主动脉-左室隧道6例的诊断及治疗

目的 探讨主动脉-左室隧道(ALVT)的临床诊断及治疗。方法 对6例ALVT行常规体检、心电图、X线胸片、二维超声心动图、心导管检查结果、外科手术治疗及术后随访结果进行分析。结果 1．6例脉压差均增大并在胸骨左缘闻及双期杂音，4例并心功能不全；2．5例心电图左室肥厚伴T-ST改变；3．6例X线胸片示左心室增大；4．4例超声心动图均显示主动脉根部与左室间的异常交通及血流；5．6例均经左室造影显示ALVT的直接征象及造影剂返流左心室；6．6例均经外科治疗证实为ALVT且手术补片缝合。结论 ALVT是一种少见的先天性心脏病，凡婴幼儿患儿以主动脉瓣关闭不全就诊，具有典型的主动脉关闭不全体征，应考虑ALVT的可能性，结合超声心动图及心导管检查予以确诊，一旦确诊宜早期手术。     

小儿心血管疾病诊治进展

1　先天性心脏病先天性心脏病(CHD)是小儿最常见的心血管疾病之一。目前我国每年约有10～15万患有CHD的婴儿出生,部分严重、复杂的心血管畸形患儿如生后得不到及时、正确的诊治将很快夭折。近年来CHD的诊断和治疗进展较快,患儿预后明显改观。1.1　CHD的诊断　永存动脉干、三尖瓣闭锁、主动脉 左室隧道、主动脉缩窄、完全性肺静脉异位引流等几种少见的CHD的诊断,除参考心电图、X线胸片等常规检查改变外,目前应用超声心动图检查已可进行初步诊断。有作者报道了上述各病的超声心动图特征,并认为最后确诊仍需做心导管及心血管造影检查[1…     

微纤维蛋白-1与先天性二叶型主动脉瓣

先天性二叶型主动脉瓣较为常见,人群中发生率约为1%~2%,男女之比为2∶1,常伴发于其它先天性心脏病,可致主动脉瓣狭窄(AS)和(或)主动脉瓣关闭不全(AI),并发感染性心内膜炎。患者组织学异常不仅限于瓣叶,发生主动脉扩张、主动脉瘤形成、主动脉夹层的危险性较高[1-2],主动脉瓣与主     

心室内隧道法治疗儿童右室双出口并主动脉瓣下室间隔缺损

目的　探讨右室双出口 (DORV)并主动脉瓣下室间隔缺损 (VSD)儿童采用心室内隧道法的手术效果。方法　对 9例DORV并主动脉瓣下VSD儿童采用心室内隧道法纠治 ,其中 5例有肺动脉瓣及瓣下狭窄 ,1例双腔右心室。体外循环下行心室内隧道法 ,经右心室切口用涤纶片修补VSD ,形成左心室至主动脉的内隧道 ,右心室与肺动脉相连。结果　 9例术后恢复均良好 ,超声心动图显示心室内隧道功能正常 ,无血流梗阻征象。结论　掌握好术式的适应证 ,心室内隧道法是纠治DORV并主动脉瓣下VSD的满意术式     

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目的观察动脉转位术(ASO)治疗复杂先天性心脏病术后早中期超声心动图的随访结果,评价该手术后左心室的功能和大动脉的发育。方法2000年10月至2004年3月,复旦大学儿科医院对实施ASO后4~37(19.6±11.1)个月的16例患儿进行超声心动图检查,其中室间隔完整的完全性大动脉转位(TGA/IVS)8例,完全性大动脉转位合并室间隔缺损(TGA/VSD)6例,右室双出口合并肺动脉瓣下室间隔缺损(Taussig-Bing畸形)2例。将获取的资料与正常参考值作比较。结果左室短轴缩短率(LVSF)为(38.10±5.90)%,其中14例(87.5%)在正常范围,2例(12.5%)大于正常范围;左室射血分数(LVEF)为(69.16±7.01)%,均在正常范围。主动脉根部直径平均较正常值扩大(5.5±3.2)mm,其中14例(87.5%)大于正常值95%可信区间的上限;瓣环直径较正常值平均扩大(3.6±3.3)mm,其中10例(62.5%)大于正常值95%可信区间的上限。主肺动脉直径较正常值平均小(1.3±1.5)mm,其中9例(56.25%)在正常值95%可信区间内;瓣环直径较正常值平均小(1.1±1.7)mm,其中10例(62.5%)在正常值95%可信区间内。6例存有主动脉瓣轻度反流;肺动脉瓣上狭窄极轻度3例,轻度狭窄1例;2例存有肺动脉瓣轻度反流。结论超声心动图随访结果证实目前在本中心开展ASO治疗TGA等复杂性先天性心脏病术后左心室功能良好,新主动脉根部和瓣环有所扩张,新肺动脉根部和瓣环发育良好。     

