小儿心内膜弹力纤维增生症心率变异改变及其与心脏结构功能的相关性研究

目的 研究心内膜弹力纤维增生症(EFE)患儿的心率变异(HRV)改变及其与心脏结构和功能的相关性.方法 对山东省立医院儿科30例EFE患儿和20例健康儿童分别进行24h心率变异分析,得出SDNN、SDANN、PNN50、rMSSD、LF、HF及LF/HF等指标.行常规超声心动图检查,取得左房舒张未期前后径(LAED)、左室舒张末期前后径(LVED)、室间隔舒张末期厚度(IVSTd)、心脏指数(CI)、左室射血分数(LVEF)、短轴缩短率(LVFS)、二尖辩舒张早期血流速度峰值(E)、舒张晚期血流速度峰值(A)、峰值速度比值(E/A)及左房射血力(LAF)等指标.对两组的上述指标进行比较,并分析心内膜弹力纤维增生症患儿的心率变异性与心脏结构和功能的相关性.结果 与对照组相比,EFE组的LAED和LVED明显增大,IVSTd明显增厚,CI、LVEF、LVFS、二尖瓣E峰均显著降低,LAF明显增大,二尖辩A峰及E/A变化不明显;心率变异指标中SDNN、PNN50、rMSSD、LF、HF均明显降低,LF/HF升高,SDANN无明显改变;SDNN与LVEF、LVFS强相关,与CI、LAF、LVED、IVSTd中等相关,IF/HF与LAF中等相关,其余HRV指标与心脏的结构和心功能的指标间无明显的相关性.结论 EFE患儿存在HRV的异常改变,表现为植物神经的普遍受损,HRV中部分指标的改变与房室腔扩大、心内膜增厚和心功能下降有一定的相关性,部分HRV指标是相对独立的,将HRV指标与超声指标结合起来能更好的指导临床.     

儿童扩张型心肌病心率变异性分析

目的 探讨儿童扩张型心肌病(DCM)心率变异性(HRV)特点.方法 对36例DCM儿童(DCM组)和54例健康儿童(对照组)长程心率变异指标进行分析.结果 DCM组的时域指标,包括全部正常心动周期(NN间期)的标准差(SDNN)、全程每天5分NN间期平均值的标准差(SDANN)、全程相邻NN间期之差的均方根值(rMSSD)、相邻NN间期之差＞ 50 ms的心搏数占心搏总数的百分比(PNN50)明显降低,与正常对照组比较有统计学意义(P ＜ 0.05);DCM组的频域指标,包括极低频段功率(VLF)、低频段功率(LF)、高频段功率(HF)明显下降,与正常对照组比较差异有统计学意义(P ＜ 0.05);不同心功能的DCM组及2例DCM死亡病例随着心功能的恶化,HRV各指标均呈进行性下降.结论 DCM患儿的HRV明显降低,显示其存在明显的自主神经功能受损和失衡,HRV对预测DCM患儿疾病的病情发展和预后有一定实用价值.     

川崎病患儿心率变异性与冠状动脉损害的相关性研究

目的 探讨川崎病(KD)患儿的心率变异性(HRV)指标与肌钙蛋白I(cTnI)和氨基末端脑钠肽前体(NT-proBNP)的相关性及其在预后中的应用价值。方法 将130 例KD 患儿分为冠状动脉损害组(n=47, CAL 组)和无CAL 组(n=83, NCAL 组), 同期选取110 例健康儿童为对照组, 29 例非心血管疾病恢复期患儿为非KD 组。各组儿童均行长程HRV 指标检测及分析。检测KD 组及非KD 组患儿血清NT-proBNP 及cTnI 水平。结果 同年龄性别KD 组患儿正常窦性N-N 间期标准差(SDNN)、相邻N-N 间期标准差的平均值(SDNNindex)、相邻N-N 间期之差>50 ms 的心搏数占心搏总数的百分数(PNN50)、极低频功率(VLF)、低频功率(LF)和高频功率(HF)值较对照组均明显下降, LF/HF 值较对照组升高(P<0.05)。CAL 组SDNN、全部记录中每5 min N-N 间期平均值的标准差(SDANN)、SDNNindex、相邻N-N 间期差值的均方根值(rMSSD)、PNN50、VLF、LF 和HF 值均低于对照组和非KD 组, LF/HF 值高于对照组(P<0.05)。CAL 组及NCAL 组的cTnI 和NT-proBNP 水平均高于非KD 组(P<0.05)。KD 患儿cTnI 与SDNN、HF 呈负相关, 与LF/HF 呈正相关(P<0.05);NT-proBNP 与SDNN、SDANN、HF 呈负相关(P<0.05)。结论 HRV 指标对KD 患儿的CAL 判断具有一定的临床意义。     

