• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.976-981
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• 2007

Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by echocardiography from January 2002 to December 2003. Of 130 patients, we identified 17 patients with AoRo dilatation, which was defined as ratio of expected AoRo size by standard nomogram (AoRo ratio) >1.5 (dilator group), and 113 TOF controls, with AoRo ratio <1.5 (non-dilator group). Results : Mean indexed AoRo size ($mm/m^2$) in the first echo was $24{\pm}3.2$ in the dilator group and $18{\pm}3.4$ in the non-dilator group (P<0.0001). AoRo rate of change (mm/year) from the first to latest echo study was $1.6{\pm}3.8$ in dilator group and $0.05{\pm}1.6$ in the non dilator group (P=0.0021). Patients from the dilator group showed a higher prevalence of pulmonary atresia (P=0.031) and a history of aortopulmonary shunt before repair (P=0.048), moderate to severe AR (P=0.0065), and increased left ventricular end-diastolic dimensions (P=0.003). Conclusions : A subset of patients late after TOF repair may show progressive dilatation of AoRo. To identify and prevent long-term sequelae in this patient group, regular follow-up and speculation about AoRo after TOF repair is recommended.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.970-975
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• 2007

Purpose : Accurate measurement of defect size is important in transcatheter closure of atrial septal defect (ASD). We performed this study to analyze the difference between the measured ASD size and balloon occlusive diameter (BOD) by transthoracic (TTE) or transesophageal echocardiography (TEE). Methods : We investigated 78 patients who underwent transcatheter closure of ASD. The defect size and the distance between the surrounding structures were measured by TTE and TEE. The BOD was measured by TEE during cardiac catheterization. Clinical characteristics and echocardiographic data were compared and analyzed. Results : The difference between BOD and diameter by TTE was $4.8{\pm}3.6mm$ on short axis view, $5.4{\pm}3.2mm$ on long axis view. The difference between BOD and diameter by TEE was $3.6{\pm}2.2mm$ on short axis view, $4.2{\pm}3.1mm$ on long axis view. The difference between BOD and the diameter of defects on TTE, TEE had statistically significant positive correlations with the age of the patients, distance between the, defect and posterior atrial septal wall, the distance between the defect and the mitral valve leaflet, and the diameter of defects and the length of the atrial septum on TTE (P<0.05). Conclusion : BOD of ASD can be estimated by the diameter on TTE and TEE. BOD is expected to measure larger, depending on the size of defects, the distance from surrounding structures and the location of defects on echocardiography. Our data offers important information on details of transcatheter ASD closure which can be helpful in predicting suitability and judging the procedural appropriateness during the procedure.

• Clinical and Experimental Pediatrics
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• v.57 no.5
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• pp.232-239
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• 2014

Purpose: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. Methods: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. Results: The LV mass-for-height z scores were significantly below normal in children with MD ($-1.02{\pm}1.52$, P<0.001) or DMD ($-0.82{\pm}1.61$, P =0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R =0.377, P<0.001) and those with DMD (R =0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R =0.462, P<0.001) and cardiac index (R =0.358, P<0.001). Conclusion: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients.

• Journal of Chest Surgery
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• v.46 no.6
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• pp.391-401
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• 2013

Mechanical circulatory support (MCS) in the pediatric heart failure population has a limited history especially for infants, and neonates. It has been increasingly recognized that there is a rapidly expanding population of children diagnosed and living with heart failure. This expanding population has resulted in increasing numbers of children with medically resistant end-stage heart failure. The traditional therapy for these children has been heart transplantation. However, children with heart failure unlike adults do not have symptoms until they present with end-stage heart failure and therefore, cannot safely wait for transplantation. Many of these children were bridged to heart transplantation utilizing extracorporeal membranous oxygenation as a bridge to transplant which has yielded poor results. As such, industry, clinicians, and the government have refocused interest in developing increasing numbers of MCS options for children living with heart failure as a bridge to transplantation and as a chronic therapy. In this review, we discuss MCS options for short and long-term support that are currently available for infants and children with end-stage heart failure.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.895-897
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• 2006

Systemic arterial supply from the aorta to the lung is a rare congenital anomaly within the spectrum of bronchopulmonary sequestration according to Pryce's terminology. We describe our experience of this anomaly in an infant with congenital cardiac disease confirmed by multidetector CT scan. We found a systemic arterial supply from the aorta to the right lower lobe of lung without right lower lobar pulmonary artery and bronchopulmonary sequestration. This combination of congenital anomaly is most rare form.

• Journal of Chest Surgery
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• v.38 no.11 s.256
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• pp.773-775
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• 2005

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 1140g premie with TGA, IVS after 4 week care 1317gm.

• Clinical and Experimental Pediatrics
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• v.64 no.2
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• pp.49-59
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• 2021

Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.630-634
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• 2009

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.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.238-243
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• 2006

Chemoport is widely used in pediatric surgery field. But various complications can occur during the process of insertion or removal of chemoport. Surgeons must be familiar with the treatment of these complications. We had one catheter cuts off during chemoport removal, become a catheter embolism. Interventional radiologic removal was successful. Verifying the length of removed catheter and careful observation of the catheter tip during removal procedure is important to prevent the possibility of catheter embolus. Radiologic intervention was accessible to remove the retained catheter.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.494-498
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• 2009

Purpose : Applicability of transcatheter closure of atrial septal defect (ASD) has been expanded by accumulation of clinical experiences and evolutions of the device. This study was performed to evaluate the safety and efficacy of transcatheter closure of ASD with Amplatzer septal occluder (ASO) in young children less than 3 years of age. Methods : From May 2003 to December 2005, 295 patients underwent transcatheter closure of ASD with ASO in the Severance Cardiovascular Hospital, Yonsei University Health System. Among them, 51 patients less than 3 years of age were enrolled in this study. We investigated procedural success rate, rate of residual shunt, frequency of complications, procedure/fluoroscopy time, and need of modified techniques for device implantation. Results : The median age was 2.1 years and median body weight was 12 kg. Implantation of device was successful in 50 patients (98%). Seven patients (15%) showed a small residual shunt 1 day after the procedure, but complete occlusion had been documented at 6 month follow-up in all patients (100%). The pulmonary to systemic flow ratio (Qp/Qs), peak systolic pulmonary artery pressure, and peak systolic right ventricular pressure had decreased significantly after closure of ASD. There were 2 complications including device embolization (1, 2%) and temporary groin hematoma (1, 2%). Conclusion : Transcatheter closure of ASD with ASO can be performed with satisfactory results and acceptable risk even in young children less than 3 years of age. We could suggest that even in very young children with ASD, there is no need to wait until they grow to a sufficient size for the transcatheter closure.