超声心动图在诊断先天性紫绀型复杂心脏大血管畸形的应用

超声心动图是唯一可见心内结构的无创伤性检查方法。近来实时双维超声心动图的应用,可提供有关心脏、大血管结构及其空间关系有意义的资料,为先天性心脏病,尤其是复杂畸形的诊断开辟了新的前景。本文就M型和实时双维超声心动图对先天性心脏大血管复杂畸形(紫绀型)的诊断应用作一概述。     

3个月内婴儿先天性心脏病193例回顾性分析

目的总结3个月内婴儿先天性心脏病的临床特点,提高早期诊断率。方法对193例经彩色多普勒超声心动图诊断为先天性心脏病患儿资料进行回顾性分析,其中新生儿145例,~3个月婴儿48例。结果新生儿期先天性心脏病复合畸形占21.4%(31/145),较~3个月婴儿8.3%(4/48)明显增多,其先天性心脏病主要类型为房间隔缺损合并室间隔缺损;~3个月婴儿中以单纯畸形为主,占91.7%(44/48),其先天性心脏病主要类型为室间隔缺损。临床表现多种、无特异性,以青紫、呼吸急促、喂养困难、心脏杂音多见,易反复呼吸道感染。结论 3个月内婴儿先天性心脏病类型多,临床表现复杂,对可疑者应尽早行超声心动图检查,早期确诊及时治疗。     

先天性右位心并复杂心血管畸形的超声诊断

目的观察超声心动图对先天性右位心合并复杂心血管畸形的诊断价值。方法以心血管造影结果为对照,应用超声心动图按节段分析法评价63例先天性右位心合并复杂心血管畸形患儿的心脏解剖结构。结果63例右位心患儿中镜像心28例,右旋心35例。超声心动图和心血管造影对心脏各节段诊断特异性有高度一致性,两方法相比较差异有统计学意义(P>0.05)。超声心动图对心房方位、心室形态、房室连接、心室动脉连接及主动脉位置诊断准确率均大于90%。镜像心大多各节段连接一致,可合并房间隔缺损、室间隔缺损等简单畸形(42.9%),也存在合并肺动脉狭窄的复杂心血管畸形(50%),主要为法洛四联症、右室双出口、单心室;右旋心大多各节段连接不一致,多合并肺动脉狭窄或闭锁的复杂畸形(88.6%)如单心室、右室双出口、大动脉转位、肺动脉闭锁等。结论超声心动图对先天性右位心合并复杂心血管畸形患儿大多能做出明确诊断,可作为右位心诊断的首选或筛选方法。     

胎儿先天性心脏病产前诊断与临床意义

先天性心脏病是最常见的出生缺陷之一.孕早期先天性心脏病筛查的方法主要有颈后透明层厚度测量、静脉导管血流测量以及直接进行胎儿超声心动图检查等.孕中期绝大部分的胎儿心脏病可以直接通过胎儿超声心动图检查获得诊断,除心脏解剖结构畸形外,心律失常的诊断以及心功能的评估也是重要检查内容.加强先天性心脏病的相关基因研究,也能够为产前...     

儿童主动脉缩窄3D打印及模拟手术应用一例及文献复习

正主动脉缩窄(coarctation of aorta,Co A)是一种较常见的复杂型先天性心脏病,文献报道Co A在国内的发病率占所有先天性心脏病的3%～5%,国外则占所有先天性心脏病发病率的5%～8%。Co A常合并心内结构性畸形,部分主动脉缩窄病变涉及主动脉发育不良和变异~([1])。其临床表现多样,主要取决于主动脉的缩窄程度、有无侧枝血管形成、动脉导管是否未闭以及是否合并复杂心内畸形等。主动脉缩窄的诊断途径主要包括心脏CT、磁共振成像、心脏彩超等,但上述检查的局限性在于无法立体呈现病变结构的空间影像,特别是对于年轻医师而言,仅通过二维图像准确重建病变部位的空间结构及毗邻关系来确定手术径路显得尤为困难。近年     

Role of transesophageal echocardiography in the management of aortic-left ventricular tunnel

A 10-year-old patient has been followed for 10 years after repair of an aortic-left ventricular tunnel (ALVT) for residual aortic regurgitation and significant recurrent leak through the ALVT. Transesophageal echocardiography (TEE) was done prior to surgery and intraoperatively to (1) clarify the precise anatomy of the ALVT, (2) evaluate the degree of aortic regurgitation, and (3) assess the repair. Delineation of the anatomy of the ALVT and assessment of the residual aortic insufficiency helped with the intraoperative decisionmaking. We describe the TEE findings of a residual ALVT in this patient and discuss the role of TEE in managing the lesion.     

