and to compare results with those from other countries. Most children belonged to large families who lived in an urban setting with ready access to medical care. Unlike reports from many developing countries, the clinical manifestations in this study paralleled data from the West and included arthritis in 76% of the cases, carditis in 43%, and chorea in 8%. Among the 22 cases with carditis, 18 had mitral regurgitation, three developed combined mitral and aortic regurgitation, and one had aortic regurgitation. This study demonstrates the mild nature of ARF in Saudi Arabia and supports the concept that climate and geography appear to bear little relationship to the incidence and severity of ARF.     

Acute rheumatic fever in Konya, Turkey

Abstract Background : Patients with acute rheumatic fever (ARF), who were admitted to Pediatric Cardiology Unit of Selçuk University Faculty of Medicine from July 1993 to 1998, were studied retrospectively to verify the clinical profile of the disease and to compare the results with those from other countries. Methods : All patients were examined by one of the two pediatric cardiologists in our study group. Every patient had a chest X‐ray, electrocardiogram and an echocardiographic investigation. Throat culture, antistreptolysin O test, C‐reactive protein and sedimentation rates were investigated for each patient. Results : During the study period, 274 cases with ARF were identified among patients admitted to the present institution. There were 8032 visits during the study period, giving an occurrence rate of 3.4%. Arthritis was the most common major manifestation (81.4%). It was followed by carditis (60.9%) and chorea (17.9%). Subcutaneous nodules (0.7%) and erythema marginatum (0.4%) were both seen in patients with carditis. The mitral valve was the most commonly affected valve (95.8%), followed by the aortic valve (40.1%). Two patients died and regurgitation disappeared in 21% of patients with mitral regurgitation. Fifteen patients (14%) with isolated arthritis and pure chorea had mitral regurgitation demonstrated by echocardiographic investigation but without any significant murmur. Conclusion : The present study indicates that ARF is still a significant problem in Konya and that recurrences can be prevented by administering a 3‐week benzathine penicillin G regimen.