实时三维超声心动图在小儿先天性二尖瓣反流机制研究中的应用

目的探讨实时三维超声心动图在小儿先天性二尖瓣反流病变定性诊断及定量测量中的应用价值。方法选取24例先天性二尖瓣反流患儿(病变仅累积二尖瓣,未合并其他心内畸形)及24例正常儿童,应用实时三维超声心动图全面观察二尖瓣装置形态特点,测量二尖瓣装置三维参数包括收缩期二尖瓣环面积(AA1)、舒张期二尖瓣环面积(AA2)、收缩期与舒张期瓣环面积比(T%)、前后组乳头肌距瓣环距离(AL-A、PM-A)、前后组乳头肌间距离(AL-PM)、前后组乳头肌与瓣环中心点夹角(argAL-C-PM)、前后瓣叶活动度(a1、a2)、左室舒张末期容量(LVEDV)、左室收缩末期容量(LVESV)、左室射血分数(LVEF),量化二尖瓣装置定量测量指标,明确先天性二尖瓣反流的确切机制。结果实时三维超声心动图能全面显示二尖瓣装置的整体形态,可显示二尖瓣裂缺,AA1、AA2、AL-A、PM-A、AL-PM、argAL-C-PM、a1、a2、LVEDV、LVESV、LVEF等三维参数两组间差异有统计学意义,提示二尖瓣病变瓣叶裂缺、瓣叶活动度、瓣环大小、乳头肌位置、左室容量等因素共同影响小儿二尖瓣的正常关闭。结论实时三维超声心动图在立体显示小儿二尖瓣病变组织、定量测量二尖瓣装置三维参数、确定先天性二尖瓣病变反流机制方面有极大优势。     

先天性左冠状动脉主干闭锁3例超声心动图特征及文献复习

目的 通过研究先天性左冠状动脉主干闭锁（LMCAA）的超声心动图表现,提高对LMCAA诊断的准确性。方法 回顾性分析经冠状动脉造影证实的3例LMCAA患儿的超声心动图检查结果,并复习相关文献,总结LMCAA超声心动图特征。结果 LMCAA特异性超声心动图特征：①主动脉左冠状动脉窦内无左冠状动脉主干开口,左冠状动脉主干近心端闭锁呈盲端,远心端内径细窄,发育不良;②右冠状动脉内径增宽;③多切面未显示左冠状动脉与肺动脉确切连接的证据。LMCAA非特异性超声心动图特征：①左心室明显扩大,左室收缩功能可正常或减低;②二尖瓣腱索、乳头肌回声显著增强,可伴有二尖瓣脱垂。彩色多普勒超声特征： ①收缩期二尖瓣口可见中至大量反流信号;② 左、右冠状动脉之间形成细小侧支循环; ③左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;④发育不良的左冠状动脉虽然在肺动脉周围分布,但彩色多普勒超声不能显示其与肺动脉连接的确切逆灌血流信号。结论 LMCAA有特异性的超声心动图特征,提高对LMCAA的全面认识是诊断本病的关键。     

成人型与婴儿型左冠状动脉异常起源于肺动脉的超声诊断

目的总结不同类型左冠状动脉异常起源于肺动脉（ALCAPA）的超声心动图表现,提高对本病的诊断及鉴别诊断能力。方法回顾性分析首都医科大学附属北京安贞医院儿童心血管病中心收治并经手术证实的24例ALCAPA患儿的超声心动图结果,根据侧支循环的发育情况分为婴儿型和成人型,并总结婴儿型和成人型ALCAPA的超声心动图特征。 结果婴儿型和成人型ALCAPA相同的超声心动图特征：①左冠状动脉窦内无左冠状动脉主干开口,其主干直接与肺动脉连接;②左心室高度扩张,室间隔和左室前壁节段性运动障碍,左室收缩功能减低;③左心室广泛纤维化,以心内膜下区域最为显著;④二尖瓣腱索、乳头肌纤维化,回声显著增强;⑤右冠状动脉起源位置正常,内径增宽;⑥左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;⑦收缩期二尖瓣口可见少至大量反流信号。婴儿型和成人型ALCAPA不同的超声心动图表现：彩色多普勒显示成人型侧支循环血流丰富,婴儿型侧支循环少。 结论婴儿型和成人型ALCAPA具有其特异性的超声心动图特征,结合其临床及心电图等表现,可以对本病做出正确诊断。     

