Doppler echocardiographic evaluation of left ventricular diastolic function after surgical correction of atrial septal defect during childhood

Summary Transmitral flow was studied using Doppler echocardiography with the A/E ratio as a parameter of left ventricular diastolic function in 21 patients (ages 2.5–30.0 years) who had undergone early surgical correction of an isolated secundum type atrial septal defect (ASD) compared to a healthy cohort of 21 subjects. Pre- and postoperative M-mode recordings were compared in 12 of the 21 patients to evaluate the effect of operation on interventricular septal motion (IVS) and left ventricular (LV) and right ventricular (RV) end-diastolic diameter ratio (LVDD/RVDD) as parameters of right ventricular volume overload. No significant difference in A/E ratio was found between the patient and control groups. IVS was shown to normalize in 11 of 12 patients postoperatively and to improve from paradox to flattened in 1 of 12. LVDD/RVDD increased from 1.26±0.31 to 2.10±0.51 (p=0.0008), with no significant difference remaining between the control and patient groups postoperatively. These findings support the conclusion that an intrinsic abnormality of the left ventricle is not responsible for its diastolic dysfunction in patients with ASD who develop left ventricular failure, thereby implicating an acquired abnormality. Mechanical sequelae of right ventricular volume overload were shown to normalize in all patients subsequent to operation in the present study, and therefore cannot be excluded as a cause of left ventricular failure in ASD.     

Interventricular septal motion and left ventricular function in patients with atrial septal defect

Summary In order to assess whether the paradoxical motion of the interventricular septum seen in patients with atrial septal defect (ASD) is due to a true abnormality in septal contraction, eight patients with ASD (age, 1.6–17 years) and eight age-matched control patients were studied using qualitative and quantitative two-dimensional (2D) and M-mode echocardiography. 2D-echocardiographic images recorded from the parasternal short-axis projection at the level of the papillary muscles and 2D-directed M-mode tracings at this level were obtained. Comprehensive wall motion analysis of the left ventricular (LV) endocardial and epicardial borders was performed using both fixed reference and center of mass (floating reference) models.Our results indicate that interventricular septal wall motion and function are normal in patients with ASD. The apparent paradoxical motion is due to excessive anterior motion of the entire left ventricle, and is present only when a fixed reference system is used to assess myocardial motion, but is not present when a center of mass (floating reference system) is employed. Left ventricular function assessed by % area and perimeter change, mean radial shortening fraction, and mean radial wall thickening (2D) as well as LV shortening fraction and septal and posterior wall thickening (M-mode) was not significantly different between the two groups. Standard M-mode tracings can therefore be used to assess LV function despite this apparent abnormal septal motion.     

Correlation of Plasma B-Type Natriuretic Peptide with Shunt Severity in Patients with Atrial or Ventricular Septal Defect

The goal of this study was to test the utility of bedside plasma concentration of B-type natriuretic peptide (BNP) assay as a screen for large shunts in pediatric patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). Thirty-five children at a mean age of 70 ± 129 weeks with ASD or VSD were included in the study. Nine patients had VSD and 26 had ASD. Plasma BNP values were compared with the Q _p/Q _s ratios derived from quantitative Doppler flow measurements. Mean BNP was 29 ± 42 pg/ml, with a range between <5 pg/ml and 208 pg/ml. Sixteen patients had Q _p/Q _s values >1.5 and 19 had values <1.5. The difference of mean BNP in these patient groups was statistically significant (45 ± 56 vs 14 ± 17, p = 0.03). BNP was positively correlated with shunt significance. Receiver operating characteristic curve analysis revealed a sensitivity of 69% and a specificity of 79% at a plasma BNP cut-off level of ≥20 pg/ml. Bedside measurement of BNP correlates with magnitude of ASD and VSD in children. BNP can provide information for the management of children with ASD or VSD. It can be used as part of the evaluation of a child with a preliminary diagnosis of a congenital defect.     

