Biventricular Repair after Bilateral Pulmonary Artery Banding as a Rescue Procedure for a Neonate with Hypoplastic Left Heart Complex

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.

Aortic Root Translocation with Arterial Switch for Transposition of the Great Arteries or Double Outlet Right Ventricle with Ventricular Septal Defect and Pulmonary Stenosis

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

Heart Transplantation in Pediatric Patients: Twelve-Year Experience of the Asan Medical Center

Heart transplantation is a standard treatment for end-stage heart disease. Pediatric heart transplantation, however, is not frequently performed due to the shortage of pediatric heart donors. This is the first report of pediatric heart transplantation in Korea. Our retrospective study included 37 patients younger than 18 yr of age who underwent heart transplantation at Asan Medical Center between August 1997 and April 2009. Preoperative diagnosis was either cardiomyopathy (n = 29, 78.3%) or congenital heart disease (n = 8, 22.7%). Mean follow up period was 56.9 +/- 44.6 months. There were no early death, but 7 late deaths (7/37, 18.9%) due to rejection after 11, 15, 41 months (n = 3), infection after 5, 8, 10 months (n = 3), suspicious ventricular arrhythmia after 50 months (n = 1). There was no significant risk factor for survival. There were 25 rejections (25/37, 67.6%); less than grade II occurred in 17 patients (17/25, 68%) and more than grade II occurred in 8 patients (8/25, 32%). Actuarial 1, 5, and 10 yr survival was 88.6%, 76.8%, and 76.8%. Our midterm survival of pediatric heart transplantation showed excellent results. We hope this result could be an encouraging message to do more pediatric heart transplantation in Korean society.

Transplantation of an Extremely Oversized Heart after Prolonged Extracorporeal Membrane Oxygenation Assistance in a 3-month-old / 대한흉부외과학회지

According to the 2007 International Society for Heart and Lung Transplantation (ISHLT) report, a congenital diagnosis, infantile transplantation and being on extracorporeal membrane oxygenation (ECMO) at the time of transplant are risk factors for mortality for the patients who undergo a heart transplant, and a large body weight ratio also increases the risk of mortality. The patient of this case underwent a Ross operation and mitral valve repair due to left ventricle outflow track obstruction and mitral regurgitation. But the baby was treated with ECMO due to heart failure after the operation. When he was 3-months-old and had been on 30 days of ECMO, he underwent a heart transplant with a heart that had a high donor-recipient weight ratio (4.42). We present this case from a technical standpoint and we include a review of the relevant literature.

Mid to Long Term Outcomes of Surgical Treatment for Isolated Coarctation of Aorta / 대한흉부외과학회지

BACKGROUND: The surgical repair of an isolated coarctation of the aorta, without complex cardiac anomalies, has improved, with very good results. However, despite the success of surgical repair, many long-term complications, such as hypertension, re-coarctation and an aortic aneurysm, still exist. MATERIAL AND METHOD: Between 1991 and 2006, 50 patients diagnosed with an isolated coarctation of the aorta were reviewed retrospectively. The incidence of re-coarctation and hypertension were compared with respect to age and surgical methods. RESULT: There were no early & late mortality, or post operative aortic aneurysms. Hypertension developed in 11 patients (22%). A greater number of patients in the child/adult group had hypertension (52.4%) than in the neonate/infant group (0%). With respect to the surgical methods, the patients in the graft interposition group suffered more hypertension (88.9%) than those in the EEEA (extended end to end anastomosis) group (5.3%). Post operative re-coarctation developed in 2 out of the 29 patients (6.9%) in the neonate/infant group and 2 out of the 21 patients (9.5%) in the child/adult group, but without any statistical difference. There were no statistical differences between the operative type-related groups. CONCLUSION: Even though the surgical outcomes have greatly improved, an isolated coarctation of the aorta still has many long-term problems, such as hypertension and re-coarctation. An isolated coarctation is accepted as a systemic vascular dysfunction, and often progresses to other cardiovascular diseases. Therefore, patients with a coarctation of the aorta have to be carefully followed-up, and aggressive management must be given when required.