新生儿心肌损害后心率变异的意义

目的 探讨新生儿心肌损害后心率变异(HRV)的变化及其临床意义.方法 应用Holter分析系统检测60例有心肌损害新生儿(心肌损害新生儿组)和50例无心肌损害新生儿(无心肌损害新生儿组)及160例有心肌损害的不同年龄组儿童的心电信号,对HRV参数进行分析.结果 心肌损害新生儿组窦性心率R-R间期标准差(SDNN)、每5分钟窦性心率R-R间期平均值的标准差(SDANN)、相邻窦性心博R-R间期之差>50 ms的心搏数占心搏总数的百分比(PNN50)、总功率(TP)、低频功能(LF)等与无心肌损害新生儿组比较,均无显著性差异(Pa>0.05),而相邻窦性心博R-R间期差的均方根(rMSSD)、高频功率(HF)显著低于无心肌损害新生儿组(Pa<0.05),LF/HF值显著高于无心肌损害新生儿组(P<0.01);心肌损害新生儿组与不同年龄心肌损害组中HRV比较,新生儿组SDNN、SDANN显著低于小婴儿组(Pa<0.05);而HR、LF/HF值显著高于小婴儿组(Pa<0.01);新生儿组SDNN、SDANN、rMSSD、PNN50、TP、LF、HF均显著低于婴幼儿及儿童组(Pa<0.01),HR、LF/HF值显著高于婴幼儿及儿童组(Pa<0.01).结论 新生儿心肌损害后心脏自主神经功能明显受损,HRV参数是判断新生儿心肌损害及预后判断较稳定的指标之一.     

Analysts on heart rate variability and deceleration capacity of heart rate in preschoolers with viral myocarditis

目的 探讨病毒性心肌炎患儿心率变异性(HRV)与心率减速力(DC)各项指标变化的临床意义.方法 对56例病毒性心肌炎患儿与58例正常对照儿童进行24h动态心电图检查,应用美国DMS公司的动态心电分析系统软件,对HRV及DC各项参数进行分析对比;并分析DC与其他各项指标之间的相关性.结果 与正常对照儿童比较,病毒性心肌炎患儿时域分析的各项参数包括正常窦性RR间期的标准差(SDNN)、每5 min时段内平均正常窦性RR间期的标准差(SDANN)、全程RR间期差的均方根(RMSSD)及频域分析的低频功率(LF)的差异均无统计学意义(P＞0.05),而高频功率(HF)显著减低,差异有统计学意义(P＜0.05),且DC显著减低,差异有统计学意义(P＜0.01).相关性分析中,DC与SDNN、LF、HF均成正相关,其中与HF的相关性最强(r=0.51,P＜0.01).结论病毒性心肌炎患儿存在迷走神经功能受损,心率变异性中的HF与DC反映迷走神经功能的指标均显著降低,且两者具有很大相关性.     

婴幼儿血管瘤患者口服小剂量普萘洛尔前后动态心电图的比较

目的探讨口服小剂量普萘洛尔在治疗婴幼儿血管瘤过程中对患儿心率变异性(HRV)、心率加速力(AC)、心率减速力(DC)以及心脏传导功能等方面的影响。方法对118例1岁以内血管瘤患儿口服小剂量普萘洛尔[1 mg/(kg·d)]治疗前及治疗1个月后行24 h动态心电图检查,观察治疗后HRV时域指标[RR间期总体标准差(SDNN)、RR间期平均值的标准差(SDANN)、相邻RR间期差值的均方根(RMSSD)、NN50占所有NN间期个数的百分数(PNN50)]与频域指标[低频功率(LF)、高频功率(HF)]、AC、DC的变化,同时观察治疗后有无心脏传导功能及其他方面的异常。结果普萘洛尔治疗后SDNN、RMSSD、LF、HF、PNN50均大于治疗前(P0.01);治疗后AC、平均心率(HR)、最慢心率均小于治疗前(P0.01)。治疗后24 h动态心电图结果异常比例高于治疗前,但差异无统计学意义。结论普萘洛尔治疗婴幼儿血管瘤可抑制交感神经活性,使心脏传导功能受阻,但不会造成严重不良后果。     