心肌肿瘤并二尖瓣置换一例

患儿:女,10岁.因自幼发现心脏杂音于2010年2月29日入院.5岁时诊断为二尖瓣狭窄并关闭不全,因家庭经济原因未行进一步诊治.检查:心率120次/min,血压90/60mmHg,心尖部闻及双期杂音,肺动脉瓣区第二心音增强;心电图:窦性心律,右心室增大;胸片:心脏增大呈球型;心脏彩超:双房、右心室增大,左侧房室沟外侧心包腔内(冠状静脉窦后方)探及52mm×49mm等回声光团,无蒂、边界清楚、位置固定,周边及内部未探及血流信号.二尖瓣增厚6 mm,腱索增粗、缩短,开放受限,瓣口面积0.6 cm2.     

原发性良性二尖瓣脱垂

二尖瓣脱垂(MVP)是常见的良性心脏缺损,也是最常见的先天畸形。由于二尖瓣叶组织过多,或腱索过长,当心脏收缩时二尖瓣前叶或后叶,部分或全部脱入左房。本病在儿童期临床表现多不明显,到成年以后听诊特点才变得明显,并出现症状。认识MVP的心音改变是困难的。作者提出了如何诊断、处理儿童无症状性二尖瓣脱垂的具体方法。二尖瓣脱垂的诊断方法根据左室造影确诊的MVP采用听诊法只能诊断出75%,而采用超声心动图检查可以诊断出80～90%,本病听诊阳性发现是收缩中期     

二尖瓣成行术后二尖瓣特征参数的三维量化分析

目的量化分析二尖瓣成形术前、术后二尖瓣特征参数,明确二尖瓣成形术后残留二尖瓣反流（MR）的发生机制。方法选取行二尖瓣成形术患儿30例,根据患儿术后MR改善程度,分为反流改善组与未改善组,量化分析和比较患儿二尖瓣装置三维参数术前、术后的改变。结果二尖瓣成形术后,MR改善组与MR未改善组相比,瓣环扩大程度均较术前改善,差异有统计学意义（P<0.05）;后瓣活动度幅度、前后组乳头肌至瓣环的空间三维距离、前后组乳头肌之间的空间距离、乳头肌与瓣环夹角、左室容量及射血分数等参数,MR改善组较术前明显改善（P<0.01）,而MR未改善组,除后瓣活动度外,其余参数术前、术后差异无统计学意义（P>0.05）。结论MR改善组患儿术后二尖瓣装置参数较术前有明显好转,而MR未改善组除二尖瓣环大小与瓣叶活动度较术前好转外,乳头肌位置、瓣下牵拉力、左室功能等无明显改变,而这些因素可能是术后残余反流的关键所在。     

左心室肌致密化不全8例临床分析

目的　探讨左心室肌致密化不全的临床及超声心动图特点。方法　分析左心室肌致密化不全临床表现、心电图、胸片、超声心动图特点。结果　左心功能不全 3例 ,肾栓塞 1例 ;室性期前收缩 2例 ;超声心动图均发现左心室腔内二尖瓣水平、腱索水平或乳头肌水平以下的心肌被网格状或蜂窝状的海绵样心肌组织所充填 ,可合并先天性心脏畸形。结论　左心室肌致密化不全临床上可表现为左心功能不全 ,室性心律失常及心内膜血栓伴系统栓塞 ,超声心动图是诊断本病的首选方法。     