Hybrid Palliation for Functionally Single Ventricle with Systemic Outflow Obstruction / 대한흉부외과학회지

Hybrid procedure was performed for a thirteen-day-old girl with a functionally single ventricle, who weighed 2.2 kg and had been prematurely born at 32(+5) weeks of gestation. She underwent bilateral pulmonary artery banding using 3.5 mm Gore-Tex graft, ductal stenting using balloon expandable stent, and reverse Blalock-Taussig shunt with 3.5 mm Gore-Tex vascular graft. After discharge, she was followed up for 4 months, and underwent 2nd stage operation (extensive arch reconstruction with Damus-Kaye-Stansel anastomosis, atrial septectomy, bilateral pulmonary artery angioplasty, bidirectional cavopulmonary shunt). She has been followed up for 4 months after the 2nd operation with an excellent clinical condition.

Left Ventricular Remodeling in Pediatric Orthotopic Size Mismatched Heart Transplantation / 대한흉부외과학회지

Recently, heart transplantation has been accepted as a standard treatment for infant and children with end-stage cardiomyopathy or complex congenital heart disease. Due to the shortage of donors, size-mismatched cardiac transplantation is common. After size-mismatched transplantation, there could be side-effects such as hypertension and hypertensive encephalopathy because of the big-heart. Donor heart is also known to do remodel as time goes by. This is a case report of a size-mismatched heart transplantation between 9-year-old boy and a 39-year-old female whose body weight is almost twice of him. In this case, classical postoperative hypertension and hypertensive encephalopathy developed but was successfully managed. The donor heart has remodeled to normal dimension during 3 years follow-up period.

Arterial Switch Operation in 1140 gm LBW Premie Baby with TGA, IVS / 대한흉부외과학회지

Cardiac surgery in the neonate with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight premie with congenital heart disease continue to challenge the intellectual and technical skills of those who care for them. We report a case of successful arterial switch operation in 1140 g premie with TGA, IVS after 4 week care 1317 gm.

Surgical Repair of the Congenital Aneurysm of the Rght Atrium / 대한흉부외과학회지

The four most common types of congenital malformations involving the right atrium(RA) and the coronary sinus(CS) are congenital enlargement of the RA, single RA diverticulum, multiple diverticula of the RA, and aneurysm of the RA or CS. A previously healthy 6year-old child was presented with signs of upper respiratory tract infection. Chest X-ray and echocardiogram revealed a severely isolated right atrial enlargement. The abnormally dilated right atrim was widely resected under cardiopulmonary bypass. Pathology revealed multifocal myocardial loss associated with mild fibrotic changes of the endocardium and epicardium. Our experience on this rare congenital disease is presented along with a review of the literature.

Surgical Repair of the Congenital Aneurysm of the Rght Atrium / 대한흉부외과학회지

The four most common types of congenital malformations involving the right atrium(RA) and the coronary sinus(CS) are congenital enlargement of the RA, single RA diverticulum, multiple diverticula of the RA, and aneurysm of the RA or CS. A previously healthy 6year-old child was presented with signs of upper respiratory tract infection. Chest X-ray and echocardiogram revealed a severely isolated right atrial enlargement. The abnormally dilated right atrim was widely resected under cardiopulmonary bypass. Pathology revealed multifocal myocardial loss associated with mild fibrotic changes of the endocardium and epicardium. Our experience on this rare congenital disease is presented along with a review of the literature.

Congenital Polyvalvular Disease: Report of A Case / 대한흉부외과학회지

Congenital polyvavular disease is a connective tissue disorder affecting more than one heart valve with variable involvement of the entire valvular and subvalvular apparatus. It is frequently associated with the Trisomy 18 and trisomy 13-15 or ventricular septal defect and patent ductus arteriosus. We present an isolated case of congenital polyvalvular disease in a new born baby with a review of the pertinent literatures, which has not been described in Korea. The mass was discovered as a right atrial mass in the prenatal ultrasonography and it was thought to be either a hematoma or a myxoma in the preoperative echocardiography. Microscopic examination of the surgically resected mass showed irregular thickening, nodulation, and additional features of calcification and ossification in the valvular connective tissue on the body of anterior and septal leaflet of tricuspid valve. Congenital polyvalvular disease should be included in the differential diagnosis in cases showing valvular calcification or ossification in the fetal echocardiography.

