Anterolateral muscle bundle of the left ventricle in atrioventricular septal defect: Left ventricular outflow tract and subaortic stenosis

Summary The anatomy of the left ventricular outflow tract (LVOT) in 77 hearts with atrioventricular septal defect (AVSD), 36 with a separate AV orifice and 41 with a common AV-orifice, were investigated.In all specimens, an anterolateral muscle bundle of the left ventricle was identified between the superior bridging leaflet and the left coronary aortic cusp. It displaced the attachment of the superior bridging leaflet, resulting in its clockwise rotation. The muscle bundle frequently bulged into the LVOT, but was never prominent enough to have caused significant subaortic stenosis.Measurement of the LVOT aortic ratio was possible in 54 hearts and ranged from 36–100%. In 23 cases (43%), there was mild to moderate subaortic narrowing with a ratio ranging from 53–88%. In six cases (11%), unequivocal subaortic stenosis was present, mainly in AVSD with separate AV orifices (five of six) and iatrogenic in one case with surgically corrected complete defect.A decreased ratio was mainly due to decreased anteroposterior width of the septum in the subaortic area, with anterior displacement of the superior bridging leaflet in cases with dense septal attachment of the superior bridging leaflet (i.e., in AVSD with separate AV orifices, type A complete defect with small ventricular septal defect, or surgically corrected complete defect).Significant subaortic stenosis was caused by hypertrophy of the ventricular septum in the subaortic area with anteroseptal twist in four cases, by anomalous chordal insertion of the superior bridging leaflet in one case, and iatrogenic in one case after surgical correction with left AV valve replacement in a type C complete defect. Other additional obstructive forces were an anomalous papillary muscle, a small left ventricle, and an aneurysm of the membranous septum.     

Right ventricular outflow tract tachycardia in children

OBJECTIVE: To assess the clinical spectrum of right ventricular outflow tract tachycardia and its management in children. STUDY DESIGN: Five centers identified patients for retrospective review. Patients (age <18 years) demonstrating ventricular tachycardia with an inferior axis and left bundle branch block were included. Patients with structural heart disease, myocarditis, cardiomyopathy, or long QT syndrome were excluded. Demographics, clinical presentation, investigations, and treatment were analyzed. Holter data were used to quantify ectopy. RESULTS: Patients (n = 48) were referred for evaluation of incidental findings (39/48), near syncope or syncope (7/48), or other (2/48). Investigations included magnetic resonance imaging (51%), endomyocardial biopsy (25%), and angiography (23%). Medical treatment was initiated in 26 of the 48 patients. The most common indications for treatment were frequent ectopy and symptoms. Medical treatment (P <.007) and observation alone (P <.02) were both associated with a reduction in ectopy. Symptoms persisted in 3 of 13 patients who were treated medically and in all untreated patients. At follow-up, there were no deaths and no difference in ectopy (P <.46) between patients who were treated medically and patients who were observed. Ablation was attempted in 6 of the 48 patients (successful in 4/6). CONCLUSION: The clinical spectrum and management of right ventricular outflow tract tachycardia in children are diverse. Both medical therapy and observation alone were associated with a reduction in ectopy.     

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??Objective??To investigate the utility of the electrocardiogram??ECG?? for differentiating origins of outflow tract ventricular arrhythmias??OTVAs?? in children. Methods??From January 2009 to July 2015??94 children with OTVAs were involved in this study??who were managed by radiofrequency catheter ablation??RFCA??. The 4 types of ECG algorithm???1??V1/V2 R wave duration index and amplitude index????2??V2 transition ratio????3??Transition zone index????4??V2S/V3R index??were used to predicting the origins of OTVAs and the accuracy of prediction was compared with the results of RFCA. Results??The positive predictive rate of 4 kinds of ECG algorithm localizing the right ventricular outflow tract??RVOT?? ventricular arrhythmia in children was 78.6%??88.2%??the sensitivity was 85.5%??91.3%??and the specificity was 36.0%??68.0%. The positive predictive rate of 4 kinds of ECG algorithm localizing the left ventricular outflow tract??LVOT?? ventricular arrhythmia was 47.4%??71.4%??the sensitivity was 36.0%??68.0%??and the specificity was 85.5%??91.3%. The positive predictive rate of 4 kinds of ECG algorithm localizing the LVOT ventricular arrhythmia in children was 47.4%??71.4%??the sensitivity was 36.0%??68.0%??and the specificity was 85.5%??91.3%. However??the subgroup analysis showed that for OTVAs originating from LCC??the positive predictive value??sensitivity and specificity were much higher than RCC origin of ventricular arrhythmia??predictive value??66.67%??90.90% vs. 10.00%??15.38%??sensitivity??83.33%??100.00% vs. 20.00%??60.00%??specificity??57.58%??90.91% vs. 50%??76.47%??. Conclusion??However??the 4 kinds of ECG algorithm used in adults are limited to predict the LVOT ventricular arrhythmias in children. If LVOT origin group is divided into the LCC and RCC origin groups??the results show that in LCC group the prediction level increases significantly.     