镜面右位心合并膜部瘤型室间隔缺损封堵治疗1例报告

临床资料患儿女.11岁.因发现心脏杂音11年来我院就诊.体检:BP 119/60 mmHg,HR 84次/min,胸骨右缘3-4肋间4/6级收缩期杂音伴震颤,向左胸及胸前区传导.心电图示右位心,不完全性右束支传导阻滞.心脏正侧位片示心影不大,心胸比例0.41,主动脉结、心尖位于右侧,肺动脉段稍突出.右侧膈下可见胃泡影.诊断为镜面右位心,左向右分流性先天性心脏病.超声心动图示心脏位于右侧胸腔,心尖朝右,心房反位,心室左袢,房室连接关系、各房室腔大小正常.房间隔中段呈瘤样突向右房,基底宽16.8 mm,未见明显过隔血流.室间隔膜周部回声缺损约6.7 mm,呈瘤样突向右室,瘤呈漏斗型,其上缘距主动脉瓣0 mm,其余心内结构未见异常.彩色多普勒超声图像(CDFI)示缺损处室水平左向右过隔血流束,峰值流速475 cm/s,压差90 mmHg.诊断为:[1]先天性心脏病,镜面右位心;[2]膜部瘤型室间隔缺损(VSD),室水平左向右分流;[3]房间隔膨出瘤形成.     

左向右分流先天性心脏病合并三尖瓣反流患儿血流动力学变化及发病机制

目的 探讨新生儿左向右分流先天性心脏病(先心病)发生三尖瓣反流(TR)的临床特征和心脏血流动力学变化及TR的发生机制.方法 对左向右分流(分流直径≥2 mm)先心病伴TR和不伴TR各52例患儿进行前瞻性对照研究,观察其临床及多普勒超声心动图特征:心腔内径大小、TR最大反流速度、瓣膜反流程度、肺动脉估测压力变化,分析与TR发生有关的影响因素.结果 TR组中宫内窘迫、娩出窒息、X线胸片显示心影饱满或增大、心胸比大于0.6、心力衰竭、缺氧心肌损害、二尖瓣反流的发病率比对照组高(P＜0.05),前5项因素使TR发生的危险分别增加了3.6、9.3、4.7、3.6、7.5倍.TR组与对照组比较,左、右心房明显增大,肺动脉内径增宽(P＜0.05).52例中,轻度反流37例,中度10例,重度5例,平均反流速度(2.98±0.65)m/s.TR患儿同时存在房、室水平分流的肺动脉估测压＞心室(包括导管)水平分流＞心房水平分流(P＜0.05).对同时存在房、室水平分流的肺动脉估测压,TR组明显高于对照组(P＜0.05).结论 左向右分流先心病发生TR时,表明已存在心脏血流动力学异常,左向右分流使容量负荷增加,肺动脉压升高是形成TR的主要因素,宫内窘迫、娩出窒息、心脏扩大和心力衰竭也是形成TR的重要影响因素.     

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     

新生儿三尖瓣腱索断裂合并重度肺动脉高压一例并文献复习

目的分析新生儿三尖瓣腱索断裂引起的三尖瓣脱垂合并重度肺动脉高压的临床特点及治疗方法。方法对河北省儿童医院新生儿科2018年11月收治的1例三尖瓣腱索断裂、三尖瓣脱垂合并重度肺动脉高压新生儿的临床资料进行回顾性分析。中文以"新生儿、三尖瓣脱垂、乳头肌或腱索断裂、肺动脉高压"为检索词在中国知网、万方数据库,英文以"(neonate OR newborn) AND (tricuspid valve prolapsed) AND (rupture OR necrosis) AND (papillary muscle OR chordae tendineae) AND (pulmonary hypertension)"为检索式在PubMed数据库中检索自建库至2020年9月报道的临床资料完整的文献,总结该病的临床特点及治疗方法。结果患儿男,9小时龄,因"生后皮肤青紫9 h"入院。患儿系足月剖宫产出生,生后10 min出现全身皮肤发绀、呼吸发憋,出生医院正压通气不能缓解,予气管插管、呼吸机辅助通气,生后9 h病情无好转转至河北省儿童医院。入院查血气分析提示动脉氧分压22.5 mmHg(1 mmHg=0.133 kPa),胸部正位X线片提示双肺透光度正常,超声心动图表现为三尖瓣前叶脱垂、三尖瓣大量反流、重度肺动脉高压、卵圆孔未闭,卵圆孔水平右向左分流。予继续呼吸机辅助呼吸,吸入一氧化氮、静脉用曲前列尼尔联合降肺动脉压治疗,患儿低氧血症逐渐缓解,好转出院。患儿在11月龄时行三尖瓣瓣叶成形术+三尖瓣瓣环成形术+卵圆孔未闭缝合术,术中证实为三尖瓣腱索断裂导致三尖瓣前叶脱垂,随访至22月龄发育状况良好。文献检索未发现中文文献,检索到英文文献12篇,加上本例共分析21例患儿资料,其中仅1例出生胎龄33周早产儿,其余20例均为足月新生儿;21例患儿中男12例。21例患儿均在生后早期出现发绀,接受气管插管机械通气,其他治疗包括吸入一氧化氮、静脉用米力农、血管活性药、利尿剂、前列环素类药物等,6例患儿接受了体外膜氧合治疗。早期报道的2例生后因心肺功能衰竭死亡,其余病例均在接受外科手术后存活,术中或尸检均发现三尖瓣脱垂、三尖瓣乳头肌或腱索断裂。结论乳头肌或腱索断裂导致三尖瓣脱垂在新生儿期罕见,患儿可发生严重低氧血症,早期识别、充分心肺支持稳定血流动力学,适时外科手术可显著降低病死率。     

