ventricular septal defect ( vsd ) is one of the most common congenital cardiac defects . 

traditional open - heart repair has been established as the gold standard for closure of the 
 perimembranous vsd . due to the 
 close proximity to the conduction system
, complete atrioventricular block ( avb ) occurs in 
 03% of vsd patients and is a serious complication that can occur both during and after 
 operation . on the other 
 hand ,
the most significant risk factor for avb is down syndrome , and 2.7% of these patients 
 with avb require permanent pacemaker implantation .
but there 
 are some problems , such as abrasion of leads due to the friction between leads , between the 
 lead and generator , or between the lead and first rib or clavicle .
a 6-year - old girl with down syndrome , mild mental retardation , congenital heart disease 
 ( patent ductus arteriosus ( pda ) and vsd ) was referred to our outpatient pacemaker clinic 
 ( clinical course is summarized in table 
 1table 1clinical course of the casedateageheightweightbmievents1996.02.20birth1996.07.055 monthspda ligation1996.10.117 monthsoccurrence of avb during vsd patch closureindwelling epicardial 
 ventricular lead1996.11.219 monthspacemaker implantation2000.11.174 yearsepicardial atrial lead implantation .
mode change : vvi to dddbattery 
 exchange2002.03.166 yearsreferred to our pacemaker clinic2003.06.117 years116 cm18 kg13.38battery exchange2005.12.139 years133 cm26 kg14.70battery exchange2007.01.2410 years140 cm29.5 kg15.05intravenous pacemaker implantation ( medtronic e2dr21 enpulse 2 
 dr)2013.08.0517 years140 cm40 kg20.41generator exchange ( medtronic advisa ) ) .
she had suffered from congestive heart failure and been treated with a 
 mechanical ventilator for approximately 6 months just after birth .
patch closure for vsd 
 was performed when she was 7 months old . due to a complete surgical avb
, a vvi pacemaker 
 with a permanent epicardial pacing lead was implanted on the right ventricle .
when she was 4 
 years old , an atrial lead was implanted through a mini - thoracotomy on the anterior surface 
 of her right atrium , and the pacing mode was switched from vvi to ddd .
when she was 7 years 
 old , we exchanged her generator again because of battery depletion . due to the pacing 
 threshold of the ventricular lead not being good
when she was 
 10 years old , we implanted a permanent pacemaker transvenously under general anesthesia 
 ( medtronic e2dr21 enpulse 2 dr ) .
the chest x - ray photos taken before and after implantation 
 are shown in figure 1figure 1chest x - ray just before transvenous pacemaker implantation at the age of 10 
 ( 2006.12.22 ) . and figure 2figure 2chest x - ray just after transvenous pacemaker implantation ( 2007.1.25 ) .
note that both new leads have loops long enough in 
 her right atrium to keep up with her growth .. since she has a persistent left superior vena cava variation , we performed cutdown 
 of the right cephalic vein , fixed screw - in leads to her right ventricular apex and right 
 atria , and implanted a generator under her right subpectoral muscle .
she was discharged from 
 the hospital on day 1 after the surgery and showed a satisfactory outcome thereafter .
this 
 was the youngest case to receive a transvenous permanent pacemaker in akita prefecture .
chest x - ray just before transvenous pacemaker implantation at the age of 10 
 ( 2006.12.22 ) .
note that both new leads have loops long enough in 
 her right atrium to keep up with her growth .
the approach to permanent pacemaker implantation in young patients is determined by many 
 factors such as age , structural congenital heart disease , venous access to the heart , venous 
 thrombosis , and pacing - induced dyssynchronous cardiomyopathy .
a previous paper showed that the probability of continued epicardial pacing in children 
 increased to 76% at 10 years after implantation .
bipolar steroid - eluting leads and an automatic output 
 adjusting system significantly increased pacing system longevity .
a study showed pediatric pacing patients with epicardial lead 
 systems have a high incidence of lead failures , and transvenous lead systems were 
 recommended when anatomy permits . in our case , we were able to operate when her body size was 
 large enough .
transvenous permanent pacemaker implantation has been demonstrated to be a safe procedure 
 with fewer complications and a lower ventricular threshold than the epicardial route in 
 children from 0.09 to 12 years of age ( median , 2.3 years ) . in another report ,
transvenous permanent pacemaker 
 implantation was proved to be a safe and effective method in children ( mean age 9.2  4.7 
 years ) .
