a 13-year - old girl had a history of partial atrioventricular septal defect ( pavsd ) total correction at the age of 4 years .
however , a right ventricular assist device was applied at the operation because of the akinetic right ventricle ( rv ) .
two years later , based on our echocardiography results , the patient was transferred due to severe tricuspid regurgitation and rv chamber enlargement with rv dysfunction .
she underwent tricuspid septal commissuroplasty , de vega - type tricuspid annuloplasty , right atrial reduction plasty , and isthmus ablation .
further , we performed one - and - a - half ventricle repair . in spite of the operation ,
six years after the one - and - a - half ventricle repair , cardiac magnetic resonance imaging was performed to evaluate her cardiac function and measure the left ventricle ( lv ) volume .
the patient s rv end diastolic volume index and rv ejection fraction were 500.4 ml / m , and 13.2% , respectively .
the lv stroke volume index and ejection fraction were 38.2 ml / m and 28% , respectively .
echocardiographic evaluation showed global rv akinesia and lv ejection fraction of 25% with paradoxical interventricular septal motion .
we suspected that the rv enlargement affected both the rv and the lv function and it resulted in decreased lv contractility .
she underwent extracardiac conduit fontan operation with polytetrafluoroethylene ( ptfe , gore - tex ; wl gore & associates , flagstaff , az , usa ) 24-mm tube graft , rv exclusion , atrial septectomy , and permanent pacemaker implantation .
rv exclusion procedures include tricuspid valve obliteration ( from the rv side ; 5 - 0 prolene double layer , reinforcement suture from the ra side ; 4 - 0 polyester ptfe pledget - supported interrupted mattress suture ) and pulmonary valve obliteration ( 6 - 0 prolene running suture ) to reduce the rv volume with no flow connection , thrombin soaked gel - foam packing to the rv , and rv free - wall wide resection , and it was performed under the condition of cardiac arrest ( fig .
permanent pacemaker bipolar leads were implanted at the lv apex , rv apex , left atrial roof , and ra free wall owing to a history of frequent atrial flutter and junctional rhythm .
we did not perform arrhythmia surgery because the patient underwent an electrophysiology study and radiofrequency catheter ablation for supraventricular arrhythmia before the operation .
the cardiopulmonary bypass time was 308 minutes , and the aortic cross clamp time was 146 minutes .
we performed the computed tomographic angiography not cardiac magnetic resonance imaging to evaluate the patient s postoperative cardiac function and chamber size , because she was implanted with a permanent pacemaker .
the rv volume was markedly reduced , and the rv was occluded with thrombosis ( fig .
further , the stroke volume index and the ejection fraction of lv were increased to 48.03 ml / m and 33% , respectively ( table 1 ) .
the patient s vital signs were stable with a central venous pressure of 18 to 21 mmhg .
she was extubated in 14 hours , and she stayed in the intensive care unit for 90 hours .
we removed the chest tubes 5 days later , and she was discharged on postoperative day 21 .
she has been followed up for 21 months without any complaint of dyspnea and palpitation .
3 ) , and in the latest echocardiography , the ejection fraction of the lv had increased to 54% .
recently , the importance of rv failure has been noted , in light of the incomplete understanding of the rv failure mechanism and a poorer prognosis than lv failure has .
further , it has been reported that an increased rv volume and decreased ejection fraction are associated with lower survival rates in patients with congestive heart failure .
the rv volume overload is one of the causes of right ventricle failure ( rvf ) .
further , it is known that the rv volume over load leads to the leftward displacement of the interventricular septum and changes in the lv geometry , thus resulting in decreased lv contractility .
, several reports have shown the successful outcome of the original and the modified rv exclusion procedures in the case of ebstein s anomaly .
. she could have lived in a biventricular state if she had undergone the total correction of pavsd at the appropriate time . however , this case is meaningful in determining how to manage patients with rvf .
we performed an rv exclusion to normalize the motion of the interventricular septum by reducing the rv volume .
further , we demonstrated that both the end diastolic volume index and the stroke volume index increased after the operation .
another beneficial effect of rv exclusion is lung expansion , because an enlarged heart can compress the lungs .
in addition , adequate lung expansion helps to reduce pulmonary vascular resistance , and decreased pulmonary vascular resistance is a good prognostic factor among functional univentricular patients . in conclusion ,
the rv exclusion procedure in selected patients with severe rvf might be a safe and beneficial option to improve lv function .
 a 13-year - old girl , who had undergone the total correction of partial atrioventricular septal defect at the age of 4 years , was admitted with severe tricuspid regurgitation in echocardiography . 
 she had received one - and - a - half ventricle repair during follow - up . 
 her right ventricle showed global akinesia , and the ejection fraction of the left ventricle was 25% with paradoxical interventricular septal motion . 
 we performed right ventricular exclusion adjunct to the fontan procedure . 
 she is doing well two years after the operation without complications . 
CASE REPORT
DISCUSSION