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??Abstract??Objective??To study prevention and treatment methods for tricuspid injury in transcatheter closure procedure of perimembranous ventricular septal defects??PMVSDs??. Methods??Eight cases with tricuspid injury in transcatheter closure procedure of PMVSDs were enrolled from Oct. 2002 to Dec. 2009. Tricuspid stenosis presented in 4 cases??of whom 2 needed surgery. The right disc and screw of PMVSDs occluder got entangled into the chordae of anterior tricuspid leaflet in both operative cases. Tricuspid regurgitation occurred in 4 cases. Three cases received surgery repair and device removal. The operation finding was anterior papillary muscle rupture in 1?? anterior papillary muscle rupture combined with anterior tricuspid leaflet tear in 1 and chordae tendineae of septal leaflet stretched by device screw in 1 case. Another patient with mild to moderate tricuspid regurgitation and septal leaflet prolapse also received follow-up study. Results??Among the 5 patients with surgery?? tricuspid valve got to normal in 4 cases and valve flow velocity slightly accelerated in 1 case. In the follow-up study?? there were no clinical symptoms in the cases with mild or mild to moderate tricuspid injury with no repair. Conclusion??In procedure of transcatheter closure of PMVSDs?? passing the arteriovenous circuit wire through tricuspid chordae can lead to tricuspid injury. Sever tricuspid injury should be repaired by surgery.     

Mitral Valve Injury During Balloon Valvuloplasty for an Infant with Severe Aortic Stenosis: Spatial Evaluation Using Three-Dimensional Echocardiography

We report on a 4-month-old infant with mitral valve injury that was induced by percutaneous balloon valvuloplasty for severe aortic valve stenosis. Three-dimensional echocardiography revealed a laceration at the anterior leaflet of the mitral valve. The mitral valve injury was successfully repaired by surgical operation 1 year after the valvuloplasty. This unexpected complication is associated with anatomical disorders of congenital aortic stenosis, including hypoplastic left ventricle, short chordae tendae, and large papillary muscles.     

Mitral Regurgitation due to Chordae Tendineae Rupture in an Infant with Aortic Coarctation

A 2-month-old male suddenly developed mitral regurgitation aggravation while waiting for surgical repair of aortic coarctation. There were no signs of infection or history of trauma. Emergency surgery was performed, during which rupture of chordae tendineae of the A2 portion of the anterior mitral leaflet was confirmed. At last follow-up, 11 months postsurgery, the patient was in an acceptable state, with continuing moderate mitral valve regurgitation. This case demonstrates an unusual but dangerous natural course of aortic coarctation and suggests an additional basis of urgent repair of aortic coarctation.     

