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02 September 2014, Volume 29 Issue 9 Previous Issue    Next Issue

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Current pathogenesis of cardiomyopathy in children.        ZOU Run-mei， WANG Cheng. 2014, 29(9): 641-644.  DOI: 10.7504/ek2014090601 Abstract ( )   PDF (931KB) ( )   Abstract： Cardiomyopathies are defined as heterogeneous diseases of myocardium with associated structural and functional abnormalities， and their pathogenesis is unclear. Pathogenesis of cardiomyopathies involves genetic defect，congenital metabolic defects，congenital heart defect，inflammation reaction，autoimmune disorder and so on. All these mechanisms work together， among which genetic defect is the dominant factor. Further study in pathogenesis of cardiomyopathy is helpful to provide new ideas for the specific treatment of cardiomyopathy.

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Etiology，diagnosis and treatment of cardiomyopathy in children. LIU Xiao-yan，ZHANG Lei. 2014, 29(9): 644-650.  DOI: 10.7504/ek2014090602 Abstract ( )   PDF (995KB) ( )   Abstract：Cardiomyopathy in children is a rare disorder with poor outcome. According to its pathology and pathophysiology， it’s classified as dilated cardiomyopathy， hypertrophic cardiomyopathy， restrictive cardiomyopathy， arrhythmogenic right ventricular cardiomyopathy and unclassified types. The pathogenic basis for cardiomyopathy includes genetic mutation， metabolic disorder and inflammation. Diagnostic procedure is based on clinical features， family history and morphological details of heart，and genetic and metabolic disorder screening. Management of cardiomyopathy now mainly aims at improving heart function. Many more treatments are expected to improve its prognosis.

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Progress in diagnosis and treatment of the neonatal cardiomyopathies . WU Ben-qing. 2014, 29(9): 650-654.  DOI: 10.7504/ek2014090603 Abstract ( )   PDF (953KB) ( )   Abstract：The distinguishing feature of neonatal cardiomyopathies are different from other age period. The primary causes include endocardial fibroelastosis， familial hypertrophic cardiomyopathy and noncompaction of the ventricules. The secondary causes include myocarditis， perinatal asphyxia and anomalous origin of the left coronary artery with myocardial insufficiency，and metabolic disorder. The clinical presentations are atypical. Etiological diagnosis and combined treatment should be given adequate attention.

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Diagnosis and treatment of children with dilated cardiomyopathy complicated with arrhythmia. YANG Zuo- cheng. 2014, 29(9): 654-658.  DOI: 10.7504/ek2014090604 Abstract ( )   PDF (955KB) ( )   Abstract：Dilated cardiomyopathy （DCM） can be complicated with various types of arrhythmia. The size of left ventricular diameter was positively related to the occurrence of malignant ventricular arrhythmia. The diagnosis of DCM complicated with arrhythmia should exclude the cardiomyopathy induced by other causes. The medication therapy for DCM complicated with arrhythmia could choice amiodarone， or beta blockers. Part of patients should employ the methods such as radiofrequency ablation， implantation of pacemaker or implantable cardioverter defibrillator.

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Imaging examination  of cardiomyopathy in children. ZHONG Yu-min. 2014, 29(9): 658-665.  DOI: 10.7504/ek2014090605 Abstract ( )   PDF (1403KB) ( )   Abstract：Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction and are due to a variety of causes that frequently are genetic. The incidence of cardiomyopathy in children was 1.1 ~ 1.2/100，000. Classification of clinical and imaging examination is mainly according to the 2008 ESC （European Society of Cardiology） at present. Imaging modalities are mainly echocardiography and cardiac magnetic resonance imaging （CMR）. CMR can display morphological， tissue and functional characteristics of cardiomyopathy and is especially suitable for children in the diagnosis and follow-up.

