• Biomedical Science Letters
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• v.12 no.3
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• pp.289-301
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• 2006

Cardiopulmonary bypass (CPB) for cardiac surgery triggers the production and release of numerous chemotactic substances and cytokines, ensuing systemic inflammatory response that leads to postoperative major organ dysfunction. Traditionally, corticosteroids (steroid) have been administered to patients undergoing cardiac surgery to ward off these detrimental physiologic alterations. However, the majority of the studies have been performed on adult patients with high-dose steroid. We carried out a randomized, prospective, double-blind study to compare the efficacy of low-dose steroid with that of high-dose steroid and to determine the adequate dose of pretreated-steroid for prophylactic effects in pediatric cardiac surgery. Thirty pediatric patients scheduled for elective cardiac surgery were randomly assigned to two groups; fifteen patients received low-dose methylprednisolone (10mg/kg intravenously, n=15, low-dose group) and the others received high-dose methylprednisolone (30mg/kg intravenously, n=15, high-dose group) 1 hour prior to CPB. Arterial blood samples were taken before CPB (Pre-CPB), 10 minutes after start of CPB (CPB-10), and immediately after CPB-end (CPB-OFF) for measuring total leukocyte counts (T-WBC) and diff-counts, platelet counts, interleukin-6 (IL-6), myeloperoxidase (MPO), total antioxidant (TAO), neuron-specific enolase (NSE), troponin I (TNI), aspartate aminotransferase (AST), alanine aminotransferase (ALT), creatinine, and blood urea nitrogen (BUN) levels. Other parameters such as volumes of urine output, pulmonary index $(PI,\;PaO_2/FiO_2)$, mechanical ventilating period, intensive care unit (ICU)-staying period, postoperative complications (fever, wound problem), postoperative 24 hrs and total volumes in blood loss, and hospitalized days were also assessed. All parameters were compared between two groups. There were no significant differences in T-WBC counts, monocyte fraction, platelet counts, TA levels, NSE levels, creatinine levels, BUN levels, the volumes of total urine output, PI, the incidences of fever and wound problem, postoperative 24hrs- and total-blood loss volumes and ICU-staying period between two groups (P>0.05). At CPB-OFF, neutrophil fraction, MPO level, TNI level, and AST level were higher in the high-dose group than in the low-dose group (P<0.05). IL-6 level at CPB-10 was higher in the high dose-group than in the low-dose group (P<0.05). Furthermore, mechanical ventilating periods and hospitalized days of the high-dose group were significantly longer than those of low-dose group (P<0.05). The high-dose group had significantly low lymphocyte fi-action at CPB-OFF compared with the low-dose group (P<0.001). These findings suggest that pretreatment of high-dose steroid is not superior to that of low-dose steroid regrading its potential benefits in pediatric cardiac surgery. Therefore, the conventional strategy of steroid treatment, high-dose pretreatment, should be modified in the cardiac surgery with CPB. However, further studies must be performed on the larger number of patients in as much as small number of patients in this study.

• Journal of Chest Surgery
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• v.48 no.4
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• pp.272-276
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• 2015

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.81-87
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• 2011

This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19 % of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8 %). But 47.6 % were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arterious (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8 %), anorectal malformations were the most frequent, 70 % Vertebral and limb abnormalities accounted for 11.9 % and urogenital malformations 9.5 % of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8 % and 1.8% had full VACTERL. Almost 12 % of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8 % of patients in our study, only 21.42 % needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.

• Clinical and Experimental Pediatrics
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• v.63 no.4
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• pp.151-156
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• 2020

Background: Acute kidney injury (AKI) is one of the most significant postoperative complications of pediatric cardiac surgery. Because serum creatinine has limitations as a diagnostic marker of AKI, new biomarkers including neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and interleukin-18 (IL-18) are being evaluated to overcome these limitations and detect AKI at an early stage after cardiac surgery. Purpose: This study aimed to investigate the clinical usefulness of these biomarkers in young children. Methods: Thirty patients with congenital heart diseases who underwent cardiac surgery using cardiopulmonary bypass (CPB) were selected, and their urine and blood samples were collected at baseline and 6, 24, and 48 hours after surgery. Serum creatinine and blood urea nitrogen levels as well as NGAL, KIM-1, and IL-18 levels in urine samples were measured, and clinical parameters were evaluated. Results: Of the 30 patients, 12 developed AKI within 48 hours after cardiac surgery. In the AKI group, 8 of 12 (66.6%) met AKI criteria after 24 hours, and urine KIM-1/creatinine (Cr) level (with adjustment of urine creatinine) peaked at 24 hours with significant difference from baseline level. Additionally, urine KIM-1/Cr level in the AKI group was significantly higher than in the non-AKI group at 6 hours. However, urine NGAL/Cr and IL-18/Cr levels showed no specific trend with time for 48 hours after cardiac surgery. Conclusion: It is suggested that urine KIM-1/Cr concentration could be considered a good biomarker for early AKI prediction after open cardiac surgery using CPB in young children with congenital heart diseases.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.536-545
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• 2007