Use of an Amplatzer Duct Occluder for Closing an Aortico-Left Ventricular Tunnel in a Case of Noncompaction of the Left Ventricle

Aortico-left ventricular tunnel (ALVT) is a very rare congenital anomaly. Noncompaction of the left ventricle or spongy myocardium is another rare congenital cardiomyopathy. We report probably the first case in which these two rare anomalies causing pump failure coexisted in a 4-year-old male and the tunnel was successfully treated nonsurgically. Transcatheter closure of ALVT with the Amplatzer duct occluder is feasible and safe in a case of noncompaction of the LV with reduced LV function.     

Pericardial Single-Patch Repair of Right Coronary Artery From Aorto-Left Ventricular Tunnel

Aortico-left ventricular tunnel (ALVT) is a rare congenital paravalvar communication between the aorta and the left ventricle. This anomaly has been associated with the right coronary artery (RCA) arising directly from the tunnel. Surgical repair of this later condition is challenging and is usually undertaken by closure of the ventricular and aortic ends of the tunnel and transfer of the RCA. Regardless of the surgical technique, development of early and late aortic regurgitation (AR) remains a concern. We report a neonate with ALVT and the RCA coming off the tunnel that was successfully managed by closure of both ends of the tunnel, reinforcement of the annulus with autologous pericardium, and leaving the RCA perfused by way of the tunnel. After 6 months of follow-up, no AR has been shown on echocardiography.     

Sudden Death Due to Ventricular Septal Defect

Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16–31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and ½ months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.     

组织多普勒成像对早期新生儿心室功能的评价

目的 应用组织多普勒成像（TDI）检测新生儿房室环运动，探讨不同胎龄早期新生儿左右心室功能的特点。方法 采用TDI技术检测生后3—7d的36例胎龄32～36周早产儿（早产儿组）和33例足月新生儿（足月儿组）二、三尖瓣环运动，测量收缩期峰值速度（Sa）、舒张早期峰值速度（Ea）及舒张晚期峰值速度（Aa），以脉冲多普勒显像测量舒张期二、三尖瓣口血流E峰及A峰峰值速度。结果 早产儿组各部位Ea、Sa均显著低于足月儿组，早产儿组三尖瓣环Ea／Aa显著低于足月儿组，两组Aa及E／Ea无显著差异；各部位Ea、Sa与胎龄及出生体质量均呈正相关。结论 早产儿左、右室收缩及舒张功能均低于足月儿，早期新生儿心室功能与其出生前成熟程度有关，早产儿及足月新生儿生后早期左室舒张功能均明显增强。     

Ultrasound grading of cerebral ventricular dilatation in preterm neonates

OBJECTIVE: To compare three grading systems previously described for the measurement of cerebral ventricular dilatation in preterm neonates using ultrasound scans and then to compare these with expert clinical judgement. METHODS: Eighty-one preterm neonates <1500 g birthweight or < 32 weeks' gestation, with normal, mild, moderate or severely dilated ventricles recorded predominantly during the first 2 weeks of life were selected to ensure a broad representation of ventricular size. Their cranial ultrasound scans obtained nearest to 6 weeks of age were studied. The ventricular index, the diagonal width in the coronal plane and the ventricular height in the parasagittal plane were measured adjacent to the foramen of Monro and were used to grade the degree of dilatation. In 20 of these neonates, this grading was compared with grading based on expert clinical judgement. RESULTS: Grading according to the ventricular index defined 79% of the scans as normal. However, only 44% and 39% were considered to be normal according to vertical height and diagonal width, respectively. There was a high level of correlation between expert clinical judgement and grading based on ventricular height measurement (r = 0.812; area under receiver operating characteristic (ROC) curve = 0.922) and the diagonal width (r = 0.806; area under ROC curve = 0.935). Ventricular index correlated poorly with clinical judgement (r = 0.298; area under ROC curve = 0.604). CONCLUSIONS: The measurement of ventricular height and the measurement of diagonal width correlated well with expert clinical judgement. The ventricular index correlated poorly and was less sensitive to mild dilatation. There was no correlation between the ventricular index and the other two measures. The ventricular height and the diagonal width are more appropriate for assessing ventricular dilatation in preterm neonates.     