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The changes of ventricular late potentials in children with vasovagal syncope of vasodepressor response. LI Yun-li， WANG Cheng， LI Fang， LIN Ping， CHU Wei-hong， RAN Jing， WU Li-jia，ZOU Run-mei. 2014, 29(9): 673-676.  DOI: 10.7504/ek2014090608 Abstract ( )   Abstracts： Objective To investigate the changes of ventricular late potentials （VLP） in children with vasovagal syncope of vasodepressor response （VVS-V）. Methods Totally 184 children ［aged from 4 to 14 years old，the average age being （10.54±2.15） years old］ had been diagnosed with VVS-V by head-up tilt table test （HUTT） （study group） from July 2006 to January 2013 in the Second Xiangya Hospital of Central South University， male 85 cases， the average age of （10.85±2.01） years old； female 99 cases， mean age （10.27±2.24） years old. Totally 105 healthy children were matched as controls. All subjects underwent VLP examination. Investigate the difference in HR， TQRS， LAS40 and RMS40 between the study group and the control group. Results Compared with the control group， in the study group heart rate （HR） decreased ［（83.96±12.27） beats/min vs（87.28±13.75） beats/min， t = -2.113， P＜0.05］，the total QRS time（TQRS） ［（84.89±12.05） ms vs （81.21±8.23） ms， t = 3.070， P＜0.05］ and high frequency and low amplitude limit（LAS40） ［（62.43±19.17） ms vs （56.79±1.83） ms， t = 2.442， P＜0.05］ were prolonged，and the root mean square plant（RMS40） increased ［（28.73±7.23） μV vs （26.89±7.36） μV， t = 2.059， P＜0.05］. Conclusions Compared to healthy controls，in VVS-V children HR reduces，TQRS and LAS40 prolong，and RMS40 increases. It suggests that VVS-V childrenare more likely to have cardiovascular incidents.

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Meta-analysis of the association between TBX1 gene mutation and conotruncal defects. QIAO Wei-hua， SHI Jia-wei， DONG Nian-guo. 2014, 29(9): 677-680.  DOI: 10.7504/ek2014090609 Abstract ( )   Abstracts： Objective To evaluate the association between TBX1 gene mutation and conotruncal defects （CTDs） using Meta-analysis. Methods Studies on the relationship between TBX1 gene mutation and CTDs were searched from the databases of Wanfang， VIP， CNKI， PubMed， Elsevier Science Direct and Cochrane Library from their establishment date to September of 2013. According to the standards of inclusion and exclusion， articles were evaluated. Poor quality studies were excluded. Relevant data were extracted from eligible studies to conduct meta-analysis. R2.15.3 software was applied for statistical analysis. Results Eight eligible studies involving 851 patients were analyzed in the study， including 6 English literatures and 2 Chinese literatures. Three studies found 15 gene mutations， and 5 studies found 26 gene polymorphisms. The results of Meta-analysis showed that the pooled TBX1 gene mutation rate was 2.13%（95％CI：0.76%—5.87%）. Conclusion The TBX1 gene mutation rate is low in CTDs patients. TBX1 gene mutation may not be the main genetic factor for CTDs.

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Clinical grading and risk factors of coronary arterial lesions in patients with Kawasaki disease. SHI Yan-yan，LIU Fang，WU Lin，MA Xiao-jing，LIANG Xue-cun，HUANG Guo-ying. 2014, 29(9): 681-686.  DOI: 10.7504/ek2014090610 Abstract ( )   Abstract： Objective To analyze the characteristics and risk factors of coronary artery lesions （CAL） in Kawasaki disease （KD） based on the clinical grading. Methods The clinical records of 1253 KD patients admitted to Children’s Hospital of Fudan university from January 2000 to June 2011 were analyzed retrospectively. Results （1）Coronary arterial lesions occurred in 304 cases with the incidence of 24.3% in KD patients. According to the clinical grading of CAL of KD， among 304 CAL patients， 32 cases （10.5%） were grade II，251 cases （82.6%） were grade Ⅲ， 13 cases （4.3%） were grade Ⅳ，and 8 cases（2.6%） were grade V， with the prevalence in KD patients of 2.6%，20.0%，1.0%，and 0.64% respectively. （2）In all CAL patients， single， double， three and four coronary branch lesions accounted for 42.4%， 29.3%，16.1% and 12.2% respectively. The lesions of left main trunk，left anterior descending branch，left circumflex branch and right coronary artery accounted for 43.5 %，18.6%，6.8 % and 31.1% of all the branches involved respectively. （3）The results of single factor analysis and multivariate logistic regression analysis showed that incomplete KD and elevated C reactive protein （＞100 mg/L） were the risk factors of grade Ⅱ CAL， while male sex， age younger than 1 year， duration of fever over 10 days， incomplete KD and low serum albumin （＜30 g/L） were the risk factors of Grade Ⅲ～ⅤCAL. Conclusions （1）Grade III CAL is the most common type in KD patients, accounting for 82.6% of CAL. （2）About 57.6% CAL occurs in two and abovetwo branches of coronary arteries. （3）The most frequently involved branch is left main trunk, followed by right coronary artery，left anterior descending branch and circumflex branch. （4）Being male， age＜1 year old， incomplete KD， long-lasting fever （＞10 d） and low serum albumin （＜30 g/L） are the independent risk factors of grade Ⅲ～ⅤCAL.