Background: Compared to adult patients, mitral regurgitation in pediatric patients is uncommon and it shows a wide spectrum of morphologic abnormalities. We retrospectively evaluated the midterm results of mitral valve repair in pediatric patients. Material and Method: Between December 1993 and August 2006, mitral valve repair was performed in 35 patients who were aged less than 18 years, The mean age was $5.3{\pm}5.3$ years and the mean body weight was $20,0{\pm}16.3\;kg$. 18 patients had associated cardiac anomalies. The most common pathologic finding was leaflet prolapse (n=17). The most common method of repair was the double orifice technique (n=15). Result: There was no early mortality. Eight patients underwent reoperation (24.2%), and five of them required mitral valve replacement. Among the four ring annuloplasty cases, two have developed mitral stenosis. Four out of the 14 double orifice cases required reoperation. One case of early mortality and one case of late mortality occurred in the reoperation cases. The 5-year survival rate and the freedom from reoperation rate were $93.3{\pm}4.6%$ and $76.1{\pm}8.2%$, respectively. The 5-year freedom from mitral valve replacement rate was $83.6{\pm}6.7%$. There was no significant risk factor for reoperation. Conclusion: The midterm results of mitral valve repair are very acceptable in pediatric patients compared to the adult cases, although the reoperation rate is slightly higher.

• Journal of Chest Surgery
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• v.49 no.2
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• pp.107-111
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• 2016

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.

• Advances in pediatric surgery
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• v.3 no.2
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• pp.164-167
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• 1997

Conjoined twins are one of the rarest and most challenging congenital anomalies in pediatric surgery. Successful surgical separation is difficult because it the majority of conjoined twins in is technically typical to separate shared vital organs successfully. The timing of separation is variable, but separation is usually delayed until such infants are relatively mature(i.e, 9-12 months of age). Operative survival was 50 % in the neonatal period, but 90 % in those over 4 months of age. The present case was successfully separated early beacase of cardiac problems in one of the twins. These twins were omphalopagus and only the liver was shared through a bridge. The vascular and biliary trees were independent from each other. Successful surgical separation was on 11th day of life, but one died of sepsis 18 days after operation.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.68-72
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• 2009

Neuroblastoma is the most common extracranial solid tumor in children, and accompanies various clinical symptoms including hypertension. Hypertension is associated with catecholamines secreted from the tumor, and is usually not severe. We report one case of malignant hypertension with cardiac failure in a patient with adrenal neuroblastoma, successfully treated with adrenalectomy. A 3 year-old boy complained of protrusion of the chest wall. Physical examination revealed severe hypertension with cardiac failure. The levels of metabolites of catecholamine were increased in blood (norepinephrine >2000 pg/mL) and urine (norepinephrine 1350.5 ug/day). Abdominal CT showed a 7 cm-sized solid mass arising from the right adrenal gland. After stabilizing the hemodynamics with oral phenoxybenzamine, right adrenalectomy was performed. Pathological diagnosis was a ganglioneuroblastoma. The hypertension and cardiac failure were resolved after tumor removal.

• Journal of Chest Surgery
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• v.39 no.3 s.260
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• pp.226-229
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• 2006

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.

• Journal of Chest Surgery
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• v.49 no.4
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• pp.292-294
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• 2016

Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most patients with heart failure treated with CRT are adults, middle-aged or older with idiopathic or ischemic dilated cardiomyopathy. We treated a 12-year-old boy, who was transferred after cardiac arrest, with dilated cardiomyopathy, left bundle-branch block, and ventricular tachycardia. We performed cardiac resynchronization therapy with a defibrillator (CRT-D). After CRT-D, left ventricular ejection fraction improved from 22% to 4 4% assessed by echocardiogram 1 year postoperatively. On electrocardiogram, QRS duration was shortened from 206 to 144 ms. The patient's clinical symptoms also improved. For pediatric patients with refractory heart failure and ventricular arrhythmia, CRT-D could be indicated as an effective therapeutic option.