Three-dimensional morphological magnetic resonance imaging in infants and children with congenital heart disease

Background Cardiac MRI has become a clinically useful supplement to ECHO and conventional X-ray angiography in the diagnostic work-up of patients with congenital heart disease (CHD). Three-dimensional (3D) sequences are capable of depicting both intracardiac and extracardiac structures with high accuracy in adults and adolescents. However, diagnostic image quality in infants and young children has not yet been reported. Objective To apply an optimized 3D steady-state free-precession (SSFP) MR sequence in infants and children with CHD. Materials and methods In 20 patients (median age 1.8 years; ten male) with CHD, whole-chest imaging was performed with navigator-gated, isotropic 3D SSFP MRI at 1.5 T. Sequence parameters were adapted to special requirements in infancy. Measurements of intra- and extracardiac structures were performed by two independent observers and compared to spin-echo and cine gradient-recalled-echo sequences. Results Diagnostic image quality was achieved with the 3D SSFP technique in all patients, allowing the establishment of a diagnosis in all cases. Interobserver comparison of measurements from reformatted 3D SSFP datasets revealed only minor differences with standard deviations ranging from 0.3–1.3 mm for intracardiac and 0.3–0.7 mm for extracardiac anatomy (P = ns). Conclusion Isotropic 3D SSFP MRI allows reliable and accurate assessment of CHD, even in free-breathing infants and young children.     

Visibility of the origin and proximal course of coronary arteries on non-ECG-gated heart CT in patients with congenital heart disease

Background: There is little information on the ability of non-ECG-gated cardiac CT to demonstrate the coronary arteries of children. Objective: To evaluate the visibility of the origin and proximal course of coronary arteries on non-ECG-gated cardiac CT, in which the coronary artery was not of primary diagnostic concern, in children with congenital heart disease. Materials and methods: From December 2002 to March 2004, 126 cardiac CT examinations from 104 children (median age 11 months; age range 1 day to 15 years) were evaluated. All patients had ventriculo-arterial concordance and no malformations of the great arteries; those with coronary artery anomalies were excluded. Contrast-enhanced 16-slice spiral CT was performed without ECG-gating and multiplanar images for coronary arteries were obtained. The visibility of coronary artery origins was graded on a three-point scale, while nine segments of the arteries were graded on a four-point scale. CT images in which it was possible to trace the coronary arteries were considered diagnostic. The visibility of each whole coronary artery and the origins and proximal four segments of coronary arteries were calculated. The visibility of coronary arteries was also correlated with patient age. Results: The percentage of CT images of diagnostic quality was 49.3% for the whole coronary artery and 81.7% for the origins and proximal four segments. There was a significant positive correlation between the visibility of coronary arteries and age. Conclusions: Non-ECG-gated cardiac CT, in which the coronary artery is not of primary diagnostic concern, is frequently able to visualize the origin and proximal course of coronary arteries and may be helpful in detecting coronary artery anomalies in children with congenital heart disease.     

Evaluation of image quality and radiation dose at prospective ECG-triggered axial 256-slice multi-detector CT in infants with congenital heart disease

Background

There are a limited number of reports on the technical and clinical feasibility of prospective electrocardiogram (ECG)-gated multi-detector computed tomography (MDCT) in infants with congenital heart disease (CHD).

Objective

To evaluate image quality and radiation dose at weight-based low-dose prospectively gated 256-slice MDCT angiography in infants with CHD.

Materials and methods

From November 2009 to February 2010, 64 consecutive infants with CHD referred for pre-operative or post-operative CT were included. All were scanned on a 256-slice MDCT system utilizing a low-dose protocol (80?kVp and 60?C120?mAs depending on weight: 60?mAs for ??3?kg, 80?mAs for 3.1?C6?kg, 100?mAs for 6.1?C10?kg, 120?mAs for 10.1?C15?kg).

Results

No serious adverse events were recorded. A total of 174 cardiac deformities, confirmed by surgery or heart catheterization, were studied. The sensitivity of MDCT for cardiac deformities was 97.1%; specificity, 99.4%; accuracy, 95.9%. The mean heart rate during scan was 136.7?±?14.9/min (range, 91?C160) with a corresponding heart rate variability of 2.8?±?2.2/min (range, 0?C8). Mean scan length was 115.3?±?11.7?mm (range, 93.6?C143.3). Mean volume CT dose index, mean dose-length product and effective dose were 2.1?±?0.4?mGy (range, 1.5?C2.8), 24.7?±?5.9?mGy·cm (range, 14.7?C35.8) and 1.6?±?0.3?mSv (range, 1.1?C2.5), respectively. Diagnostic-quality images were achieved in all cases. Satisfactory diagnostic quality for visualization of all/proximal/distal coronary artery segments was achieved in 88.4/98.8/80.0% of the scans.