窒息新生儿心率变异性的临床研究

目的 探讨窒息新生儿心率变异性(heart rate variability,HRV)与心肌损害的相关性.方法 对53例窒息新生儿与40例健康新生儿进行24h全程动态心电图记录,对24h窦性心律进行HRV时域分析,时域分析指标包括:正常窦性R-R间期的标准差(SDNN);全程记录中每5分钟R-R间期平均值的标准差(SDANN);全程记录中每5分钟R-R间期标准差的平均值(SDNNindex);相邻R-R间期差值的均方根值(rMSSD);正常R-R间期标准差＞50 ms的百分比(PNN50).结果 (1)窒息新生儿与正常对照新生儿24h最快心率、最慢心率、平均心率比较,差异均有统计学意义(P＜0.05);轻、重度窒息新生儿间以上指标比较差异也有统计学意义(P＜0.05);重度窒息3种心率指标明显降低.(2) HRV时域分析中,轻、重度窒息新生儿SDNN、SDANN与正常对照新生儿比较差异有统计学意义(P＜0.05),轻、重度窒息SDNN、SDANN、SDNNindex、rMSSD及PNN50 5项指标组间比较差异无统计学意义(P＞0.05).SDNNindex、rMSSD及PNN503组间未见明显差异.结论 新生儿窒息心肌损害会导致心脏自主神经功能受损并影响心率的变化,受损程度与窒息缺氧严重程度有关.     

学龄前儿童病毒性心肌炎心率变异性与心率减速力分析

目的探讨病毒性心肌炎患儿心率变异性(HRV)与心率减速力(DC)各项指标变化的临床意义。方法对56例病毒性心肌炎患儿与58例正常对照儿童进行24 h动态心电图检查,应用美国DMS公司的动态心电分析系统软件,对HRV及DC各项参数进行分析对比;并分析DC与其他各项指标之间的相关性。结果与正常对照儿童比较,病毒性心肌炎患儿时域分析的各项参数包括正常窦性RR间期的标准差(SDNN)、每5 min时段内平均正常窦性RR间期的标准差(SDANN)、全程RR间期差的均方根(RMSSD)及频域分析的低频功率(LF)的差异均无统计学意义(P>0.05),而高频功率(HF)显著减低,差异有统计学意义(P<0.05),且DC显著减低,差异有统计学意义(P<0.01)。相关性分析中,DC与SDNN、LF、HF均成正相关,其中与HF的相关性最强(r=0.51,P<0.01)。结论病毒性心肌炎患儿存在迷走神经功能受损,心率变异性中的HF与DC反映迷走神经功能的指标均显著降低,且两者具有很大相关性。     

小儿扩张型心肌病心率变异性分析

目的分析扩张型心肌病(DCM)儿童的心率变异性(HRV)。方法DCM儿童23例(研究组),匹配健康儿童23例为对照(对照组)。采用康泰TLC3000A12通道动态心电图(EKG)分析系统描记24hEKG,分析心率、HRV的时域指标和频域指标。应用SPSS11.0软件进行统计学处理。结果与对照组比较,研究组最低心率明显增高(P<0.05),最高心率稍增高(P>0.05);HRV时域指标SDNN、SDANN、pNN50明显降低(P<0.05),rMSSD稍降低(P>0.05);HRV频域指标TP、ULF明显降低(P<0.05),VLF、LF、HF、LF/HF稍增高(P>0.05)。结论DCM儿童自主神经功能明显受损。     

正常儿童动态心电图窦性心率及心率变异分析

目的探讨正常儿童心率及心率变异性(HRV)特点。方法对804例正常儿童进行24h全程动态心电图检查,分析心率及HRV。结果不同年龄儿童窦性心率范围不同,年龄越小心率越快;儿童不同性别间HRV中24h内全部正常心动周期的标准差(SDNN)、24h内每5minNN间期标准差的平均值(SDNNindex)、NN50占所有N-N间期个数的百分数(PNN50)、极低频率(VLF)、低频(LF)差异有显著性;儿童不同年龄组间24h内5min节段平均心动周期的标准差(SDANN)、VLF、LF、全程相邻NN间期之间的均方根值(rMSSD)差异有显著性;儿童组与成人正常参考值中SDNN、SDANN、rMSSD差异有显著性,rMSSD儿童组中明显高于成人组。结论HRV是一种反映自主神经活性及其平衡的能定量、可重复的非侵入性的检测方法,是自主神经系统与心血管系统相互制约的结果。不同年龄、不同性别间儿童HRV存在显著差异;儿童组与成人参考值存在显著差异,尤其rMSSD儿童明显高于成人,提示儿童的自主神经功能较成人活跃,而随着年龄增长自主神经功能减退,尤其是迷走神经的紧张抑制功能明显减退。     