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The relationship between plasma hydrogen sulfide and flow-mediated vasodilation in children with postural orthostatic tachycardia syndrome. ZHAO Juan，LIAO Ying，YANG Jin-yan，LIN Jing，LIU Ping，WANG Yu-li，ZHANG Chun-yu，YAN Hui，JIN Hong-fang，DU Jun-bao. 2014, 29(9): 687-690.  DOI: 10.7504/ek2014090611 Abstract ( )   Abstract：Objective This study was designed to explore the changes in hydrogen sulfide（H2S） and flow-mediated vasodilation（FMD） in postural orthostatic tachycardia syndrome（POTS） children and the relationship between them. Methods The study included 36 POTS children aged 11±3 and 18 normal children aged 11±4 as controls. The diagnosis of POTS was based on clinical manifestation and head-up test or head-up tilt test. The plasma concentration of H2S was determined by a sensitive sulphur electrode method and FMD of brachial artery was measured using color Doppler vascular ultrasound. Results There were no significant differences between the POTS and control groups in age， sex ratio， height， weight， body mass index and mean arterial pressure（P＞0.05）. In POTS children， the supine heart rate（88±15 bpm） was higher than the controls（77±11 bpm，P=0.006 ）. Plasma H2S level was significantly higher in POTS group than in the control group（30.25±11.64 μmol/L in POTS group vs 17.58±1.74 μmol/L in control group，P＜0.01）. The FMD in POTS group was significantly higher than in the controls（9.87±1.63% vs 5.92±2.14%，P＜0.001）.In addition， there was a significant positive correlations between H2S and FMD（r=0.565，P＜0.001）.Conclusion Plasma H2S and FMD are significantly increased in POTS children. The abnormal vasodilation may play an important role in POTS of children.

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The short - term follow - up of the frequent atrial premature contraction in suspected viral myocarditis children. MA Lan-fang， AN Jin-dou， FENG Song， GE Wei. 2014, 29(9): 691-695.  DOI: 10.7504/ek2014090612 Abstract ( )   Abstract： Objective To investigate the features and short-term prognosis of the frequent atrial premature contraction in suspected viral myocarditis children. Methods We collected 41 suspected viral myocarditis patients admitted by the Pediatric Department of our hospital during the latest 10 years, whose main manifestations were frequent atrial premature contraction，and then analysed their Holter，short-term prognosis and the relationship between the two. Results （1）There were 30 cases （73%） whose total atrial premature contractions in the Holter were below 20000；4 cases （10%） were atrial pararrhythmia，35 cases （85%） with the atrial ectopic P’ wave merged into T wave，14 cases （32%） coexisted with atrial tachycardia；most premature contractions were monophyletic and originated from the lower part of the atria. （2）After 2 weeks of antivirus，myocardial nutrition，antioxidant treatment and traditional Chinese medicine therapy，the premature contraction decreased more than 30% in half of the patients. （3）The premature contraction decreased more remarkably in patients with atrial tachycardia than those with pure atrial premature contraction. Conclusion For the frequent atrial premature contraction in suspected viral myocarditis children，especially for those coexisted with atrial tachycardia，the most common reason is viral myocarditis，and the atrial premature contranction could decrease or disappear after treatment based on this diagnosis.