Conclusion

Low-dose prospectively gated axial 256-slice CT angiography is a valuable tool in the routine clinical evaluation of infants with CHD, providing a comprehensive three-dimensional evaluation of the cardiac anatomy, including the coronary arteries.     

Evaluation of obstructive airway lesions in complex congenital heart disease using composite volume-rendered images from multislice CT

Background Multislice CT (MSCT) allows high-quality volume-rendered (VR) and composite volume-rendered images. Objective To investigate the clinical usefulness of composite VR images in the evaluation of the relationship between cardiovascular structures and the airway in children with complex congenital heart disease (CHD). Materials and methods Four- or 16-slice MSCT scanning was performed consecutively in 77 children (mean age 6.4 months) with CHD and respiratory symptoms, a chest radiographic abnormality, or abnormal course of the pulmonary artery on ECHO. MSCT scanning was performed during breathing or after sedation. Contrast medium (2 ml/kg) was administered through a pedal venous route or arm vein in all patients. The VR technique was used to reconstruct the cardiovascular structures and airway, and then both VR images were composed using the commercial software (VoxelPlus²; Daejeon, Korea). Results Stenoses were seen in the trachea in 1 patient and in the bronchi in 14 patients (19%). Other patients with complex CHD did not have significant airway stenoses. Conclusions Composite VR images with MSCT can provide more exact airway images in relationship to the surrounding cardiovascular structures and thus help in optimizing management strategies in treating CHD.     

Radiation dose for thoracic and coronary step-and-shoot CT using a 128-slice dual-source machine in infants and small children with congenital heart disease

Background  

For coronary artery visualization, retrospective ECG-gated acquisition by dual-source computed tomography (DSCT) was superior to spiral non-ECG-gated acquisition in a paediatric population of congenital heart disease (CHD) patients. However, retrospective cardiac CT is associated with substantial radiation doses to the patient. Recently, DSCT with end-systolic reconstruction was found to be robust for imaging the coronary arteries in patients with high heart rates.     

Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease.     

Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT

Background Precise visualization of the pulmonary vasculature is mandatory for adequate treatment of patients with pulmonary atresia and ventricular septal defect (PA-VSD). Aortopulmonary collateral arteries (APCs) can be visualized by selective injections of contrast agent in the catheterization laboratory.Objective To evaluate multidetector CT (MDCT) and different image postprocessing methods for analysis of complex pulmonary blood supply in patients with PA-VSD.Materials and methods Eight patients (6 weeks to 27.8 years of age) with PA-VSD and APCs underwent MDCT and cardiac catheterization. Using multiplanar reformatting, volume rendering and semiautomatic segmentation algorithms, the aorta, pulmonary arteries and APCs were displayed. MDCT and cardiac catheterization were analyzed by two independent observers.Results MDCT accurately imaged central pulmonary arteries (n=8), aortopulmonary shunts (n=2), right ventricular to pulmonary artery conduits (n=2) and origin, course and intrapulmonary connections of APCs (n=25), compared to X-ray angiography. A high correlation was found between the MDCT vessel diameter measurements by two independent observers (n=70, r=0.96, P<0.01) and between MDCT and angiographic vessel diameter measurements (n=68, r=0.96, P<0.01).Conclusions Using three-dimensional imaging software, a complex pulmonary blood supply can be non-invasively and accurately imaged with high-resolution MDCT. This technique may help to reduce the number of cardiac catheterizations or guide interventional or surgical therapy.Electronic supplementary material Supplementary material is available for this article at     

Planispheric multiplanar reformatted CT: a new method for evaluation of paediatric congenital spine abnormalities

We describe an original reconstruction method for spine CT performed in four patients with single or multiple congenital spine abnormalities. Conventional radiographic imaging is at the forefront of diagnosis and follow-up of congenital scoliosis, but is frequently difficult to interpret. Three-dimensional CT reconstruction facilitates visualization of complex anatomic structures, but does not give a reliable assessment of failures of segmentation. Mental three-dimensional reconstruction of the information displayed by classical multiplanar reformatted CT remains difficult. Planispheric reformatted imaging allows the visualization of all deformities in a single plane.     