Aneurysm of the right ventricular outflow tract: A complication of aorta-main pulmonary (central) shunt

Summary The ascending aorta-main pulmonary artery (central) polytetrafluoroethylene (PTFE) Gore-Tex shunt was introduced in 1975 by Gazzaniga and coworkers [5]. It is a widely used palliative procedure. We present a case study with an unusual late complication.An aneurysm was discovered at autopsy in a patient who was diagnosed at birth with hypoplstic right ventricle, pulmonary valve atresia, small main and branched pulmonary arteries. At 4 days of age, the patient underwent an aorta to main pulmonary artery (PTFE) Gore-Tex shunt. Subsequent echocardiogram showed dilatation of the right ventricular outflow tract. To our knowledge, this is the first reported case of right ventricular outflow tract (RVOT) aneurysm following central aortopulmonary shunt.     

Left ventricular diastolic dysfunction in bronchopulmonary dysplasia

We report 2 infants with severe bronchopulmonary dysplasia in whom left ventricular diastolic dysfunction contributed to clinical abnormalities, including pulmonary hypertension and recurrent pulmonary edema. We speculate that close monitoring for left ventricular diastolic dysfunction may assist with clinical management and improve outcomes of infants with severe bronchopulmonary dysplasia.     

The Pregnancy Heart Team Approach for the Adult with Hypertrophic Cardiomyopathy and Severe Left Ventricular Outflow Tract Obstruction

Hypertrophic cardiomyopathy (HCM), the most common single-gene cardiovascular disease, is associated with increased risk for arrhythmias, heart failure, and sudden cardiac death. The hemodynamic changes known to occur during pregnancy can exacerbate heart failure and arrhythmias in women with HCM. We present a 30-year-old woman with HCM to illustrate the benefits of multidisciplinary team management of severe left ventricular outflow tract obstruction (peak gradient >100 mmHg) for optimal maternal and fetal outcomes.     

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??Objective??To investigate the relationship between β1 adrenergic receptor??β1-AR??gene polymorphism and outflow tract ventricular premature??OTVP??in Han Chinese children. Methods??A case-control study was performed. A total of 151 children with OTVP were recruited??who were diagnosed in Beijing Children’s Hospital from January 2014 to December 2015??and 150 healthy children served as controls. Peripheral blood samples were collected from all subjects and the genotype of β1-AR were determined by PCR??followed by direct sequencing. The frequency distribution of genotypes was compared between the OTVP and control groups. The association between polymorphism of β1-AR and OTVP was analyzed. Results??The frequencies of β1-AR genotypes Arg/Arg??Arg/Gly and Gly/Gly were 50.5%??38.5% and 11% in all children. The allelic frequencies of Arg389 and Gly389 were 69.8% and 30.2%. The allelic frequencies of Arg389 in female chidren were higher than in male??P??0.005??.There were significant differences in the genotype and allele frequencies of β1-AR gene Arg389Gly between the OTVP and controls ??P??0.012??P??0.002????and Arg389 allele was a risk factor ??OR??1.733??95%CI??1.218??2.466??. No differences of genotype were observed between different genders in children with OTVP??P??0.088????but there were significant differences in the genotype between loads of ventricular premature and nonsustained ventricular tachycardia??P??0.029??P??0.019??. Conclusion??The gene polymorphism of Arg389Gly in β1-AR might be associated with the OTVP in Han Chinese children and Arg389 allele gene is a risk factor. The genotype of Arg/Arg might be a genetic susceptibility factor of high-level loads of ventricular premature and nonsustained ventricular tachycardia in children with OTVP.     