Iodine and hypothyroidism in neonates with congenital heart disease

AIM—To evaluate the influence of the intravenous injection of iodine during cardiac catheterisation, and of topical iodine antiseptics during surgical procedures, on thyroid function in full term neonates.
METHODS—Twenty one full term infants with major cardiac anomalies who survived for more than a month were studied. Thyroxine and thyrotropin concentrations were measured (by radioimmunoassay) before each procedure, 24 hours after the procedure, and every week thereafter until the age of 1 month or until normal. Thyroxine values less than 64.4 nmol/l were considered low, while thyrotropin values greater than 30 mU/l were considered high.
RESULTS—Thyroid function tests before iodine exposure were within normal limits in all infants. Following catheterisation or surgery six infants had raised thyrotropin concentrations; three had low thyroxine concentrations. Two of those infants were treated with L-thyroxine.
CONCLUSION—Iodine exposure during cardiac catheterisation or surgery may induce transient hypothyroidism in term infants.

     

Treatment Strategies for Pediatric Patients with Primary Cardiac and Pericardial Tumors: A 30-Year Review

This study reviewed different types of primary cardiac and mediastinal tumors in infants and children as well as their clinical presentation and management. Altogether, 34 consecutive patients followed from 1976 through December 2005 were analyzed. Of these 34 patients, 14 (41%) underwent surgery and 20 (59%) with rhabdomyomas were managed conservatively. Histologic examination of the surgically resected tumors showed teratoma in four patients, rhabdomyoma in four patients, and hemangioma in two patients. Myxoma, myofibroma, neuroblastoma, and malignant fibrous histiocytoma were encountered in one patient each. Follow-up evaluation was completed for 97% of the patients and extended up to 30 years. Half of the nonsurgical patients with rhabdomyoma showed partial or complete spontaneous regression. One patient died after resection of a malignant histiocytoma, and one patient required a tumor-related reoperation. Freedom from tumor-related reoperation after 10 years was 91% ± 8.7%. Of the survivors, 85% were New York Heart Association (NYHA) class 1, and 100% had sinus rhythm. Spontaneous tumor regression is common in rhabdomyoma and surgery, and is indicated only for symptomatic patients with hemodynamically significant intracardiac obstruction. For all other benign primary cardiac tumors, complete resection usually can be accomplished with good results. Patients with giant tumor masses compressing or infiltrating the heart frequently cannot undergo complete resection. For these patients, restoration/preservation of sufficient heart function is the primary goal. Malignant tumors are extremely rare in pediatric patients and have a very poor prognosis.     

Cardiac mucormycosis in a child with severe aplastic anemia: a case report

Mucormycosis is an uncommon, severe, life-threatening fungal infection in the immunocompromised host. Mucormycosis with aplastic anemia is seen rarely. Only a few cases of cardiac mucormycosis with aplastic anemia have been reported in the literature. The authors present a case with severe aplastic anemia that did not respond to classic and immunosuppressive treatment for disease and developing invasive cardiac mucormycosis despite empiric treatment for febrile neutropenia.     

儿童线粒体病心脏损害23例临床分析

目的探讨儿童线粒体病心脏损害的临床表现。方法对23例线粒体病患儿临床资料进行回顾性分析,包括询问病史、体格检查、心电图、超声心动图和心肌酶谱、病理检查、分子遗传学检查。结果15例线粒体脑病-乳酸血症-卒中样发作综合征(MELAS综合征)患儿中,9例行心电图检查6例异常;9例行超声心动图检查,1例发现肥厚性心肌病;12例行心肌酶谱检查,6例CK-MB质量升高,1例心肌肌钙蛋白I(cTnI)升高。8例亚急性坏死性脑脊髓病(Leigh综合征)患儿中,5例行心电图检查,4例异常;2例行超声心动图均未发现异常;6例行心肌酶谱检查,3例CK-MB质量增高。13例MELAS综合征患儿行分子遗传学检测,9例发现外周血白细胞线粒体DNA(mtDNA)存在3243位点A→G突变。6例Leigh综合征患儿行分子遗传学检测,2例发现外周血白细胞mtDNA存在8993位点T→C突变。结论线粒体病并心肌损伤常见,且会导致心肌病发生。     

