• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.917-929
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• 2006

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

• 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.

• Clinical and Experimental Pediatrics
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• v.55 no.7
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• pp.254-258
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• 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.

• Clinical and Experimental Pediatrics
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• v.55 no.8
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.208-213
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• 2002

Purpose : The major cause of cardiac dysfunction, after open heart surgery for congenital heart disease, is perioperative myocardial injury. Cardiac troponin I is found only within the myocardial cell, so it can be used as a biochemical marker of the myocardial injury. We performed this study to evaluate the worth of cardiac troponin I as a biochemical marker reflecting the extent of perioperative myocardial injury and recovery. Methods : Thirty-four patients who had undergone elective open heart surgery of congenital heart disease(CHD) from April to July 2001 were enrolled in this study. We measured types of CHD, serial cardiac troponin I(baseline 1 day before operation, postoperative day 1, 2, 3, 7), duration of cardiopulmonary bypass(CPB), aortic cross clamping(ACC), intubation and postoperative hospital stay. Results : Compared with the baseline before operation, there was a significant, increase of cardiac troponin I on the postoperative day 1 and a significant gradual decrease on the day 2, 3, 7. The levels of cardiac troponin I were the highest in the transposition of great artery(TGA) repair on the postoperative day 1 and high in the tetralogy of Fallot(TOF), atioventricular septal defect (AVSD), ventricular septal defect(VSD) and atrial septal defect(ASD) repair with decreasing sequence. The longer duration of CPB, ACC and intubation, the higher of cardiac troponin I, but there were no significant correlations between cardiac troponin I levels and duration of hospital stay. Conclusion : Because there was significant increases or decreases of cardiac troponin I according to the perioperative time and types of the congenital heart disease, it is a worthy biochemical marker which reflects the extent of perioperative myocardial injury and recovery after open heart surgery.

• Clinical and Experimental Pediatrics
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• v.59 no.2
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• pp.59-64
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• 2016

Purpose: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Methods: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Results: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. Conclusion: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

• Clinical and Experimental Pediatrics
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• v.57 no.5
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• pp.222-225
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• 2014

Purpose: A recent study analyzing several cytokines reported that long cardiopulmonary bypass (CPB) time and long aortic cross clamp (ACC) time were accompanied by enhanced postoperative inflammation, which contrasted with the modest influence of the degree of hypothermia. In this present study, we aimed to examine the effect of CPB temperature on the clinical outcome in infants undergoing repair of isolated ventricular septal defect (VSD). Methods: Of the 212 infants with isolated VSD who underwent open heart surgery (OHS) between January 2001 and December 2010, 43 infants were enrolled. They were classified into 2 groups: group 1, infants undergoing hypothermic CPB ($26^{\circ}C-28^{\circ}C$; n=19) and group 2, infants undergoing near-normothermic CPB ($34^{\circ}C-36^{\circ}C$; n=24). Results: The age at the time of the OHS, and number of infants aged<3 months showed no significant differences between the groups. The CPB time and ACC time in group 1 were longer than those in group 2 (88 minutes vs. 59 minutes, P =0.002, and 54 minutes vs. 37 minutes, P =0.006 respectively). The duration of postoperative mechanical ventilation was 1.6 days in group 1 and 1.8 days in group 2. None of the infants showed postoperative neurological and developmental abnormalities. Moreover, no postoperative differences in the white blood cell count and C-reactive protein levels were noted between two groups. Conclusion: This study revealed that hypothermic and near-normothermic CPB were associated with similar clinical outcomes and inflammatory reactions in neonates and infants treated for simple congenital heart disease.

• Neonatal Medicine
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• v.15 no.2
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• pp.176-182
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• 2008

A congenital portosystemic shunt is a very rare portosystemic vascular anomaly which leads to jaundice, hypoglycemia, hyperammonemia, liver cirrhosis, hepatic coma, and pulmonary hypertension. Anatomically, portosystemic shunts are divided into intra- and extrahepatic shunts. Congenital intrahepatic portosystemic shunts are rare anomalies, and the early diagnosis is important to prevent hepatic encephalopathy and hypoglycemia. We report a case of an infant with symptoms of heart failure due to a congenital intrahepatic portosystemic shunt and a ventricular septal defect (VSD), which were treated successfully with four coil embolizations and open heart surgery for the VSD.

• Journal of Chest Surgery
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• v.53 no.1
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• pp.16-21
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• 2020

Background: Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. Methods: The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. Results: Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. Conclusion: Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.

• Journal of Chest Surgery
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• v.38 no.7 s.252
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• pp.501-503
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• 2005

Coronary artery fistula accounts for $0.27\~0.4\%$ of all congenital cardiac defects. In more than $50\%$ of the cases, right coronary artery is involved. The fistula drains into the right heart in $92\%$ of the cases. Left heart is the site of termination in only $8\%$ of the cases, especially less left ventricle $(3\%)$. We experienced a case of right coronary artery to left ventricular fistula in a 3-year-old boy who was diagnosed incidentally and underwent ligation of fistula.