although , lead or 
 generator exchange is inevitable , the long - term outcome is favorable .
their skin and subcutaneous 
 tissue are fragile , and a generator is quite large for their small body size . as shown in 
 figure 3figure 3enhanced computed tomography showed that the right subclavian vein and cephalic vein 
 were large enough ( 2006 .
the right external and internal 
 jugular veins are identical . , our patient had a large enough right subclavian vein and cephalic vein .
her right 
 jugular vein , which is an alternative access vein in the case of any trouble with the 
 cephalic vein , was large enough , although it was not used .
enhanced computed tomography showed that the right subclavian vein and cephalic vein 
 were large enough ( 2006 .
puncture into intrathoracic vessels can cause many complications such as vessel rupture , 
 brachial plexus injury , pneumothorax , or hemothorax .
recently , the axillary vein puncture 
 method has been demonstrated to be less invasive , more cosmetic , and without the 
 complications encountered with the intrathoracic method .
cutdown of the cephalic vein can prevent such 
 complications and enables perfect hemostasis at the access point of the vein .
as it is less 
 stressful to leads anatomically , lead fracture was significantly decreased .
but since screw - in leads were used 
 extensively , dislodgement of leads decreased significantly .
however , heart perforation by screw - in leads should 
 be noted . in our past 380 
 cases with these techniques ,
1 patient had late tamponade due to perforation of the right 
 atrium . in the present case ,
3.5 cm of excess lead was formed into a loop in the right atrium 
 and ventricle in order to prevent lead dislodgement due to growth of her body .
the ventricular lead was anchored 
 carefully so that it could not reach the orifice of ivc .
the patient recovered well after 1 week of bed rest 
 without any surgical intervention .
a study reported an improved cosmetic 
 result , less abrasion , less infection , no neurovascular and muscular damage , no generator 
 damage by the ribs , no serious hematomas , and no chronic pain .
so the subpectoral pocket is recommended as the 
 preferred site for implantation of transvenous pacemakers in pediatric patients .
we chose the smallest generator 
 ( e2dr21 enpulse 2 dr ) as the replacement because we planned to change the generator when her 
 growth stops at 17 or 18 years old .
the small generator contributed to prevention of skin 
 ulcer on her generator pocket and no restriction of movement of her shoulder joint .
in fact , 
 we exchanged her generator to a larger one ( medtronic advisa ) when she was 17 years old .
the 
 chest x - ray photos taken before and after exchanging are shown in figure 4figure 4chest x - ray taken 7 years after transvenous pacemaker implantation when she was 17 
 years old ( 2013.6.20 ) .
the lengths of both leads are long enough for fit with her 
 grown up body . and figure 5figure 5chest x - ray taken after her generator was exchanged ( 2013.8.5 ) .. chest x - ray taken 7 years after transvenous pacemaker implantation when she was 17 
 years old ( 2013.6.20 ) .
the lengths of both leads are long enough for fit with her 
 grown up body .
our case indicated that transvenous permanent pacemaker implantation is safe and useful in 
 young patients .
when the patient s anatomy permits , transvenous lead systems should be 
 considered as one of the treatment options in young patients .
 objective : the aim of this report was to discuss the type , timing , and 
 surgical techniques of permanent pacemaker implantation in a juvenile patient.patients : a 17-year - old girl with down syndrome and congenital heart defects 
 comprised of ventricular septal defects ( vsd ) and patent ductus arteriosus ( pda ) suffered 
 from postoperative complete atrioventricular block ( avb ) when she was 7 months old.methods and results : an epicardial pacemaker was implanted just after the 
 occurrence of complete avb . due to the pacing threshold of a ventricular lead not being 
 good , 
 the battery showed rapid depletion . 
 her generator had to be exchanged under general 
 anesthesia every 23 years . 
 when she was 10 years old , we implanted a permanent pacemaker 
 transvenously by using cutdown , screw - in and subpectoral pocket techniques . 
 she has shown 
 a satisfactory outcome since then.conclusion : transvenous pacemaker implantation was safe and effective in our 
 young patient without any complications . 
 the timing of surgery and surgical technique are 
 quite important for pacemaker implantation in juvenile patients . 