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Clinical analysis of congenital supravalvular aortic stenosis in 26 cases. GU Yan，JIN Mei，ZHENG Ke， LIANG Yong-mei，WANG Xiao-fang，XIAO Yan-yan，JIAO Meng，HAN Ling. 2014, 29(9): 696-699.  DOI: 10.7504/ek2014090613 Abstract ( )   Abstract： Objective To investigate the diagnosis and management of congenital supravalvular aortic stenosis. Methods Data of 26 cases is collected and analyzed retrospectively， among which there were mental retardation in 10 cases，growth retardation in 12 cases，positive family history in 2 cases and other heart abnormalities in 4 cases. Results Local supravalvular aortic stenosis was proved in 26 cases by thoracic echocardiography. Catheterization and angiography was performed in 2 cases，and one patient died because of respiration and circulation failure after angiography. Computed tomography angiography was done in 12 cases. Surgical operation was conducted in 13 cases，and 11 patients recovered uneventfully. Of the other two patients，one died during operation，and the other was complicated with brain problem. Conclusions Echocardiography is the basic method for diagnosis; computed tomography angiography and/or catheterization is needed necessarily in establishing diagnosis. Surgical operation is feasible in correcting supravalvular aortic stenosis with satisfied recovery in short-term follow-up，and sometimes pulmonary artery plasty performed if necessary.

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Clinical value of dual-source computed tomography angiography （DSCTA） in diagnosis of heterotaxy syndrome in children. XIONG Qing-feng*， FU Xiao-rong， XU Juan， CHEN Xian-feng， PENG Zhi-yuan. 2014, 29(9): 700-704.  DOI: 10.7504/ek2014090614 Abstract ( )   Abstract： Objective To evaluate the clinical value of dual-source computed tomography angiography （DSCTA） in diagnosis of the heterotaxy syndrome. Methods Nine patiens with heterotaxy syndrome underwent DSCTA and echocardiography before operation， then， retrospective analysis was performed. Prospective ECG-triggering DSCTA scanning with low dosage was applied， scanning range was from the superior aperture of thorax to the epigastrium， and then anomalies of the heart， great vessels， lung， spleen， and liver were observed by means of multiplanar reconstruction. Result Left atrium isomerism was seen in 5 cases， 1 was atrial septal defect （ASD）， 1 was ASD and ventricular septal defect （VSD）， 1 was single ventricle （SV）， and 2 were double outlet of right ventricle （DORV）. Bilateral morphologic left bronchi， bilaterally hyparterial bronchus and polysplenia were identified， accompanied with abnormal abdominal visceral position in 4 cases， left ventricular loops and mirror dextrocardia in 1 case， interrupted inferior vena cava and double superior vena cava in 3 cases， right aortic arch in 2 cases， total anomalous pulmonary venous connection in 1 case， and a right-sided stomach in 4 cases. Right atrial isomerism was seen in 4 cases，3 of which were associated with SV. Bilateral morphologic right bronchi， bilaterally eparterial bronchus, asplenia, and symmetrical liver were identified in the patients， accompanied with a right-sided stomach in 2 cases. As well as right-sided atrioventricular connectionless in 1 case and transposition of the great artery in 1 case. The main vascular malformations outside the heart were 21 detected with DSCTA ， contrasting to 14 detected with echocardiography. In the 9 patients， 5 cases were performed anatomic surgical correction， 3 cases were performed palliative treatment， 1 case died of persistent hypoxia and function failure in multiple organ after Fanton operation. Following-up time was from half year to seven years. Conclusion DSCTA can accurately diagnose heterotaxy syndrome and clear show the status of atrium， spleen， liver and bronchus; furthermore， it can provide an accurate diagnosis of complex congential malformations of cardiovascular， which is very helpful in making decision before operation.

