Computed Tomographic Angiography of Infants with Congenital Heart Disease Receiving Extracorporeal Membrane Oxygenation

Patients with respiratory and/or cardiac failure occasionally require the use of extracorporeal membrane oxygenation (ECMO), which presents an obstacle for standard imaging modalities. Computed tomographic angiography (CTA) can be used in patients with congenital heart disease, usually to define extra-cardiac anatomy in the chest. We describe the use of CTA to evaluate two infants with congenital heart disease while on ECMO. The first infant had totally anomalous pulmonary venous connection, and the second had a pulmonary sling that resulted in tracheal stenosis. The studies achieved high diagnostic quality with minimal radiation exposure. Subsequently, both infants had successful surgical repairs.     

64-Slice Multidetector-Row Computed Tomographic Angiography for Evaluating Congenital Heart Disease

This study aimed to assess critically the role of 64-slice multidetector-row computed tomographic (MDCT) angiography for evaluating congenital heart disease. The study enrolled 60 consecutive patients (median age, 4.7 years; median weight, 16.5 kg) with congenital heart disease who underwent 64-slice MDCT angiography during the period June 2006 through September 2007. The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was critically analyzed. In each of the groups, the current technique offered a clear advantage over conventional imaging and provided specific clues for surgical/interventional management. A management algorithm was evolved based on questions frequently asked about pulmonary artery anatomy. The correlation with surgical anatomy in all cases that involved surgery was excellent. Early results suggest that 64-slice MDCT angiography is a major breakthrough in cardiovascular imaging with an important diagnostic and decision-aiding role. Diagnostic cardiac catheterization, especially for evaluating great vessel anomalies, could be largely replaced by the described technique for congenital heart disease.     

Image Quality of Coronary Computed Tomography Angiography with 320-Row Area Detector Computed Tomography in Children with Congenital Heart Disease

The objective of this study was to assess factors affecting image quality of 320-row computed tomography angiography (CTA) of coronary arteries in children with congenital heart disease (CHD). We retrospectively reviewed 28 children up to 3 years of age with CHD who underwent prospective electrocardiography (ECG)-gated 320-row CTA with iterative reconstruction. We assessed image quality of proximal coronary artery segments using a five-point scale. Age, body weight, average heart rate, and heart rate variability were recorded and compared between two groups: patients with good diagnostic image quality in all four coronary artery segments and patients with at least one coronary artery segment with nondiagnostic image quality. Altogether, 96 of 112 segments (85.7 %) had diagnostic-quality images. Patients with nondiagnostic segments were significantly younger (10.0 ± 11.6 months) and had lower body weight (5.9 ± 2.9 kg) (each p < 0.05) than patients with diagnostic image quality of all four segments (20.6 ± 13.8 months and 8.4 ± 2.5 kg, respectively; each p < 0.05). Differences in heart rate and heart rate variability between the two imaging groups were not significant. Receiver operating characteristic analyses for predicting patients with nondiagnostic image quality revealed an optimal body weight cutoff of ≤5.6 kg and an optimal age cutoff of ≤12.5 months. Prospective ECG-gated 320-row CTA with iterative reconstruction provided feasible image quality of coronary arteries in children with CHD. Younger age and lower body weight were factors that led to poorer image quality of coronary arteries.     

Clinical Diagnostic Approach to Congenital Acyanotic Congenital Heart Disease in Infants and Children

Congenital heart diseases have varied presentations depending on the age of presentation. Regression of neonatal pulmonary hypertension and the timing of establishment of left to right shunt determines the onset of symptoms. Pre-tricuspid shunts generally remain asymptomatic during the childhood while large post-tricuspid shunts present with heart failure in late neonatal or early infancy period. Admixture lesions have pathophysiology similar to large post tricuspid shunts with additional small right to left shunt causing mild systemic desaturation. Murmurs are prominent in valvular heart diseases. Careful clinical assessment of features of high pulmonary blood flow, presence of absence of systemic desaturation, status of second heart sound and murmur would help to arrive at a reasonable bedside diagnosis.     

Lung Mechanics in Normal Infants and Infants with Congenital Heart Disease

The results of a study of pulmonary mechanics in 24 normal infants and 28 infants with congenital heart disease aged 1 to 32 weeks are presented. The two groups were matched for length and weight. The diagnosis of the infants with congenital heart disease varied from simple stenoses to complex septal defects and transpositions. 6 infants were studied before and after operation designed to improve their haemodynamic situation.     