小婴儿先天性心脏病术后的呼吸管理

目的 总结年龄小于6个月的先天性心脏病患儿术后呼吸管理经验。方法回顾性分析550例小婴儿先天性心脏病术后呼吸道管理的临床资料,心内畸形根治术占95．6％,患儿手术时年龄11h至6个月,平均年龄（3．1±2．3）个月;手术时平均体重（4．3±1．5）kg,其中体重小于5kg316例。均使用了人工呼吸机支持,麻醉清醒前均采用压力控制（PC）模式,清醒后均采用同步间歇指令通气（SIMV）＋压力控制（PC）＋压力支持（PS）模式。结果本组治愈530例,死亡20例,死亡率约3．8％,其中死于呼吸功能衰竭5例;存活510例,术后合并肺部并发症23例（占4．3％）,包括低氧血症5例,肺部感染7例,肺不张11例;再次气管插管8例。术后呼吸机辅助4～306h,平均20．8±19．3h,ICU停留时间36～384h,平均63．7±53．4h。结论呼吸道并发症是小婴儿先天性心脏病术后死亡的重要原因,重视术后呼吸道管理,有助于提高疗效,降低死亡率。     

双源CT诊断小儿心血管疾病应用进展

双源CT具有两套数据采集系统,实现了83ms的恒定时间分辨率,可对患者在不行心率干预的情况下,获得高质量血管图像和心脏显像.另外,其独特的双能量成像技术可用于评价组织血流灌注以及鉴别组织成分等,拓宽了CT的临床应用价值.双源CT能够从心脏的形态学以及功能学两方面获得心脏的信息,提高了对病变诊断的正确性.该文对双源CT的...     

婴幼儿先天性心脏病合并重症肺炎的治疗

目的：探讨婴幼儿先天性心脏病合并重症肺炎并心力衰竭的治疗及转归。方法：收集2007年1~12 月我院PICU 病房收治的先天性心脏病（左向右分流型）合并重症肺炎并心力衰竭患儿24 例, 对其治疗方法和转归等资料进行分析。结果：痊愈22 例, 死亡2 例。其中6例<6个月婴幼儿合并重症肺炎反复迁延不愈,反复出现心衰,在机械通气和气管插管条件下行体外循环、心内直视手术矫正心脏畸形,痊愈出院。其他18例患儿经积极抗感染及控制心衰治疗后,重症肺炎得到控制,心衰缓解而择期手术,其中16例痊愈,2例因并发低心排综合征、弥散性血管内凝血死亡。结论：婴幼儿先天性心脏病合并重症肺炎,在内科保守治疗效果不佳时应尽早进行手术治疗,以降低婴幼儿先天性心脏病的病死率。［中国当代儿科杂志,2009,11（8）：635-637］     

Developmental delay in infants with congenital heart disease

Summary The mental and motor development of 173 infants with congenital heart disease was assessed by means of the Bayley Scales of Infant Development and clinical neurological examinations. The relationship between age, sex, congestive heart failure, hypoxemia, hospitalization, and test results was evaluated. The presence of congestive heart failure was found to be significantly associated with both mental and motor developmental delay. Hypoxemia and hospitalization were associated with delayed motor development. Developmental delay could be recognized as early as 2 months of age. Supported in part by training grant HL 05855 and program project grant HL 10436 from the National Institutes of Health, Bethesda, Maryland     

Delayed sternal ossification in infants with congenital heart disease

A review of the lateral chest radiographs of 118 children less than two years old and suspected of having congenital heart disease yielded 11 who had delayed ossification of the mesosternum. In all 11 congenital heart disease was confirmed by cardiac catheterization, with the most frequent lesions being patent ductus arteriosus, ventricular septal defect, and atrial septal defect. A review of the lateral chest radiographs of 110 normal infants failed to demonstrate any case of delayed ossification of the sternum. If sternal segments do not appear ossified on chest radiographs of infants having no other clinical abnormalities, congenital heart disease should be suspected.     