Fetal and Neonatal Cardiac Tumors

Primary tumors of the heart are uncommon in the fetus and neonate. Nevertheless, the widespread use of new imaging techniques has contributed significantly to earlier diagnosis, treatment, and thus improved survival. The clinical findings, imaging studies, pathology, and outcome of 224 fetuses and neonates with cardiac tumors collected from the literature are evaluated and discussed. Most tumors are benign, and of these rhabdomyoma is the most common, followed by teratoma, fibroma, oncocytic cardiomyopathy, vascular tumors, and myxoma. Malignant and metastatic tumors are described but are rare. Murmurs, arrhythmias, cyanosis, respiratory distress, and cardiac failure are the main presenting signs of cardiac tumors in the perinatal period. Disturbances in hemodynamic function are correlated with the size and location of the tumor. Cardiac vascular tumors have the best outcome, whereas malignant tumors have the worst. The purpose of this review is to concentrate on the fetus and neonate in an attempt to determine the various ways cardiac tumors differ clinically and morphologically in this age group from those occurring in older children and adults and to show that certain types of tumors have a better prognosis than others.     

Young athlete with sudden cardiac arrest treated with therapeutic hypothermia

Reported herein is a coronary anomaly that occurred in a young adolescent athlete who presented with cardiopulmonary arrest. The patient was resuscitated and treated with therapeutic hypothermia. The patient had no associated neurological complications at follow up. Enhanced computed tomography of the heart indicated an anomalous left main coronary artery originating from the right coronary sinus and coursing between the aorta and the pulmonary artery. The patient underwent surgical intervention with coronary artery bypass grafting to prevent symptom recurrence.     

Vitamin D deficiency and its relationship with cardiac iron and function in patients with transfusion-dependent thalassemia at Chiang Mai University Hospital

Background: Vitamin D deficiency is common in patients with thalassemia. Vitamin D deficiency could be related to cardiac dysfunction. Increased parathyroid hormone (PTH) is also known to be associated with heart failure. Objectives: To determine the prevalence of Vitamin D deficiency and to explore the impact of Vitamin D deficiency on cardiac iron and function in patients with transfusion-dependent thalassemia. Method: A cross-sectional study in patients with Transfusion-dependent thalassemia was conducted. Patients with liver disease, renal disease, type 1 diabetes, malabsorption, hypercortisolism, malignancy, and contraindication for MRI were excluded. Calcium, phosphate, PTH, vitamin D-25OH were measured. CardiacT2^* and liver iron concentration (LIC) and left ventricular ejection fraction (LVEF) were determined. Results Sixty-one (33M/28F) patients with Transfusion-dependent thalassemia were enrolled. The prevalence of Vitamin D deficiency was 50.8%. Patients with cardiac siderosis had tendency for lower D-25OH than those without siderosis (15.9 (11.7–20.0) vs. 20.2 (15.85–22.3) ng/mL); p = 0.06). Serum calcium, phosphate, PTH, LIC, cardiac T2^*, and LVEF were not different between the groups with or without Vitamin D deficiency. Patients with Vitamin D deficiency had significantly lower hemoglobin levels compared to those without Vitamin D deficiency (7.5 (6.93–8.33) vs. 8.1 (7.30–8.50) g/dL; p = 0.04). The median hemoglobin in the last 12 months was significantly correlated with D-25OH. Cardiac T2^* had significant correlation with PTH. Conclusion: Vitamin D deficiency is prevalent in patients with Transfusion-dependent thalassemia. Vitamin D level is correlated with hemoglobin level. Vitamin D status should be routinely assessed in these patients. Low PTH is correlated with increased cardiac iron. This study did not demonstrate an association between Vitamin D deficiency and cardiac iron or function in patients with Transfusion-dependent thalassemia.     

Aortic Root Abscess with Coronary Artery Involvement: Diagnostic and Surgical Management

We describe two cases of infectious endocarditis in infants complicated by aortic root abscess involving the coronary artery treated by the Ross operation. Multislice computed tomography was important in defining the diagnosis and in planning subsequent surgical management.     

Severe calcification of the aorta (porcelain aorta) associated with sarcoidosis in a pediatric heart transplant recipient

We report a unique case of severe calcification of the aorta, bilateral coronary ostial stenoses and calcification of the mitral valve and left ventricle due to sarcoidosis. The patient underwent neonatal orthotopic heart transplantation secondary to hypoplastic left heart syndrome and developed hypercalcemia with other features of sarcoidosis at 10 yr of age. The mechanism for severe extra-renal calcification localized to the allograft is poorly understood, but may involve discordant local immune modulation and/or calcification-regulation between graft and host tissues.