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myocardial inflammatory diseases are an important cause of dilated cardiomyopathy ( dcm ) in children .
epidemics of viral myocarditis have been reported , particularly coxsackie b virus , and the enteroviruses which are considered to be the most common cause of viral myocarditis .
thus infants and young children may be more prone to develop myocarditis , due to the higher rate of enteroviral and adenoviral infections in this age group .
although myocarditis has previously been speculated to account for most instances of dcm , it is now clear that 2030% may be due to familial or genetic forms of dcm ; yet myocarditis might still be implicated in the initiation of the process .  
troponin i has a high specificity for diagnosing myocarditis but a sensitivity of only 34% , and creatine kinase and its cardiac isoform ck - mb are less sensitive and specific than troponin .
tenascin - c ( tn - c ) is an extracellular matrix glycoprotein   that is not expressed in normal adult hearts but is expressed in various myocardial diseases such as acute myocarditis [ 79 ] , dilated cardiomyopathy , myocardial infarction , and myocardial hibernation .
speckletracking echocardiography ( ste ) is a noninvasive ultrasound imaging technique that allows for an objective and quantitative evaluation of global and regional myocardial function independently from the angle of insonation and from cardiac translational movements .
its advantage over conventional echo warrants its use in the assessment of the global and regional myocardial performance in acute and chronic dcm .
we hypothesized that serum tn - c might serve as a marker of active myocardial inflammation in children with new onset dcm .
this case controlled study was conducted in the pediatric cardiology clinic and echocardiography laboratory , children 's hospital , ain shams university .
the study included 24 consecutive patients with dcm aged 672 months with a mean age of 45.19  11.03 months and 20 healthy children .
the subjects were divided into 3 groups : group i included twelve patients with acute dcm of less than 6 months duration ; group ii included twelve patients with chronic dcm for more than 6 months ; control group included twenty ( 20 ) healthy age- and sex - matched children .
the control group was chosen from healthy children who came with their sick siblings to the outpatient clinic of the children 's hospital .
clinical diagnosis of dcm was based on the who   and american heart association   criteria . at time of diagnosis
all patients had an ejection fraction < 45% and/or a fractional shortening of < 25% and a left ventricular end diastolic dimension ( lvedd ) of > 112% of the predicted value corrected for age and body surface area .
all patients received antifailure measures in the form of inotropes ( lanoxin ) , diuretics ( furosemide and spironolactone ) , and angiotensin enzyme inhibitor ( captopril ) .
a written informed consent was given by parents of patients and controls , and the study protocol was approved by the institutional review board . 

studied patients were subjected to full medical history , thorough clinical examination , and investigations that included the following . 

serum levels of tn - c with the large subunit containing the c domain of fitiiii repeats were determined using an elisa technique with 2 monoclonal antibodies , 19c4ms and 4f10tt ( ibl , gunma , japan ) . 

m - mode , two dimensional , continuous , pulsed colors doppler and speckle track echocardiography ( ste ) transthoracic echocardiography was done using device model ge medical system vivid7 dimension n-3190 , horten , norway .
longitudinal strain ( ls ) was assessed in standard four - chamber , three - chamber ( apical long - axis ) , and two - chamber apical views .
the images were obtained at end - expiratory phase using a 5  mhz center frequency phased - array probe with second - harmonic imaging .
they were stored in cine loop format for offline analysis by vendor customized software ( echo pac pc-2d strain ; ge medical systems ) .
ls was measured in 16 lv segments by tracing the endocardial contour on an end - systolic frame that allowed the software to automatically place the contour on subsequent frames by temporally tracking the  natural acoustic speckle  in the b - mode images .
adequate tracking for the study was verified in real time , and in segments with poor tracking , the endocardial trace line was readjusted until a better tracking score was achieved .
ls curves reflected the average value of all of the acoustic markers in each segment . 