QT Dispersion after Chemotherapy with Anthracyclines in Children

PURPOSE: Anthracycline induced cardiotoxicity is well known and its pathology is characterized by localized myocardial cell necrosis and myocardial fibrosis. The variability in QT interval duration amongst the different leads of the standard 12-lead ECG(QT dispersion) is considered to reflect inhomogenous repolarization of the myocardium. The aim of this study was to assess the effects of anthracycline on cardiac electrophysiology, with special emphasis on dispersion of QT interval and its relation to cumulative doses. METHODS: Heart rate-corrected QT interval(QTc) and QT dispersion(QTd) were measured in standard 12-lead ECG in 34 cancer patients and compared with those of normal control. RESULTS: QTc was increased in cancer patients(462.2+/-36.0 msec vs 447.0+/-19.7 msec) but QTd was not different between the cancer patients and normal control as a whole(40.8+/-12.5 msec vs 36.6+/-9.2 msec). But in the 5-10 year age group, QTd was increased in cancer patients in comparison with that of age matched control(44.1+/-14.8 msec vs 34.0+/-9.7 msec). Also QTc in the 5-10 year age group, but not in the 11-15 year age group, was increased in cancer patients in comparison with that of age matched control(478.0+/-40.8 msec vs 446.5+/-20.9 msec). QTc and QTd were not different according to the cumulative doses of anthracycline in the cancer patients. Left ventricular systolic function was found normal in all cancer patients by echocardiographic examination. CONCLUSION: In the absence of a significant modification of echocardiographic parameters, increased inhomogeneity of ventricular repolarization could be an early marker of anthracycline cardiotoxicity. The changes of repolarization parameters were significant only in the younger age group and were not significant according to the cumulative doses. Anthracycline seemed to induce cardiotoxity from the small dose and more significantly in the younger heart.

Changes in ventricular repolarization after balloon valvuloplasty in patients with pulmonary stenosis

BACKGROUND: Alterations in ventricular loading conditions lead to changes in action potential duration via mechanoelectrical feedback. A decrease in load immediately leads to prolongation of repolarization. QT interval and QT dispersion were measured to determine the long-term effect of changes in ventricular systolic load on the ventricular repolarization. METHOD: Corrected QT interval and QT dispersion were measured in 26 patients before and 3-6 months after valvuloplasty for pulmonary stenosis. To determine the effect of ventricular load on ventricular repolarization, patients were divided in 2 groups; Group 1 was those patients with a greater than 30 mmHg and Group 2 was those patients with a less than 30 mmHg decrease in right ventricular systolic pressure. RESULTS: Corrected QT interval (412.6+/-14.5 msec vs 426.4+/-16.8 msec, p < 0.05) and QT dispersion (35.0+/-7.3 msec vs 45.7+/-14..1 msec, p < 0.05)were increased significantly only in Group 1 after vlavuloplasty. CONCLUSION: It suggested that mechanoelectrical interactions are operative for long duration in humans that changes in ventricular load after successful pulmonary valvuloplasty showed long-term effect on the ventricular repolarization.