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Feasibility and safety of transcatheter closure of ventricular septal defects in 184 children under 3 years old. YANG Liu-qing， LI Yu-fen， ZHANG Zhi-wei， XIE Yu-mei， QIAN Ming-yang， LI Jun-jie， WANG Shu-shui. 2014, 29(9): 705-707.  DOI: 10.7504/ek2014090615 Abstract ( )   Abstract：Objective To evaluate the clinical effect of transcatheter closure for ventricular septal defect in infants and young children. Methods Clinical data from 184 children under 3 years old who underwent the transcatheter VSD closure from Jan. 2011 to Dec. 2012 were analyzed. Results Procedure was successful in 176 cases （95.7%）. No death occurred， residual shunt in 13 cases， tricuspid inflow accelerated in 2 cases， late-onset complete atriovenous block in one case （6 months after procedure）. All cases had no vascular complications. Conclusion Transcatheter closures for ventricular septal defects are effective in small children. Long-term follow-up is needed to evaluate the safety of this operation. The keys to the success of operation are the proper indications,suitable apparatus,skillful vascular punction and good anaesthetic management.

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Correlation among exhaled nitric oxide ，lung function and Asthma Control Test in asthmatic children. HOU Ping，SHAN Li-shen，CAI Xu-xu，ZHANG Han，WANG Zhi-jia，SHANG Yun-xiao. 2014, 29(9): 708-712.  DOI: 10.7504/ek2014090616 Abstract ( )   Abstract： Objective To investigate the correlation among exhaled nitric oxide ，lung function and Asthma Control Test in asthmatic children and the clinical significance. Methods A total of 136 cases of asthmatic children aged 5 to 15 years old were recruited from Shengjing Hospital of China Medical University.The fraction of exhaled nitric oxide， lung function and ACT score were measured，and the correlation among them was analyzed. Results Negative correlation was found between FeNO and ACT（r= -0.251，P <0.005）.Negative correlation was found between FeNO and FEV1/FVC of lung function（r= -0.206，P<0.05），but not FEV1 or PEF.Positive correlation was found between ACT score and FEV1/FVC of lung function （r= 0.242，P<0.01）， but not FEV1 or PEF. Conclusion For all subjects，FeNO is negatively correlated with ACT score and FEV1/FVC.ACT is positively correlated with FEV1/FVC.When the subjects are grouped，the three don’t have stable correlations.FeNO is a good biomarker to evaluate the airway inflammation of asthmatic children.ACT score evaluates the level of asthma control in children.Lung function evaluates airway obstruction.FeNO，lung function and ACT are different measurements that evaluate asthma in different ways.None of them can be used instead of another in clinical practice，and they are complementary.Combined applications can effectively improve the diagnosis，treatment and asthma control in asthmatic children.

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Caesarean section and risk of allergic rhinitis and asthma in children. HUANG Jin-mei，LIU Ji-xian，ZHAO Xian-hong 2014, 29(9): 713-717.  DOI: 10.7504/ek2014090617 Abstract ( )   Abstract： Objective To investigate if there is an association between caesarean section and allergic rhinitis as well as asthma in Suzhou children and whether the association is affected by family history of allergies. Methods This study was based on the epidemiological investigation of asthma among children aged 0-14 years from Pingjiang area of Suzhou city in 2010. From 540 asthmatic children we selected 427 children aged 5 to 14 years， 290 males and 137 females ， the average age being （8.46±2.3）years. Another 427 non-asthmatic children were chosen by 1:1 matching by the same sex and age with the asthma children ， A total of 854 cases were chosen as the study population．Then analyze the survey data by using Chi-square test and Logistic regression analysis. Results Of the 854 children， 388 were delivered by cesarean section（allergic rhinitis 158 cases，asthma 191 cases） and 466 by vaginal（allergic rhinitis 146 cases，asthma 236 cases）．After adjusting for potential confounders，caesarean section was associated with an increased risk of allergic rhinitis（OR=1.66，95%CI 1.21，2.29）．Specifically，while approximately 2.6-fold higher odds of allergic rhinitis was observed in children with a family history of allergies（OR=2.58，95%CI 1.46，4.56），no association was observed in children without a family history of allergies（OR=1.42，95%CI 0.95，2.13）．Children born by cesarean section had no higher risk of asthma than those delivered vaginally（OR=0.91，95%CI 0.68，1.22）. Conclusion The association of caesarean section and allergic rhinitis appears more remarkable in children with family history of allergies．However，caesarean section is not associated with asthma in childhood.