Radionuclide angiographic and echocardiographic quantitation of left-to-right shunts in children with ventricular septal defect

Summary Pulmonary to systemic blood flow ratios (Qp:Qs) were estimated in 16 children with ventricular septal defects using simultaneous echocardiography and radionuclide angiography, and compared to Qp:Qs measured at cardiac catheterization by the Fick principle method (Fick). When ratios of echographic left atrial dimensions (LAD) to body surface area (LAD/M²), body length (LAD/ht), and aortic root diameter (LAD/Ao) were compared to the Qp:Qs determined by Fick, the correlation coefficients were r=0.70 for LAD/M², r=0.66 for LAD/ht, and r=0.54 for LAD/Ao. The correlation coefficients between Qp:Qs by Fick, and left ventricular dimensions/M² and fractional shortening of the left ventricle were not significant. The correlation coefficients between Qp:Qs and the ratios estimated by gamma-variate and area-ratio analysis of radioisotope pulmonary dilution curves were r=0.92 and r=0.84, respectively. Thus, radionuclide angiography provided more accurate quantitation of left to right shunting through a ventricular septal defect than echocardiography. However, difficulty in obtaining adequate bolus injections of the radioisotope may result in technical failures whereas echocardiographic measurement is possible in almost all pediatric patients. Finally, the gamma-variate method cannot accurately quantitate shunt ratios greater than 3.5 to 1.Supported in part by USPHS Grant Nos. 1-T32HL07417-01 and 5-T01HL05728-09 and American Heart Association Southwest Ohio Chapter     

Left ventricular end-systolic wall stress to volume relationship before and after surgical closure of ventricular septal defect

Summary Left ventricular function was examined angiographically in 64 patients with ventricular septal defect and 13 postoperative patients with a preoperatively large shunt (postoperative group). The unoperated 64 patients were divided into three groups; small (left-to-right shunt ratio <35%), moderate (35%–50%), and large (>50%). The control group consisted of 27 patients with Kawasaki disease. For assessing left ventricular function, left ventricular shape and the end-systolic wall stress to end-systolic volume index ratio, as well as left ventricular ejection fraction were examined. Left ventricular ejection fraction was higher in the small-shunt group (p<0.05) than in the control group, but normal in the other groups. Left ventricular end-diastolic shape was normal only in the small-shunt group and more spherical in the other groups. The large-shunt group alone manifested more spherical left ventricular end-systolic shape and lower end-systolic wall stress to end-systolic volume index (p<0.001). These findings suggest that the left ventricular dysfunction is present in patients with a left-to-right shunt larger than 50%, but this change was reversible in patients who underwent early repair of ventricular septal defect.     

Validity of electrocardiographic criteria for left ventricular hypertrophy in children with pressure- or volume-loaded ventricles: Comparison with echocardiographic left ventricular muscle mass

To determine the correlation between electrocardiographic (ECG) findings and anatomy utilizing echocardiography in children with pressure- or volume-loaded left ventricles, we analyzed the preoperative ECG tracings of 19 patients who underwent surgery for significant aortic stenosis and 12 patients who underwent cardiac catheterization or surgery for clinically significant ventricular septal defects. We then compared them with a group of 21 normal controls. The left ventricular muscle mass in these patients was calculated from echocardiograms using the simplified cubed formula. Posterior and septal wall thickness and cavity size were significantly greater in the aortic stenosis group than in the normal group. Only cavity size was significantly greater in the ventricular septal defect group than in the normal group. Eighteen aortic stenosis patients (95%) and ten ventricular septal defect patients (83%) had a left ventricular muscle mass greater than 2 standard deviations above the mean for the normal group. Significant differences were found in the voltages of SV1+RV6 and in the voltage of RV6 alone between normals, aortic stenosis patients, and ventricular septal defect patientsregardless of age. Using conventional ECG criteria for left ventricular hypertrophy, the highest sensitivity in aortic stenosis patients (67%) and ventricular septal defect patients (60%) was modest. The likelihood ratio for a positive test in either group was the best for SV1+RV6 >98^th centile for age; RV6>98^th centile for age was the best single measurement. No correlation was found between voltage and any measurable hemodynamic or anatomic data. Conventional pediatric ECG criteria for left ventricular hypertrophy have only modest sensitivity regardless of whether the heart is under pressure or volume load. Because left ventricular muscle mass can be precisely determined by echocardiography, these ECG criteria should be applied cautiously.     