Left ventricular thrombosis during infancy: Report of two cases

Two patients with left ventricular thrombosis diagnosed by echocardiography are presented. The first patient was a 6-week-old girl with supraventricular tachycardia. Cross-sectional echocardiography showed a rounded and mobile structure protruding from the left ventricular wall. The girl was in heart failure and had signs of peripheral embolization. After sinus rhythm had been restored the thrombus diminished gradually and the girl recovered. The second patient was a newborn boy with severe aortic stenosis and a large massive thrombus along the left side of the interventricular septum. The boy died after valvotomy, the postmortem examination confirmed the diagnosis of a large thrombus. Left ventricular thrombosis is uncommon in neonates and infants but may appear secondary to abnormal hemodynamics.     

Left ventricular muscle volume in children and young adults

Summary A computer-assisted method of estimating left ventricular muscle volume and its clinical application in 193 children and young adults is described. Wall thickness was measured at the lower half of the left cardiac border in the angiographic posteroanterior projection of the left ventricle, where only three points are marked when the left ventricular contour is traced. The intra- and interobserver reproducibility of the wall thickness were both within 1 mm. The individual beat-to-beat variability was 0.6 mm, 10% of the mean thickness. In normal left ventricles, the ratio between ventricular muscle volume and end-diastolic volume, the muscle volume index (MVI), was independent of body surface area, being 1.01+0.14 (mean±SD,n=28). MVI was normal in ventricles which were volume loaded secondary to a ventricular septal defect (n=9) or ductus arteriosus (n=11). It was significantly increased in aortic coarctation (1.66±0.44,n=24,P<0.001) and in valvar aortic stenosis (1.34±0.31,n=21,P<0.01). In tetralogy of Fallot (n=34) MVI was normal. MVI was significantly reduced in the case of pressure and possibly volume-underloaded left ventricles of simple transposition of the great arteries (TGA) (0.53±0.15,n=15) at the age of one year, compared to normal (P<0.001) and to the mean in TGA shortly after birth (P<0.05). However, it increased (P<0.001) to normal after banding of the pulmonary artery in TGA. This study was supported in part by grants HE 769-1 from Deutsche Forschungsgemeinschaft and 202a from Bundesministerium für Forschung, D-5300 Bonn, FRG.     

Value of fetal cerebral MRI in sonographically proven cardiac rhabdomyoma

Background Tuberous sclerosis complex (TSC) is an autosomal dominant phakomatosis associated with intracardiac rhabdomyomas. Objective The aim of our study was to examine the value of cerebral MRI in diagnosing TSC in fetuses with intracardiac rhabdomyomas, applying the TSC Consensus Conference (TSCCC) criteria. Materials and methods In a prospective manner six consecutive fetuses with cardiac rhabdomyomas (21–34 weeks’ gestation) underwent cerebral MRI. The MRI results were correlated with clinical follow-up at 10–34 months after birth, histology, and genetic data. Results In five of the six fetuses the diagnosis of TSC was established. In two of five fetuses MRI demonstrated cerebral manifestations of TSC that correlated well with severe epilepsy manifesting during the follow-up period. In another two of five fetuses MRI as well as clinical follow-up were normal. One of five pregnancies was terminated and histology demonstrated microscopically small subependymal nodules not demonstrated by MRI. Conclusion The results of our study agree with the available literature that fetal MRI is sufficient for the detection of cerebral lesions in TSC and should be better promoted. The TSCCC criteria can also be applied to fetal MRI.     

Left ventricular thrombi in three children with dilated cardiomyopathy: Diagnostic procedure and clinical course

Summary In three of nine children with dilated cardiomyopathy (aged 1–9 years), left ventricular thrombi were diagnosed and followed by echocardiography. Thrombi recurred in two patients, in one of them, embolized to the cerebral arteries. Resolution of the thrombi was observed under therapy directed against platelet aggregation. However, this did not prevent thrombus formation.     