Prostaglandin Availability and Association with Outcomes for Infants with Congenital Heart Disease

Data regarding availability of prostaglandin E1 (PGE) and its impact on the stabilization, transport, critical care course, and surgical outcome of infants with ductal-dependent congenital heart disease in the current pediatric healthcare environment are unknown. We sought to determine the proportion of hospitals in Texas that stock PGE and to investigate associations between PGE availability and clinical outcomes. All birth institutions listed with the Texas Department of Health and Human Services were contacted to determine PGE availability as of 2011. Outcomes of all infants admitted to our institution from 2007 to 2012 who received PGE for ductal-dependent lesions were evaluated. PGE was stocked in 50 % (n = 139) of hospitals that performed deliveries in Texas in 2011 representing 79.1 % (303, 481) of births. Hospitals that did not stock PGE had less annual births and were located a further distance from a center that provided pediatric cardiac surgical services. Patients born at a hospital that did not stock PGE had significantly greater serum lactate and creatinine (p = 0.002) and serum lactate on admission (p < 0.001). The PGE availability was not associated with hospital length of stay, postoperative length of stay, or mortality. When stratifying in TGA and HLHS subgroups, lack of PGE availability remained associated with higher creatinine, higher lactate, lower glucose, and lower pH. PGE is not universally available in all healthcare institutions providing obstetrical services. Lack of availability of PGE at an outlying hospital was associated with increased morbidity, but was not associated with mortality or length of stay.     

Pulmonary Hypertension in Congenital Heart Disease: Irreversible Vascular Changes in Young Infants

Among 280 infants under 1 year of age with congenital heart disease autopsied at the University of Colorado Health Sciences Center between 1959 and 1978, there were six instances of grade IV¹ pulmonary artery hypertension. Five were patients with ventricular septal defect (four associated with other cardiovascular malformations). The sixth was a patient with atrioventricular canal. The youngest was 2¹/₂ months of age. Advanced degrees of pulmonary hypertensive arteriopathy (grade IV or more) have been said to be rare in infants, especially under the age of 1 year. The fact that all of these cases occurred within the last few years of the study suggests the possibility of improved supportive care leading to the prolonged survival of infants who might otherwise have died prior to developing severe disease. In addition, the role of altitude in accelerating the arteriopathy must be considered in the present series. In any case, this unexpected increase in the frequency of severe pulmonary hypertensive arteriopathy should stimulate consideration of early surgical correction of the underlying cardiovascular malformation, especially in areas of relatively high altitude.     

Timing of Interventions in Infants and Children with Congenital Heart Defects

Indian Journal of Pediatrics - Congenital heart defects (CHD) are the most common form of birth&nbsp;anomalies. About one-fifth of these are critical requiring very early intervention, the...     

婴幼儿先天性心脏病术后处理及监护

目的探讨婴幼儿先天性心脏病(CHD)术后处理及监护经验。方法回顾性分析1998年5月-2006年4月本科收治264例CHD婴幼儿术后处理及监护资料。其中根治性手术256例,姑息性手术8例。结果术后早期死亡16例。其中法洛四联症(TOF)8例,完全型房室管畸形2例,室间隔缺损(VSD)并肺动脉高压(PH)3例,三尖瓣闭锁、单心室、永存动脉干各1例。死亡原因包括低心排、急性肾衰竭、Ⅲ度房室传导阻滞(AVB)、肺部感染、呼吸衰竭、心律失常等。完全性肺静脉异位引流1例术后因顽固性高碳酸血症自动出院;房间隔缺损(ASD)并动脉导管未闭(PDA)1例术后1 a PH不能缓解,死于顽固性心力衰竭;TOF 1例行姑息性流出道疏通术后3 a,再行根治手术后死于肺出血。健康生存245例,心功能Ⅰ级233例,Ⅱ级12例。结论术后正确的呼吸系统管理,系统的心电监护、积极处理心律失常和低心排、营养支持等是提高CHD患儿术后成活率的重要措施。     

婴幼儿先天性心脏病的外科治疗

近年来随着小儿心脏外科技术的进步，能有效矫治的先天性心脏病适应证的扩大，手术年龄不断降低，大部分的先天性心脏病均能在婴幼儿期间得到有效的治疗。我院从1996年2月—2001年9月对160例36月以下的婴幼儿先天性心脏病行体外循环心内直视手术，现将治疗体会总结如下。     

不同剂量舒芬太尼对小儿先天性心脏病术后镇痛镇静效果的影响

目的研究不同剂量舒芬太尼对小儿先天性心脏病(先心病)术后的镇痛镇静效果。方法前瞻性选取2010年11月-2011年7月在本院小儿心脏科行手术治疗的ASD或VSD患儿84例,年龄3～144个月(平均34.2个月);体质量5.0～37.0 kg(平均13.1 kg)。将患儿随机分为3组,分别予0.02μg.kg-1.h-1(Ⅰ组)、0.03μg.kg-1.h-1(Ⅱ组)及0.04μg.kg-1.h-1(Ⅲ组)的舒芬太尼术后持续静脉泵入维持镇静镇痛,比较各组术后拔管时间、恶心呕吐发生率、用药期间临时加用咪达唑仑的发生率、拔管后因呼吸抑制二次插管的发生率,并于术后4 h、8 h、12 h、24 h、48 h记录心率(HR)、收缩压(SBP)、呼吸频率(RR)、中心静脉压(CVP)、血氧饱和度(SPO2)、Wong-Baker镇痛评分、FLACC镇痛评分及Ramsay镇静评分。结果使用舒芬太尼0.02～0.04μg.kg-1.h-1持续镇痛镇静评分结果均满意,组间差异无统计学意义(P>0.05)。术后24 hⅡ组和Ⅲ组RR高于该年龄组正常高限,其余各时间点生命体征均在正常范围。术后拔管时间比较差异无统计学意义(P>0.05)。3组恶心、呕吐发生率分别为6.7%、14.8%、22.2%,差异无统计学意义(P>0.05)。Ⅲ组较Ⅰ组单次加用咪达唑仑的发生率低,差异有统计学意义(P<0.05)。3组均无因呼吸抑制而进行二次插管。结论舒芬太尼可以安全地应用于先心病患儿术后的镇痛镇静,使用0.04μg.kg-1.h-1剂量时对疼痛治疗效果好,并且在此剂量下需要单次加用咪达唑仑对患儿进行镇静的发生率低。     