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目的探讨经胸超声心动图(TTE)及实时三维超声心动图在房间隔缺损(ASD)、室间隔缺损(VSD)封堵术中的临床应用价值。方法应用二维及三维超声心动图对2004-10—2005-10在天津市儿童医院就诊的24例继发孔ASD,10例VSD进行术前检查筛选,术中超声引导及术后随访检查。结果24例ASD患儿术前经TTE检测ASD直径(15·6±7·9)mm(5~26mm),所选Mmplatzer封堵器直径为(19·1±5·1)mm(5~32mm),23例封堵成功。室间隔膜部缺损直径:左室面缺损为5~9mm,右室侧口的直径为2·4~6·0mm,术中选择Amplatzer封堵器型号为4~6mm,10例VSD无残余分流。结论二维及三维TTE联合应用于ASD、VSD介入治疗具有很好的应用价值。     

Intra-utero diagnosis of anomalous right ventricular muscle bundles in association with a ventricular septal defect: A case report

Summary The morphology and natural history of anomalous right ventricular muscle bundles (ARVMB) have been described in a number of postnatal studies. Whether this is a congenital or acquired cardiac lesion remains obscure. A fetal echocardiogram performed in a 32-week gestation mother showed a large ventricular septal defect and anomalous right ventricular muscle bundles, which were easily appreciated at the ostium-infundibular level. The diagnosis was confirmed postnatally and at 6 months of age the child underwent surgical repair. This report documents the presence of ARVMB in a fetus, at a time when hemodynamics cannot explain the development of right ventricular muscle bundles. It suggests that at least, the morphologic substrate for this disease is congenital.     

Effect of increase in heart rate on interatrial shunt in atrial septal defect

Summary The effect of increases in heart rate by atrial pacing was investigated in 20 children [mean 9±4 (SD) years] with atrial septal defect. Systemic blood flow increased by 13±10 and 27±22% with a 25 and 50% increase in heart rate, respectively. Pulmonary blood flow, however, remained unaltered. Thus, rapid pacing decreased the pulmonary to systemic blood flow ratio significantly (–15±10 and –22±13%, respectively). This hemodynamic alteration was attributed to the difference in diastolic distensibility (compliance) between the left and right ventricles, and the ratio of right ventricular to left ventricular compliance (defined by dV/dP) near the end-diastolic pressure was estimated to be 6.5±4.2. It is suspected that the increase in heart rate may contribute to the lowering of pulmonary to systemic flow ratio during exercise in children with atrial septal defect.     

Diagnosis and management of acyanotic heart disease: Part II -left-to-right shunt lesions

In this review, the clinical features and management of most commonly encountered acyanotic, left-to-right shunt lesions are discussed. Patients with small defects, especially in childhood, are usually asymptomatic while moderate to large defects in infancy may present with symptoms. Hyperdynamic precordium, widely split and fixed second heart sound, ejection systolic murmur at the left upper sternal border and a mid-diastolic flow rumble at the left lower sternal border are present in atrial septal defects, holosystolic murmur at the left lower border is characteristic for a ventricular septal defect whereas a continuous murmur at the left upper sternal border is distinctive for patent ductus arteriosus. Clinical diagnosis is not usually difficult and the diagnosis can be confirmed and quantitiated by non-invasive echocardiographic studies. Whereas surgical intervention was used in the past, transcatheter methods are increasingly used for closure of atrial septal defect and patent ductus arteriosus. Small ventricular septal defects may not need to be closed whereas medium and large defects may require surgical closure. Transcatheter closure of both muscular and membranous ventricular septal defects is feasible by transcatheter methodology, but these techniques are experimental at the time of this writing     