婴儿先天性心脏病133例心导管检查术分析

目的　探讨婴儿先天性心血管畸形的心导管检查特点及并发症的防治经验。方法　分析婴儿期先天性心脏病患儿 133例 ,年龄为 5d至 12个月 ,体重 2 4～ 9 6kg。导管进路经股静脉穿刺 12 7例 ,大隐静脉剖开 6例 ,同时做动脉穿刺进路 7例。行右心导管检查 82例次 ,右室造影 96例次 ,左心系统造影 2 2例次。结果　诊断紫绀型复杂心血管畸形 74例 (其中法洛四联症 4 9例 ) ,无紫绀型心血管畸形 5 9例 ,4 7例伴严重肺动脉高压。心导管检查术非致死性有意义并发症 5 .3% ,无死亡。结论　心导管术是确诊婴儿复杂心血管畸形最准确的方法 ;心导管检查中要严密观察婴儿病情变化 ,由有经验医师细心操作 ,先天性心脏病婴儿心导管检查术比较安全。     

Tube current reduction in pediatric non-ECG-gated heart CT by combined tube current modulation

Background Dose reduction by combined tube current modulation has not been evaluated in pediatric low-dose CT. Objective This study was performed to evaluate tube current reduction in non-ECG-gated heart CT angiography in children by combined tube current modulation, and to assess the effects of body weight, age, sex, and scan direction on tube current reduction. Materials and methods From September 2004 to January 2005, non-ECG-gated heart CT examinations were performed with combined tube current modulation in 50 children (median age 1 year, range 1 month to 16 years; M/F 29/21) with congenital heart disease. Sixteen-slice spiral CT studies were obtained using a weight-based low-dose protocol (80 kVp, 50–160 effective mA). CT scans were obtained in the craniocaudal direction when leg veins (n=36) were used for IV injection of contrast agent and in the caudocranial direction when arm veins (n=13) were used. In one child who underwent a Fontan operation, both arm and leg veins were used. We calculated tube current reduction by combined tube current modulation and evaluated the effects of body weight, age, sex, and scan technique on tube current reduction. The quality of CT angiography images was visually evaluated by an experienced pediatric radiologist. Results Overall tube current reduction by combined tube current modulation was 15.8±11.1%. The reduction was variable among five body weight groups (9.3±7.9% for <4.9 kg, 14.3±9.3% for 5.0–9.9 kg, 16.4±12.5% for 10.0–19.9 kg, 25.8±9.8% for 20.0–39.9 kg, 15.9±12.7% for 40.0–59.9 kg) and was significantly different among the five groups (P=0.017). When eight children in the 40.0–59.9-kg group were excluded, age showed a significant positive correlation with tube current reduction (γ=0.4, P=0.003). There was no significant difference in tube current reduction between boys and girls. Tube current reduction was significantly greater for the caudocranial scan (21.2±9.2%) than for the craniocaudal scan (14.2±11.3%) (P=0.049). All CT angiography images were of diagnostic quality. Conclusions Combined tube current modulation reduced tube current in pediatric non-ECG-gated heart CT by 15.8% without degradation of image quality. With our CT protocol, for body weights up to 39.9 kg, age showed a significant positive correlation with tube current reduction, but there was a lower dose-saving effect in children heavier than 40 kg. Tube current reduction was not affected by sex, but was affected by scan direction. An erratum to this article can be found at     

Growth and nutritional intake of infants with congenital heart disease

Summary Poor weight and length gain of infants with congenital heart disease is generally considered to be related to inadequate nutritional intake, but no longitudinal data on growth and nutritional intake of such infants are available. We compared weight, length, subscapular and triceps skinfold thickness, energy and protein intake (24-h dietary intake records) as well as serum prealbumin and albumin of infants with cyanotic heart disease (n=8) or isolated left-to-right shunt (n=8) with those of healthy infants aged (n=8) 45–365 days. Weight, length, and combined (subscapular and triceps) skinfold thickness of the two groups with congenital heart disease (CHD) were significantly less from 183 through 365 days of age. However, energy and protein intake was similar to that of the control group from 45 through 365 days of age. Normal serum prealbumin and albumin in the infants with CHD ruled out protein-calorie malnutrition. It is concluded that a low level of food intake was not the main cause of inadequate growth and of small subcutaneous fat stores in these two small, but homogeneous, groups of infants with CHD.     