A Comparison of Concentrations of TNF-alpha and IFN-gamma between the Dose of Intravenous Immunoglobulin 1 gm/kg and 2 gm/kg in the Patients with Kawasaki Disease / 소아알레르기및호흡기학회지

PURPOSE: Recently, the protocol of high dose of intravenous immunoglobulin has been preferred in the treatment of acute stage of Kawasaki disease. In this study, we have compared the serum concentrations of TNF-alpha and IFN-gamma in the patients with Kawasaki disease between intravenous immunoglobulin of 1 gm/kg and 2 gm/kg groups. METHODS: Fourteen patients admitted to Asan Medical Center were enrolled in this study. These patients met the diagnostic criteria of Kawasaki disease divided into two groups by the dose of intravenous immunoglobulin, 1 gm/kg and 2 gm/kg. The concentrations of TNF-alpha and IFN-gamma using ELISA methods were measured before and after the 5th and 14th days of intravenous immunoglobulin. RESULTS: The serum levels of TNF-alpha and IFN-gamma showed no statistically significant difference between 1 gm/kg and 2 gm/kg group of intravenous immunoglobulin on before the treatment and the 5th, 14th days after the infusion of intravenous immunoglobulin. CONCLUSION: There is no difference of serum concentrations of TNF-alpha and IFN-gamma between the intravenous immunoglobulin of 1 gm/kg and 2 gm/kg doses in an acute stage of Kawasaki disease. The dose of immunoglobulin in a treatment of Kawasaki disease needs to be examined in future studies.

A Case of Bronchial Obstruction due to Right Aortic Arch and Kommerell's Diverticulum / 소아알레르기및호흡기학회지

College of Medicine, Ulsan University, Seoul, Korea The vascular rings comprise less than 1% of congenital cardiovascular malformations and can be overlooked because they are rare diseases and show nonspecific respiratory symptoms such as wheezing or airway obstruction or apnea. This case is a 2-months old infant who had been repaired patch closure due to ventricular septal defect, but failed to wean from ventilator. Chest CT showed right main stem bronchus lying between right pulmonary arery and right descending aorta, bronchoscopy showed right main bronchus was compressed by extrinsic pulsating mass which were descending aorta and Kommerell's diverticulum . After aortopexy and transfer of subclavian artery, this patient was able to be extubated and is now free of respiratory symptoms. When there is failure to weaning of ventilator and persistent bronchial obstruction and emphysema, we should have more vigorous effort to seek the cause of bronchial obstruction, using bronchoscope and chest CT.

Atrial Rhythms in Patients with Left Atrial Isomerism

PURPOSE: Histologic studies have revealed ectopic location and hypoplasia of sinus nodal tissue in left atrial isomerism. The purpose of this study is to characterize the atrial rhythms corresponding to these histologic abnormalities in left atrial isomerism. Electrocardiograms in left atrial isomerism are characterized by multiple atrial rhythms with abnormal P-wave axes, but the complex nature of atrial rhythms in left atrial isomerism remains to be clarified. METHODS: Surface electrocardiograms and Holter recordings of 16 patients with left atrial isomerism were retrospectively reviewed. For comparison, atrial rhythms in 8 patients with right atrial isomerism were studied with the same study protocol. RESULTS: In left atrial isomerism, atrial rhythm with abnormal superior P-wave axis was the most frequent in 69% of patients and multiple atrial rhythms were observed in 37% in surface electrocardiograms. In Holter recordings, transient or persistent slow atrial rhythms with or without visible P waves associated with junctional escape were documented in 9 out of 16(56%) patients. In comparison, multiple atrial rhythms were as frequent as in left atrial isomerism, but slow atrial rhythm with junctional escape was not present in 8 patients with right atrial isomerism. CONCLUSION: It is concluded that transient or persistent slow atrial rhythms associated with junctional escape, suggestive of atrial nodal dysfunction, are characteristic and helpful in the diagnosis of left atrial isomerism.