儿童室间隔缺损封堵术后早期完全性左束支传导阻滞心电图相关指标的变化

目的观察单纯室间隔缺损(VSD)患儿介入封堵术后早期发生完全性左束支传导阻滞(CLBBB)患儿的心电图变化。方法纳入21例行膜周部VSD介入封堵且术后早期出现CLBBB的患儿,同时以性别、年龄及封堵器大小进行1:1配对,纳入同时期术后未合并任何心律失常的单纯膜周部VSD的患儿21例作为对照组。比较两组患儿组间及组内术前、术后1 d、术后3 d、术后5 d、术后30 d心电图左室电压、QT间期、心率校正的QT间期(QTc)、QT离散度(QTd)、心率校正的QT离散度(QTcd)、JT离散度(JTd)和心率校正的JT离散度(JTcd)的变化。结果两组患儿左室电压、JTcd随手术时间的变化而变化(P0.05);左室电压及QTd变化存在时间因素与分组因素的交互效应(P0.05);CLBBB组与对照组的JTcd比较差异有统计学意义(P0.05);CLBBB组与对照组的左室电压在术后3 d和术后5 d组间比较差异有统计学意义(P0.05)。结论 VSD患儿介入封堵术后有无CLBBB者早期心电图指标左室电压和JTcd有差异,其中JTcd可能对预测术后早期CLBBB的发生有参考作用。     

Cross-sectional echocardiographic measurement of right atrial and right ventricular size in children with atrial septal defect before and after surgery

Summary A total of 25 children operated on for atrial septal defect were studied by cross-sectional echocardiography before and after operation. Right atrial and right ventricular dimensions and areas were measured preoperatively and 1–4 years (median, 32 months) postoperatively. The length, width, and area of the right atrium (RA) and the right ventricle (RV) were measured in the apical and the subcostal four-chamber views. The dimension of the RV outflow tract (RVOT) was measured in the parasternal short-axis view.Preoperatively, all group mean values except the subcostal four-chamber length of the RV were significantly larger than normal.After surgery, there was a significant decrease in all of the parameters studied, except for RV length. The mean postoperative values of RA measurements in the apical four-chamber view and of RVOT were still significantly larger than normal. The RV four-chamber measurements except the apical four-chamber length were not significantly enlarged. The greatest decrease in RA and RV size occurred in the first postoperative year. Longer follow-up periods did not change the measurements significantly.     

Atrial septal aneurysm in infancy

Summary Thirteen infants were found to have atrial septal aneurysms (ASAs) on twodimensional (2D) echocardiography and their characteristics and natural course have been studied by 2D Doppler echocardiography.Atrial septal aneurysm was accompanied by atrial septal defect (ASD) or other cardiac malformations in 12 of 13 patients, and as an isolated anomaly in one patient. Spontaneous closure of ASD by aneurysmal formation of the interatrial septum was observed in 10 patients. The aneurysms of all 13 patients showed a moderate reduction in size, 14 to 25 months (mean 18 months), after these aneurysms has been first diagnosed and 10 subsequently disappeared.It is likely that ASA occurring in infancy will develop in patients with spontaneous closure of an ASD, and that such an aneurysm has a tendency to regress as the patients grows up.     

Xylose absorption before and after surgical correction of atrial septal defect (ASD) and ventricular septal defect (VSD)

The aim of the study was to ascertain whether the acceleration of physical development in children after surgery for ASD or VSD is due to improved intestinal absorption. There were 17 patients with ASD and 9 patients with VSD (aged 5–15 years) examined.Significantly incrased values of the xylose test after surgery were found in both groups. The authors suggest that the improvement in intestinal absorption is due to an increase of systemic blood flow after operation. Simultaneously, the study confirms, on natural human model, the known dependence between blood flow and the intestinal absorption rate.     