Left ventricular pseudoaneurysm diagnosed by magnetic resonance imaging in a nine-year-old boy

Summary An asymptomatic 9-year-old boy was found to have a cardiac murmur in a heart disease survey. Two-dimensional echocardiogram identified a large left ventricular pseudoaneurysm. Color flow imaging showed a transtunnel to-and-fro mosaic pattern between the pseudoaneurysm and left ventricle. ECG-gated magnetic resonance imaging demonstrated the exact size and related location of the left ventricular pseudoaneurysm and its ostium. Without cardiac catheterization or angiography, he was successfully operated on by closure of the communicating tunnel.     

Left Ventricular Diastolic Function of Children in High Blood Pressure Tracking Group.

The purpose of this study was to determine whether left ventricular (LV) diastolic function in children with high blood pressure (BP) is abnormal. We measured the corrected LV isovolumic relaxation time (IRT), peak velocity of increase in LV dimension (dD/dt) and the LV muscle volume in a high systolic BP tracking group (10 boys and 22 girls) and a low BP tracking group (22 boys and 11 girls) at 3-year intervals from the ages of 6 to 15. The corrected IRT of the high BP tracking group was significantly longer than the low BP tracking group. Left ventricular dD/dt/D of the high BP tracking group was significantly lower than the low BP tracking group from the ages of 12 in boys and 9 in girls. The left ventricular muscle volume index of both groups, however, was not significantly different. Both corrected IRT and dD/dt/D were well correlated with diastolic BP. These data suggest that children in the high BP tracking group might have LV diastolic abnormalities from age 12 or 15, in contrast to children of the low BP tracking group, without increased LV muscle volume. Therefore, it might be useful to examine BP and LV function of children from ages 12 or 15 for prevention of hypertension.     

Left ventricular growth in a patient with critical coarctation of the aorta and hypoplastic left ventricle

An infant is presented who at birth met criteria consistent with hypoplastic left heart syndrome. He was followed clinically and by 11 weeks of age demonstrated substantial growth of the left ventricle. He underwent successful repair of coarctation of the aorta and continues to do well with moderate aortic stenosis. The difficulties of predicting left ventricular growth and function are discussed, and management options are reviewed.     

Angiographic and anatomical features of subvalvar left ventricular outflow obstruction in transposition of the great arteries

Left ventricular angiocardiograms were examined from a series of patients with transposition of the great arteries (TGA). Forty-one patients with systolic pressure gradients of 15 mm Hg or more between the left ventricle and pulmonary artery were selected. Seventeen of these had the combination of a pressure gradient at subvalvar level and characteristic angiographic findings. In the anteroposterior projection, there was a persistent, irregular, linear radiolucency in the region of the mitral valve during systole. It was probably produced by the close apposition of the face of the closed mitral valve leaflets to an accentuated bulge of the interventricular septum. Two of the 17 patients subsequently died. Both hearts had endocardial fibrous thickening of the septum at the predicted site of apposition. It is suggested that in TGA the small afterload caused by a relatively low pulmonary vascular resistance may contribute to exaggerated left ventricular emptying, and that this favours mitral-septal apposition.     

母亲妊娠期糖尿病对新生儿左心功能的影响

目的：评估妊娠期糖尿病（GDM）母亲的新生儿左心功能状况。方法：GDM母亲娩出的新生儿40例（GDM组）及正常新生儿40例（对照组）作为研究对象,应用超声二维斑点追踪成像技术检测室间隔厚度、左室后壁厚度、射血分数,并计算左室旋转和扭转参数。结果：GDM组新生儿室间隔舒张厚度（0.45±0.06 mm）较对照组（0.34±0.05 mm）增大;GDM组左室后壁舒张厚度（0.45±0.17 mm）亦较对照组（0.31±0.02 mm）增大,差异均有统计学意义（P0.05）。GDM组新生儿内膜旋转峰值、外膜旋转峰值、平面旋转峰值、跨壁扭转峰值均较对照组升高,差异有统计学意义（P<0.05）。结论：GDM母亲的新生儿左心室的形态和旋转及扭转运动发生变化,心功能虽受到损害,但处于代偿状态,能维持正常的射血功能。超声二维斑点追踪成像技术可作为早期检测新生儿左心功能的良好手段。     