Tachycardia Induced Cariomyopathy in Childhood

PURPOSE: Dilated cardiomyopathy in children is usually progressive and leads to death in several years. Chronic tachyarrhythmia has been shown to cause dilated cardiomyopathy in human and animals and this ventricular dysfuntion is usually reversible after control of the arrhythmia. The uncontrolled chronic tachycardia may be a curable cause of dilated cardiomyopathy in some patients. We describe six children who had persistent chronic tachycardia and ventricular dysfunction that improve significantly after control of the arrhythmia. METHODS: We retrosepctively reveiwed the medical records, electrocardiograms, Holter recordings, Echcardiographic reports of 6 children with the tachycardia induced ventricular dysfunction who have been managed at Sejong general hospital and Asan medical center from January 1992 to June 1995. RESULTS: 1) The causes of referral were dilated cardiomyopathy in 2 and tachyarrhythmia in 4. The age at diagnosis was 4 to 36 months old and follow-up period was 6 to 29 months. The symptoms of congestive heart failure were seen in 3 children. 2) The mechanisms of thachyarrhythmia causing cardiomyopathy were atrial ectopic tachycardia in 2, chaotic atrial rhythm in 3 and junctional ectopic tachycardia in 1. Atrial ectopic tachycardia has improved after combined treatment with amidarone and atenonl. One case of chaotic atrial rhythm has improved spontaneously and other 2 cases of chaotic arial rthythm have improved after treatment with digoxin or after combined therapy with amidarone and digoxin. Junctional ectopic tachycardia is partially controlled with mexiletine. 3) The shortening fractions of left ventricle at diagnosis were 14-21% and improved to over 30% in all after 2 to 9 months of follow-up. CONCLUSIONS: We have shown that control of persistent tachycardia resolved the left ventricular dysfunction. We suggest that patients with dilated cardiomyopathy be carefully screened for tachyarrhythmia as a curable cause of ventricular dysfunction and that left ventricular function be assessed in the asymptomatic patients with persistent tachycardia.

Tachycardia Induced Cariomyopathy in Childhood

PURPOSE: Dilated cardiomyopathy in children is usually progressive and leads to death in several years. Chronic tachyarrhythmia has been shown to cause dilated cardiomyopathy in human and animals and this ventricular dysfuntion is usually reversible after control of the arrhythmia. The uncontrolled chronic tachycardia may be a curable cause of dilated cardiomyopathy in some patients. We describe six children who had persistent chronic tachycardia and ventricular dysfunction that improve significantly after control of the arrhythmia. METHODS: We retrosepctively reveiwed the medical records, electrocardiograms, Holter recordings, Echcardiographic reports of 6 children with the tachycardia induced ventricular dysfunction who have been managed at Sejong general hospital and Asan medical center from January 1992 to June 1995. RESULTS: 1) The causes of referral were dilated cardiomyopathy in 2 and tachyarrhythmia in 4. The age at diagnosis was 4 to 36 months old and follow-up period was 6 to 29 months. The symptoms of congestive heart failure were seen in 3 children. 2) The mechanisms of thachyarrhythmia causing cardiomyopathy were atrial ectopic tachycardia in 2, chaotic atrial rhythm in 3 and junctional ectopic tachycardia in 1. Atrial ectopic tachycardia has improved after combined treatment with amidarone and atenonl. One case of chaotic atrial rhythm has improved spontaneously and other 2 cases of chaotic arial rthythm have improved after treatment with digoxin or after combined therapy with amidarone and digoxin. Junctional ectopic tachycardia is partially controlled with mexiletine. 3) The shortening fractions of left ventricle at diagnosis were 14-21% and improved to over 30% in all after 2 to 9 months of follow-up. CONCLUSIONS: We have shown that control of persistent tachycardia resolved the left ventricular dysfunction. We suggest that patients with dilated cardiomyopathy be carefully screened for tachyarrhythmia as a curable cause of ventricular dysfunction and that left ventricular function be assessed in the asymptomatic patients with persistent tachycardia.