先天性心脏病术后残余室间隔缺损介入治疗及中长期随访

目的 评价先天性心脏病（先心病）术后残余室间隔缺损（VSD）介入治疗的效果及中长期随访结果。 方法 回顾性分析2002年9月至2014年5月复旦大学附属儿科医院介入治疗的先心病术后残余VSD患者的临床资料,对辅助检查、介入治疗情况和随访行描述性分析。结果 21例患者进入分析,男11例,女10例,平均年龄8.8（2.7～21）岁,平均体重26.3（10.5～53.5） kg。13例为VSD修补术后残余漏,其中2例合并主动脉弓缩窄修补术后再缩窄;5例为法洛四联症（TOF）术后残余VSD,其中1例合并频发室性早搏及短阵室性心动过速;2例为右室双出口术后残余VSD;1例为完全性大动脉转位术后残余VSD。介入治疗距离外科手术时间为11～80个月。①介入治疗中左心室造影测得VSD右心室面直径为(3.73±1.56) mm,4例右室面有多处分流,1例为左室-右房瘘;肺循环与体循环血流量之比为1.53±0.23;无中度以上肺动脉高压者。②21例患者中,1例介入治疗中建立轨道时反复发生Ⅲ度房室传导阻滞而放弃堵闭行外科手术治疗;20例（95.2%）封堵成功,其中1例应用2个堵闭器。1例合并频发室性早搏及短阵室性心动过速患儿同时行射频消融治疗成功;2例合并主动脉弓再缩窄患儿经球囊扩张后缩窄解除。介入治疗中无不良事件发生。介入治疗后即刻造影和经胸超声心动图显示少量残余分流3例。ECG均无异常发现。③20例随访1～79个月,均无明显临床症状。2例在1年随访中ECG分别出现不完全性右束支传导阻滞及Ⅰ度房室传导阻滞,继续随访6~12个月无改变;2/3例即刻有残余分流患儿在随访中残余分流消失,1例介入治疗后20个月时仍有残余分流3.4 mm,心腔大小和功能正常;无新发的三尖瓣或主动脉瓣反流,3例堵闭前存在的中重度三尖瓣反流在随访中反流均为轻度;未见堵闭器移位、脱落或血栓形成。结论 经导管堵闭介入治疗先心病手术后残余VSD是一项安全、有效和易行的方法,介入治疗后即刻和中长期随访效果好,应成为此类患儿首选的治疗方法。     

Atrioventricular septal defect: Quantitative anatomy of the left ventricle

A morphometric study was performed in 18 human hearts with atrioventricular septal defect not associated with other anomalies; 16 hearts had common atrioventricular orifices, and 2 presented separate right and left atrioventricular orifices. A total of eight parameters were analyzed, characterizing ventricular wall thickness, length and circumference of the inflow and outflow tracts, and circumference of the left orifice and aortic orifice. The data were compared with previously published patterns of normality. In addition, the volume of the aortic outflow tract was calculated. The inflow tract was shorter than the outflow tract, and the length of the diaphragmatic wall was equal to the sum of the lengths of the inflow tract and ventricular wall thickness at the level of the apex     