结节性硬化105例临床特征和基因型分析

目的 分析结节性硬化(TSC)病例的临床表型和基因型特点,提高TSC诊断水平。 方法 回顾性纳入2013年8月至2015年9月复旦大学附属儿科医院符合2012年修订的TSC诊断标准并随访的TSC病例。截取脑部、皮肤、心脏、肾脏、眼底等临床特征资料,统计<1岁、~3岁、~6岁、～13岁和~18岁各临床特征的检出率。分析TSC基因突变与临床表型的相关性。结果 105例TSC病例进入本文分析,男54例,女51例。就诊年龄2月龄至12岁。 ①就诊原因：83例（79.0%）为癫发作,8例（7.6%）为皮肤异常,5例为心脏肿瘤。 ②39例（37.1%）未行基因检测,以临床特征确诊;66例行TSC基因检测病例中,符合临床特征确诊且TSC基因检测阳性47例（44.8%）,符合临床特征确诊但TSC基因检测阴性17例（16.2%）;可能符合临床特征诊断,TSC基因检测阳性2例（1.9%）。 ③临床特征总体检出率：室管膜下结节检出91/99例（91.9%）,脑皮质结构异常81/99例（81.8%）,在各年龄段检出率相近;皮肤病变中色素脱失斑97/105例（92.4%）,面部纤维腺瘤55/105例（52.4%）,鲨革斑46/105例（43.8%）,面部纤维瘤及鲨革斑随年龄增长检出率增高;心脏横纹肌瘤25/75例（33.3%）,检出率随年龄增长降低;肾脏病变14/71例（19.7%）,眼部病变5/37例（13.5%）。 ④TSC1基因突变15/66例（22.7%）,TSC2基因突变34/66例（51.5%）;TSC2基因突变病例痉挛发作更为常见（29.4% vs 13.3%）。结论 TSC可累及多种器官,在不同年龄段脑部病变检出率相近,心脏横纹肌瘤检出率不同。基因检测有助于临床疑似病例的诊断。     

三维电解剖标测系统指引下导管射频消融治疗儿童室性早搏临床分析

目的探讨三维电解剖标测系统(Carto3)指引下儿童室性早搏(室早)经导管射频消融术治疗的临床疗效及右室流出道室早消融前后自主神经功能的改变。方法回顾分析2015年1月—2019年12月收治的42例频发室早患儿经射频消融术治疗的临床资料,比较右室流出道室早患儿术前和术后3个月的心率变异性(HRV)及心率减速力(DC)。结果室早起源于右心室流出道23例、三尖瓣环5例、右室游离壁4例、左室流出道4例、二尖瓣环及左后分支各2例。其中2例术后延迟愈合,1例复发,2例失败,手术成功率92.9%(39/42),无手术并发症。23例右室流出道室早患儿射频消融术前和术后3个月24小时动态心电图HRV指标显示,时域指标即全部正常窦性R-R间期标准差(SDNN)术后高于术前,差异有统计学意义(P<0.05)。频域指标即高频功率(HF)术后高于术前,高低频功率比值(LF/HF)术后低于术前,差异有统计学意义(P<0.05)。术后DC值高于术前,差异有统计学意义(P<0.05)。结论 Carto3指引下儿童室早射频消融术治疗安全、有效;右室流出道室早患儿表现为自主神经功能受损,以迷走神经张力...     

A large infiltrating fibrous hamartoma of infancy in the abdominal wall with rare associated tuberous sclerosis

Tuberous sclerosis is a complex autosomal-dominant neurocutaneous syndrome characterized by hamartomatous malformations of fibrous and connective tissues in various organs. Although various histologic types of soft-tissue masses can occur with tuberous sclerosis, we present a unique case of fibrous hamartoma of infancy presenting as large infiltrating cutaneous and subcutaneous masses in the abdominal wall in a 4-year-old boy with tuberous sclerosis. Although the co-occurrence of tuberous sclerosis and fibrous hamartoma of infancy is very rare, it should be considered in the differential diagnosis of subcutaneous soft-tissue masses found in children with tuberous sclerosis.     

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.