Early Results of Radiofrequency Catheter Ablation of Supraventricular Tachycardia in Children

PURPOSE: Although the data of radiofrequency catheter ablation (RFCA) of supraventricular tachycardia in adults has been accumulated in several centers in Korea, few data are available on its efficacy and safety in pediatric ages. We reviewed the data in young patients who underwent this procedure in a pediatric cardiology center, to evaluate the indications, early results, complications, and short-term follow-up data. METHODS: We retrospectively reviewed the medical records and RFCA procedure reports of 17 children who underwent this procedure in pediatric cardiology center, Asan Medical Center, Seoul, Korea from January 1992 to July 1996. The mean follow-up periods was 17.8 months. RESULTS: 1) The median age of the patients was 12.3 years and all of them except two patients had structurally normal hearts. The indications of RFCA were preoperative open heart surgery in a patient, drug refractory tachycardia in 4 patients and patient's choice in other 12 patients. 2) The mechanisms of supraventricular tachycardia were 9 atrioventricular reentrant tachycardia, 6 atrioventricular nodal reentrant tachycardia (AVNRT), 1 atrial flutter and 1 atrial ectopic tachycardia (AET). Among accessory pathways, preexcitation was in 6 patients and accessory pathway located in the right side in 7 patients. In all patient with AVNRT except one, slow pathway was ablated. In the case of AET, ectopic focus located in the left atrium near the orifice of right lower pulmonary vein. 3) 16 of 18 procedures (88.9%) were successful and in 2 occasions with right sided accessory pathway and AET, the procedure failed. Ectopic focus was successfully ablated at the second attempt. The mean total procedure time was 2.7 hours (n=16) and the mean fluoroscopic time was 45.3 minutes (n=12). The average number of RF application was 11 times. There was no significant complications related with procedures. 4) There were 5 recurrences (29.4%), in 2 patients with accessory pathway and 2 patients with AVNRT. Most recurrences occured in 6 weeks after procedure. CONCLUSIONS: RFCA is a good alternative for the treatment of supraventricular tachycardia also in young patients as in adults. Early results of RFCA shows resonably good success rate but somewhat higher rate of recurrence in our center. We expect the results of RFCA in children will be improved in near future after some period of learning curve.

Idiopathic Left Ventricular Tachycardia in Childhood

BACKGROUND: Idiopathic left ventricular tachycardia with a QRS pattern of right bundle branch block and left axis deviation that is sensitive to verapamil is electrophysiologically distinct arrhythmia entity but descriptions in pediatric ages are infrequent. This study attempted to describe the clinical presentation and dignostic clues from the surface ECG in children with idiopathic left venteicular tachycardia. METHODS: We retrospectively reviewed the medical records, electrocardiograms, Holter recordings, echocardiographic reports of 10 children with idiopathic left ventricular tachycardia who have been managed at Sejong General Hospital and Asan Medical Center, Seoul, Korea from January 1991 to July 1996. Follow-up periods are 2 months to 5 years(mean=26 months). RESULTS: 1) Age of tachycardia onset was 8 months to 14 years(median=4.3 years). In 3 children tachycardia began from infancy. The main complaint in older children was palpitation and chest discomfort but young childen complained abdominal pain during tachycardia attack. Tachycardia was paroxysmal and sustained. Tachycardia responded to intravenous use of verapamil in 9 patients and was controlled by chronic verapamil medication in 6 patients. 2 patients were well without medication. Radiofrequency catheter ablation was done in an adolescent patient successfully. 2) In sinus rhythm, surface ECG showed normal QRS axis and normal QRS morphologies in all patients. During ventricular tachycardia, the heart rate was 183 to 270 beats/min. The taxhycardia of RBBB morphology was observed in all patients. The mean QRS duration was 109+-12ms and the QRS axis was -50* to -90*. Themean RS interval was 60+-12ms. AVdissociation was found on surface RCG in all patients and confirmed in 4 patients by transesophageal ECG. We tried rapid transesophageal atrial pacing in 3 patients and could induce and terminate the ventricular tachycardia. CONCLUSIONS: Idiopathic left ventricular tachycaedia is rare in childhood and is seen from infancy. Idiopathic left ventricular tachycardia is generally benign but occasionally causesmyocardial dysfunction. Idiopathic ventricular tachycardia with a QRS pattern of right bundle branch block amd left axis deviation that is sensitive to verapamil in children should not be confused with supraventricular tachycardia with aberrancy and most helpful diagnostic clue is AV dissociation in surface ECG.