婴儿心脏直视手术中不同压力灌注停搏液心肌保护效果的临床观察

目的　观察婴儿体外循环心脏手术中不同压力灌注停搏液心肌保护的临床效果。方法　选择在体外循环、心脏停搏下行心室间隔缺损修补的婴儿 45例 ,按心肌保护液灌注压力不同随机分三组 :1组 ,P =6 0mmHg ;2组 ,P =45mmHg ;3组 ,P =30mmHg。观察 :①实验室指标术前、主动脉开放 (CCR)后 30min、6h、2 4h心肌酶 (CK MB、LDH)、心肌肌钙蛋白 (cTnI)浓度 ;②临床指标 :灌注诱导停搏时间 ;主动脉开放后复跳时间、自动转窦率 ;术后正性药物使用情况 ;呼吸机辅助时间、ICU(加强护理病房 )停留时间、术后住院时间。结果　①灌注压力与诱导停搏时间、复跳时间呈负相关 ,诱导停搏时间、复跳时间组 (118.7± 5 .5 )s、(2 5 .1± 16 .0 )s小于组 3(2 3.7± 5 .5 )s、(4 3.7± 2 2 .1)s,P<0 .0 5 ;②自动转窦率组 2 (93.3% )高于组 1(86 .7% )、组 3(86 .7% ) ;③ 3组在CCR后各时点CK MB、LDH、cTnI与基础值相比均有明显升高 ,P <0 .0 1;CK MB、cTnI以CCR后 30min最高 ,与其他时点相比P <0 .0 5 ;LDH以CCR后 6h最高 ,与其他时点比较P <0 .0 5。组 1CCR后 6hLDH(6 92 .2 4± 139.9)U/L明显高于组 3(5 6 9.0± 10 8.5 )U/L ,P <0 .0 5 ;④术后多巴胺、多巴酚丁胺的使用量组间各时点无统计学差异 ,停机时组 1虽高于组 3;组     

Closure of ventricular septal defect through the pulmonary artery

Summary A transpulmonary arterial approach to the closure of a high ventricular septal defect (VSD) has been used, between 1978 and 1982, in eight patients. The reasons were ease of access and the wish to overcome the problems associated with right ventriculotomy. The patients' ages ranged from three weeks to 15 months, their weight from 2.9 kg to 9 kg. The approach was used both when the VSD was an isolated anomaly and when there were major associated defects. It is in this latter group, four with aortic arch anomalies, two with additional double outlet right ventricle (DORV), that avoidance of ventriculotomy was most helpful. It was especially important in the two patients with DORV and a perimembranous, outlet subpulmonary VSD, where it was possible to close off the left ventricular outflow tract and pulmonary valve using a patch, without opening the right ventricle, which was subsequently to become the systemic ventricle. This technique obviates the need for ventriculotomy in the closure of some perimembranous outlet and doubly committed subarterial VSDs, and is the approach of choice for the closure of a perimembranous, outlet, subpulmonary VSD in DORV.     

Blade Consolidation of Multiple Atrial Septal Defects: A Novel Approach to Transcatheter Closure

The Amplatzer atrial septal defect occlusion device has gained popularity for the nonoperative closure of secundum-type defects. We describe a case of multiple atrial septal defects that were consolidated into a single defect using blade atrial septostomy for successful closure with single Amplatzer device.     

Surgical anatomy of the antrioventricular conduction bundle in anomalous muscle bundle of the right ventricle with subarterial ventricular septal defect

Summary A stillborn baby girl was found to have an anomalous muscle bundle of the right ventricle, associated with a doubly committed subarterial ventricular septal defect. The latter was separated from the area of the atrioventricular conduction bundle by muscle. Serial histologic sectioning of the specialized atrioventricular junctional area revealed that the final bifurcation of the branching bundle came astride the septum in the inferior angle of the defect. The right bundle branch bifurcated and one of its branches continued along the anomalous muscle bundle in subendocardial position. This anatomy supports the concept that the bundle, in this heart, represented an early takeoff of the moderator band. The surgical anatomy suggests that resection of the anomalous muscle bundle and closure of the ventricular septal defect might well have produced heart block. The generally accepted rule that resection of anomalous muscle bundles in the right ventricle is surgically safe should be reconsidered. During the course of this study, H.K. was supported by a grant from the Dutch Ministry of Education and Sciences grant IB/S 188.626.