data were expressed as means  sd for continuous variables and as numbers ( percentages ) for categorical variables .
the levels of tn - c of all patients ( groups i and ii ) and controls are shown in tables 1 and 2 .
m - mode echocardiographic data of the patients in comparison to the control group are shown in table 3 and those of group i in comparison with group ii are shown in table 4 .
2d ste showed that there was a highly statistical significant difference between patients and controls as regard global peak strain longitudinal apical long axis ( g sl ap lax ) , g peak sl apical 4 chambers ( a4c ) , g peak sl apical 2 chambers ( a2c ) , and g peak sl average .
ste wall motion score was normokinetic in all controls while data of groups i and ii are shown in table 5 .
correlation between serum tn - c level and the echocardiographic findings showed a statistically significant negative correlation between serum tenascin - c and ef in all patients ( figure 1 ) and a statistically significant positive correlation between serum tn - c level and lvedd and enddiastolic volume in patients ( figure 2 ) and in acute cases .
correlation between serum tn - c level and patients , group i and group ii , is shown in figures 3 , 4 , and 5 , respectively .
serum tn - c levels have not been previously studied in pediatric patients with dcm . in the present study
, we attempted to investigate the utility of its use as a marker of inflammation in infants and children with dcm . to avoid noninfectious causes of dcm
we found serum tn - c levels to be significantly higher in children with dcm than those in controls and in acute than chronic dcm .
the significant elevation of tn - c in group i might be due to an inflammatory process since children at this age group are more prone to develop dcm following viral myocarditis .
previous data demonstrated that tnc is a useful marker for evaluation of disease activity in myocarditis [ 7 , 9 ] . in their study on adult
.   found a high prevalence of chronic myocarditis in dcm patients and suggested that tn - c might prove to be a useful marker for distinguishing inflammatory cardiomyopathy from other types of dcm .
researchers found that most of the myocardium in dcm patients shows varying degrees of inflammation and that expression of tn - c is enhanced in the areas of active inflammation with local tissue remodeling .
serum tn - c correlated negatively with the ef and positively with the lvidd and edv suggesting that a high serum tn - c associated the impaired myocardial functions .
similarly , aso et al .   found that serum tn - c levels were increased in proportion to the severity of left ventricular dysfunction in patients with idc .
the decrease in tn - c that associated the improved ef in group ii is suggestive of an improvement but not disappearance of the inflammatory process in chronic dcm . yet the role of acei in blocking vascular tn - c expression can not be excluded as our patients with chronic dcm were maintained on acei .
angiotensin ii is a potent inducer of tenascin - c , with drugs such as angiotensin ii type 1 receptor ( at-1 ) antagonists , and angiotensin converting enzyme ( ace ) inhibitors potentially block vascular tenascin - c expression in hypertensive patients . on the other hand ,
we can not exclude the role of lv dysfunction and heart failure in increasing serum tn - c in our study group .
it was suggested that the increase in serum tn - c levels was associated with the severity of heart failure and lv dysfunction and remodeling in patients with dcm [ 10 , 19 ] .
owing to the significant correlation between the tn - c level and lvdd , it was suggested as a new biomarker for detecting cardiomyopathy in patients with emery - dreifuss muscular dystrophy .  
the recently introduced ste allows easy assessment of segmental and global longitudinal lv function and provides information on top of ejection fraction .
the significant positive correlation between serum tn - c and global peak longitudinal strain average in patients indicates that the increase in serum tn - c levels was associated with deterioration in cardiac function as detected by ste .
we used the global longitudinal strain since it has been demonstrated that it is a more robust parameter than radial and circumferential strain for the assessment of myocardial function .
the ste wall motion scores in group i showed that 33.5% were normokinetic , 8.33% were hypokinetic , and 50% were akinetic .
these data are suggestive of the prognosis in this group , where 1/3 of patients with acute dcm are apt to have improved lv functions after the inflammatory process subsides while those with akinesia might either improve or die .
ste was more sensitive than conventional echocardiography in detecting wall motion abnormalities in group ii , where all the patients had hypokinetic wall motion scoring by ste inspite of the normalized ef .
this indicates an ongoing process of myocardial affection in chronic dcm that is associated by a raised serum tn - c .
drugs targeting the expression or function of tenascin - c or the tenascin - c protein itself are currently being developed .
they might help in the better management of acute and chronic cardiomyopathy of infectious origin .
after the exclusion of the familial / genetic and secondary causes of dcm , the number of patients was small .
once diagnosed all patients received angiotensin converting enzyme inhibitors which are known to block vascular tn - c expression .
serum - tn - c levels are increased in children with dcm and can be used as a marker of inflammation in acute cases .
ste is more sensitive than conventional echocardiography in the assessment of myocardial performance in dcm .
drugs targeting the expression or function of tenascin - c might offer new perspectives for therapeutic approaches in this specific population .
background . 
 tenascin - c ( tn - c ) is an extracellular matrix glycoprotein that appears at sites of inflammation in cardiac pathologies . 
 aim of the work . to evaluate the role of tn - c as a marker for active inflammation in children with dilated cardiomyopathy ( dcm ) . 
 subjects and methods . 
 24 consecutive patients with primary nonfamilial dcm aged 672 months ( mean 45.19  11.03 ) were divided into group i , twelve patients with acute onset dcm ( < 6 months duration ) , and group ii , twelve patients with chronic dcm ( > 6 months duration ) , and compared to 20 healthy age- and sex - matched controls . 
 investigations included estimation of serum tn - c and echocardiographic evaluation using m - mode and 2d speckle tracking echocardiography ( ste ) . results . 
 serum tn - c showed a higher significant statistical elevation among patients than controls ( p < 0.001 ) and in group i than group ii ( p < 0.001 ) . 
 ef was significantly decreased , and lvedd and edv increased in patients than controls and in gi than gii . 
 ste showed a statistically significant difference in global peak strain longitudinal ( gpsl ) average in patients than controls ( p < 0.05 ) and between gi and gii ( p < 0.001 ) . 
 ste wall motion scoring showed normokinesia ( 33.5% ) , hypokinesia ( 8.33% ) , and akinesia ( 50% ) in gi and hypokinesia ( 100% ) in gii . 
 there was a statistically significant positive correlation between serum tn - c and gpsl average . 
 conclusions . 
 increased serum tn - c can be used as a marker of inflammation in dcm and is associated with the severity of heart failure and lv dysfunction as detected by ste .