特发性室性期前收缩患儿心率变异性和心率减速力的研究

目的探讨不同起源特发性室性期前收缩患儿的心率变异性(HRV)与心率减速力(DC)各项指标的关联性。方法回顾性分析155例特发性室性期前收缩患儿的临床资料,按不同年龄组分为婴幼儿期(3岁),学龄前期组(3~6岁)和学龄期(~16岁),并在不同年龄组内按室性期前收缩来源部位不同,分为右室型组和左室型组;比较分析不同年龄组、不同来源期前收缩组之间HRV与DC各指标的差异。结果三个不同年龄组的DC和HRV时域分析各项参数的差异均有统计学意义(P0.05)。婴幼儿组中,右室型组和左室型组间相邻RR间期差值的均方根(RMSSD)、高频功率(HF)、低频功率(LF)/HF、DC、相邻RR间期差值50 ms的百分数(PNN50)差异均有统计学意义(P均0.05);学龄前期组中,右室型组和左室型组间的RMSSD、LF、HF、LF/HF、DC差异有统计学意义(P均0.05),学龄期组中,右室型组和左室型组间的RMSSD、HF、LF/HF、DC差异均有统计学意义(P均0.05)。结论特发性室性期前收缩患儿自主神经平衡调节受损,以迷走神经张力的降低为主;起源于优势心室(婴幼儿期及学龄前期患儿以右心室占优势,学龄期患儿左心室为优势心室)的频发室性期前收缩,增加了恶性心律失常发生的风险。     

Abiomed Biventricular Assist Device as a Bridge to Transplantation in a 14-Year-Old With Cardiomyopathy Resulting from Ventricular Noncompaction

Ventricular noncompaction is a rare but well-documented cause of cardiomyopathy. This report presents a case of ventricular noncompaction diagnosed late in end-stage cardiac failure and malignant ventricular arrhythmia, which required an Abiomed biventricular assist device as a bridge to transplantation.     

Do drugs that block transforming growth factor beta reduce posthaemorrhagic ventricular dilatation in a neonatal rat model?

AIM: Posthaemorrhagic ventricular dilatation (PHVD) after intraventricular haemorrhage (IVH) remains a significant problem in preterm infants. No treatment has reduced the need for cerebrospinal fluid (CSF) diversion. Considerable evidence implicates transforming growth factor-beta (TGF-beta) in the pathogenesis of PHVD. Pirfenidone and losartan reduce TGF-beta expression and decrease postinflammatory fibrosis in the lungs, kidneys, heart and liver. They have excellent CSF and brain penetration. We hypothesized that administration of pirfenidone or losartan would reduce ventricular dilatation. METHODS: Ninety-two rat pups underwent intraventricular blood injection on postnatal days (PN) 7 and 8, and were randomised to pirfenidone, losartan or water by gavage for 14 days. Neuromotor testing was carried out twice weekly. After sacrifice at PN21, ventricular area was measured on coronal sections using image-analysis software. RESULTS: Ninety-five percent of animals undergoing IVH developed PHVD. Ventricular size was not significantly different between animals receiving either drug or water. Neuromotor testing at PN14 was significantly worse in IVH animals than in controls; neither drug improved performance in IVH animals. CONCLUSION: Drugs that block TGF-beta do not reduce ventricular dilatation in this model. Further study is required to identify other cytokine targets and to determine how PHVD differs from postinflammatory fibrosis in other organs.     

Evaluation of children with ventricular arrhythmias

Ventricular arrhythmias are rare in childhood but may be associated with syncope and sudden death. This report describes 8 children with ventricular arrhythmias, 6 of whom suffered syncopal episodes. Ventricular tachycardia was documented in 5. One boy died suddenly. Complete cardiac investigation was carried out with exercise testing, Holter monitoring, echocardiography, cardiac catheterization, angiography, and electrophysiological studies. The spectrum of abnormalities related to the arrhythmias included prolapsing mitral valve, prolonged QT syndrome, sick sinus syndrome, congenital heart disease, cardiomyopathy, and idiopathic ventricular tachycardia. Exercise testing and Holter monitoring were particularly useful in documenting the arrhythmias and monitoring response to therapy.