209例体重5 kg以下婴儿先天性心脏病的外科治疗及围术期处理

目的总结本院体重5kg以下婴儿先天性心脏病的外科治疗效果及围术期处理方法。方法2002年11月至2008年2月本院手术治疗5kg以下先天性心脏病患儿209例,男162例,女47例。年龄10d至10个月,其中年龄〈28d9例,28d至3个月110例,3-10个月90例。体重2．3～5妇,其中2．3～3kg10例,3～5kg199例。病种包括：法洛氏四联症（TOF）8例,完全性房室间隔缺损（CAVSD）6例,右室双出口（DORV）9例,完全性肺静脉异位引流（TAPVC）19例,完全性大血管转位（TGA）9例,主动脉弓中断（IAA）2例,室间隔缺损（VSD）和（或）房间隔缺损（ASD）156例,其中部分伴动脉导管未闭（PDA）和（或）肺动脉高压（PH）。全组均在体外循环下行心内直视手术治疗,术后均给予改良超滤,2例延迟关胸,8例放置临时起搏导线,术中及术后依据病情予米力农、多巴胺、异丙肾上腺素等持续静脉滴注,呼吸机支持时间20．5—362．0h,撤机后全部用鼻塞式持续气道正压给氧。结果本组早期（术后30d内）死亡16例（7．9％）,死亡原因包括低心排出量综合征、急性肾功能衰竭、肺高压危象、多器官功能衰竭等;术后主要严重并发症包括毛细血管渗漏综合征、低心排出量综合征、肺高压危象、肾功能衰竭、低氧血症等。193例存活者随访12个月,172例肺炎及心力衰竭的发生明显减少,体重明显增加,无远期死亡及并发症发生。结论低体重先天性心脏病患儿手术风险大,应严格把握手术指征及手术时机,重视和改进围术期各个环节,可有效降低手术死亡率和术后并发症。     

Clinical and haemodynamic correlates of heart rate variability in children with congenital heart disease

Heart rate variability (HRV) represents a noninvasive parameter for studying the autonomic control of the heart. Cardiac patients have a complex autonomic disturbance. The relation of HRV to this abnormality in children with congenital heart disease (CHD) has not yet been examined. The present study examined HRV indices from 24 h Holter recordings in 258 children with an operated or non-operated CHD, to determine their differences as an indicator of the severity of heart disease. The latter was defined clinically as New York Heart Association (NYHA) functional class I to IV and haemodynamically by invasive parameters. Five time-domain measures (SDNN, SDNNi, SDANNi, rMSSD and pNN50) and three frequency-domain measures (LF, HF and balance LF/HF) were compared with normal ranges. HRV was reduced in children with CHD, except in patients of NYHA class I. The level of reduction depended on the NYHA functional class. None of the measures was significantly related to haemodynamic data. Conclusion Heart rate variability is reduced in children with Congenital heart disease depending on the functional limitation but not on haemodynamic disturbances. Heart rate variability indices are sensitive markers of the clinical state. Received: 3 December 1997 / Accepted in revised form: 22 June 1998     

婴儿先天性心脏病93例回顾性分析

目的 探讨婴儿先天性心脏病(CHD)的临床特点,提高早期诊断率.方法 研究对象为2006年1月至2007年6月安徽省蚌埠医学院第一附属医院儿科经彩色多普勒超声心动图(CFM)诊断为CHD的住院婴儿93例,按是否是新生儿分为2组:Ⅰ组:1h至28d(n=14),Ⅱ组:28d至1岁(n=79),对其临床资料进行回顾性分析.结果Ⅰ组CHD以复合畸形为主,共7例(7.5%),以房间隔缺损(ASD)合并室间隔缺损(VSD)最多见(2.2%).Ⅱ组CHD以单纯畸形为主,共66例(71.0%),最常见的CHD类型为VSD(46.2%).两组CHD类型构成比比较有统计学意义(P＜0.05).8例合并心脏外畸形(8.6%).临床表现多样,在Ⅰ组尤其不典型,以呼吸急促、青紫、心脏杂音多见,易反复患肺炎,并发心力衰竭.结论婴儿CHD类型较多,临床表现复杂,对可疑的CHD婴儿应及时行超声心动图检查,早期诊断并制定恰当的治疗方案.     

Epidemiological study of congenital heart disease

A community based survey of congenital heart disease was carried out on a random sample of 11833 children below 15 years in Delhi, India. Congenital heart disease was diagnosed on clinical history and/or clinical examination. Out of the examined sample, 50 were found suffering from congenital heart disease, giving an overall prevalence of 4.2/1000 (4.6/1000 in boys and 3.7/1000 in girts). The prevalence rate was slightly higher than other studies carried out in the country. The reason is obvious. The present study is community based while other studies were hospital or school based. The type of lesions were similar to those reported from other studies. The lesions in order of frequency were ventricular septal defect (46%), atrial septal defect (18%), patent ductus arteriosus (14%), Fallot’s tetralogy (10%), aortic stenosis (4%), and pulmonary stenosis (4%). Like other studies ventricular septal defect was the commonest lesion. The prevalence rate was higher in the age group 0–4 years and 5–9 years in boys whereas it was higher in adolescent age group (10-14 years) in girls.