Rupture of the papillary muscle of the tricuspid valve — echocardiographic diagnosis of a rare anomaly leading to critical tricuspid valve regurgitation in the newborn

We present the case of a male full-term neonate who presented at 4 h of age with severe cyanosis unresponsive to mechanical ventilation and oxygen supplementation. The diagnosis of rupture of the papillary muscle of the tricuspid valve was made by echocardiography. Preoperative stabilization was achieved by adding nitric oxide and extracorporeal membrane oxygenation therapy could be avoided. Corrective surgery was successfully performed on the 7th day of life. Follow-up examination at 2 months of age revealed an excellent postoperative result. Perinatal rupture of the papillary muscle and/or chordae tendineae of the tricuspid valve is exceedingly rare and usually lethal when untreated or diagnosed late. Therefore, timely diagnosis is mandatory for adequate preoperative management and subsequent surgical treatment.     

A case of mitral papillary muscle rupture due to blunt chest trauma

Mitral regurgitation due to papillary muscle rupture after blunt chest trauma is uncommon. Sudden onset severe mitral regurgitation may lead to death due to heart failure if surgical repair is delayed. A previously healthy 12-year-old girl underwent splenectomy and chest tube insertion for pneumothorax after a traffic accident in a vehicle 15 days before. She was discharged from the hospital after a nine-day follow-up. She was presented to our hospital due to respiratory distress. On physical examination, an apical holosystolic murmur radiating to the axillary region was recognized. Transthoracic echocardiogram showed severe mitral regurgitation with freely moving posterior mitral chordae and prolapse of the posterior mitral valve leaflet. She received reimplantation of the complete ruptured posteromedial papillary muscle of the mitral valve. Her medical condition improved after the operation. On the postoperative echocardiogram, the left ventricular systolic function was normal with no mitral regurgitation.     

Single papillary muscle of the left ventricle associated with persistent common atrioventricular canal: Variant of parachute mitral valve

Summary Six cases of single papillary muscle of the left ventricle in persistent common atrioventricular canal (AVC) are described. Except for one case with double outlet right ventricle and AVC, in the other five cases AVC occurred with normally related great vessels. Down's syndrome was present in two cases. The mitral valvular condition in AVC is not the classic parachute mitral valve since the separation between the common anterior and the common posterior leaflet leaves a space (cleft) oriented toward the ventricular septum. This mitral valve in AVC may or may not be obstructive. The common anterior leaflet fixed to the ventricular septum may result in obstruction. Clinically, single left ventricular papillary muscle should be suspected if the AVC is associated with coarctation of the aorta or tubular hypoplasia of the aortic arch. This type of mitral valve in AVC may also be associated with double outlet right ventricle. Preoperative recognition of the single papillary muscle by echocardiogram or left ventriculogram will prevent postoperative mitral obstruction following closure of the mitral cleft.     

Echocardiographic evaluation of mitral valve in patients with pure rheumatic mitral regurgitation

The aims of this study were to evaluate the structure of the mitral valve (MV) and subvalvar apparatus in patients with rheumatic mitral regurgitation (MR) by echocardiography and to compare the differences in morphologic abnormalities between subgroups of patients with and without mitral valve prolapse (MVP). Two-dimensional and color Doppler echocardiographic examinations were performed in 20 consecutive patients with isolated rheumatic MR and in 15 healthy subjects as controls. Annular diameter, left ventricular end-diastolic dimension, anterior leaflet length, and both leaflet thicknesses were greater in MR than those of controls. Anterior leaflet and chordal lengths were greater in severe MR than in mild or moderate MR. Sixty percent of rheumatic MR patients had nodules on the body or tip of the anterior mitral leaflet and MR was more severe in these patients. Nine of 20 patients (45%) had MVP. MR was more severe in the patients with MVP than those without prolapse. Rheumatic etiology should be suspected in patients with MR when irregular focal thickening of MV, relatively immobile posterior leaflet, eccentric regurgitant jet, and anterior MVP are found in echocardiographic study.     

Isolated Tricuspid Valve Endocarditis in a Child with a Normal Heart

Infective endocarditis localized in the tricuspid valve was observed in a previously completely healthy 10-yeardd male. The pathogen was identified as Staphylococcus uureus. Twodimensional and Doppler echocardiography revealed rupture of the chordae tendineae and prolapse of the tricuspid septal leaflet with tricuspid regurgitation. Isolated infective tricuspid valve endocarditis is mostly confined to drug addicts, and, to out knowledge, the literature contains only four cases of this disease in children without heart disease. The present case is considered to represent an extremely rare condition.