婴幼儿先天性心脏病的外科治疗

１１３例婴幼儿先天性心脏病患儿接受手术治疗．手术病死率２．７％，术后并发症发生率１３．３％，无后遗症发生．本文重点讨论婴幼儿先天性心脏病的手术时机，麻醉及体外循环方法，手术方法，术后呼吸系统和循环系统的处理原则．     

婴幼儿先天性心脏病围术期治疗

目的探讨3岁以下婴幼儿先天性心脏病围术期治疗要点。方法回顾性分析2000年1月～2006年8月3岁以下接受心脏畸形矫治术的109例婴幼儿临床资料。男56例，女53例；年龄31d～3岁，平均13．6个月；体质量2．1～16．0kg，平均8．6kg。93例在全麻低温体外循环下行心脏畸形纠正术；16例采用深低温低流量体外循环。62例术中采用超滤技术。结果术后死亡8例（7．3％），死亡原因为低心排血综合征4例、肺动脉高压危象3例和肺部感染1例。有并发症25例（22．9％）。其中低心排血综合征6例，呼吸系统并发症18例和心包积液1例。结论提高手术和体外循环技巧，提高术后预防和治疗低心排血综合征和呼吸系统并发症是婴幼儿心脏手术成功的关键。     

先天性心脏病患儿的免疫功能状态

目的：先天性心脏疾病(CHD)患儿易发生感染,有作者认为CHD可能是DiGeorge综合征(DGS)的一部分,CHD患儿对感染的易感性与其存在不同程度的免疫缺陷有关。本文探讨CHD患儿有无免疫功能缺陷,结合文献讨论CHD与DGS的关系。方法：通过胸部X片回顾性观察因患肺炎而住院的72例单纯性和34例复杂性CHD新生儿胸腺影的大小,50例同日龄肺炎新生儿作为对照。检测28例学龄前期CHD患儿外周血淋巴细胞亚群、淋巴细胞增殖功能及外周血单个核细胞(PBMC)白细胞介素-4(IL-4)和干扰素-γ(IFN-γ)mRNA表达情况及培养上清中IL-4和IFN-γ水平,血浆IgG、IgA、IgM及C3水平。20例同年龄健康儿童作为对照。结果：所有新生儿胸片均可见到胸腺影,单纯性和复杂性CHD新生儿胸腺影大小与肺炎新生儿比较差异均无显著性(P>0.05);学龄前期CHD患儿外周血CD3+,CD4+,CD8+,CD19+及CD16+CD56+T细胞和CD4+/CD8+与对照组差异无显著性(P>0.05);血浆免疫球蛋白及C3水平与对照组比,差异也无显著性(P>0.05);PBMC加植物血凝素(PHA)和脂多糖(LPS)刺激后的每分钟脉冲数、PBMC培养上清中IL-4,IFN-γ水平和mRNA表达与对照组比较差异无显著性(P均>0.05)。结论：并非所有CHD患儿均伴有胸腺发育不全或免疫功能缺陷,CHD患儿易感染不一定是先天性免疫功能低下的表现。     

Quantification of Right and Left Ventricular Volumes in Children with Congenital Heart Disease by Multidetector-Row Computed Tomography

Multidetector-row computed tomography (MDCT) of the heart is a new diagnostic approach for the quantitative evaluation of the coronary artery in adults. However, in children, the quantitative analysis of each cardiac chamber has not been established. We attempt to clarify the feasibility and validation of ECG-gated MDCT as a quantitative diagnostic tool to assess the right and left ventricular volume in children. The study consisted of 16 patients who had definite right and left ventricle. After obtaining multislice images by MDCT, we measured the end diastolic volume of both the right and the left ventricle by direct calculation of the region of interest and calculation from the projected image of three-dimensional reconstruction by traditional formulas. The correlation between the two calculations for both ventricles is excellent (r = 0.99 for LV and 0.94 for RV, respectively). The correlations for ventricular volume between calculation of catheterization and calculation of MDCT is also good (r = 0.99 for LV 0.99 for RV, respectively). Volume measurement by ECG-gated MDCT of the right and left ventricles is well correlated with that by catheterization in children and can reduce the necessity for cardiac catheterization.