• Clinical and Experimental Pediatrics
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• v.52 no.7
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• pp.832-836
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• 2009

Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients diagnosed with plastic bronchitis accompanied with a different underlying disease, which were treated with inhaled corticosteroid and low-dose oral clarithromycin.

• Journal of Chest Surgery
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• v.35 no.7
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• pp.517-522
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• 2002

Background: Congenital heart surgery may lead to myocardial swelling and hemodynamic instability. Delayed sternal closure may be beneficial in this setting. The purpose of this study was to assess mortality and mediastinal infection rate associated with delayed sternal closure after congenital heart surgery and to evaluate the risk factors which affect mortality and mediastinal infection rate. Material and Method: We retrospectively reviewed 40 patients who underwent delayed sternal closure after repair of congenital heart disease at Yonsei Cardiovascular Hospital, from January 1994 to May 2001. In these patients, we assessed the mortality and mediastinal infection rate, and evaluated their risk factors including operation time, bypass time, aortic cross clamp time, duration to sternal closure and postoperative artificial ventilation time. Mediastinal infection was defined to have positive culture in mediastinum. Result: Hemodynamic instability was the most common indication for delayed sternal closure(n=36) and other indications included postoperative bleeding(n=2) and conduit compression(n=2). The median age at operation was $14.4{\pm}33.4$months old(range, 2days-12years). The patients with postoperative bleeding and conduit compression were much older than the others. The sternum was left open for $4.5{\pm}3.4$ days(range, 1-20days). Overall mortality was 25%(10/40) and mediastinal infection occured in 24.3%(9/37) (3 patients were excluded in mediastinal infection for early death). In risk factor analyses, only aortic cross clamp time had statistical significance for mortality in univariate analyses. However, multivariate analyses revealed that there were no significant predictors for risk of mortality and mediastinal infection. Conclusion: Delayed sternal closure after repair of congenital cardiac disease had relatively high mortality and mediastinal infection rate. But, in patients with hemodynamic instability, postoperative bleeding and conduit compression after repair of congenital cardiac disease, delayed sternal closure may be an effective life saving method.

• Journal of Veterinary Clinics
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• v.27 no.6
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• pp.735-739
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• 2010

An 8 month old female Maltese (body weight 3.6 kg) was referred with primary complaints of dyspnea and exercise intolerance. Diagnostic imaging studies revealed marked cardiomegaly and prominent main pulmonary trunk dilation on thoracic radiography, abnormally arisen aortic roots (toward right ventricle) with left-to right shunted perimembraneous ventricular septal defect located underneath of aortic root, aortic root was located to predominantly to the right ventricle and pulmonary regurgitation (peak velocity 4.7 m/s, pressure gradient ~88 mmHg) from pulmonary over-circulation and hypertension on echocardiography, indicating double outlet right ventricle (DORV). The dog was treated with furosemide (1 mg/kg, BID) for reducing volume overload at right ventricle, spironolatcone (1 mg/kg) and enalapril (0.5 mg/kg) for minimizing deleterious cardiac remodeling, and sildenafil (1 mg/kg) for lessening pulmonary over-circulation and hypertension. The clinical condition of this dog was improved after 1 week of medical treatment. The dog is currently survived and regularly monitored.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1354-1359
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• 1996

Few observation have been made on the pericardial pressure and little is known about the composition of he pericardial fluid. So we studied the basic qualitative and quantitative analysis of the pericardial fluid in the patients with cardiac disease either congenital heart diasese(group A) or acquired heart disease(group B). The pressure of the pericardial cavity was measured by the method of open tipped water filled small polyethylene catheter connecting to the standardized monitor, which was introduced into pericardium of the patients who were performed pericardial incision for the heart or pericardial surgery. All of the data was compared to the simultaneously checked hematologic value of the same patient. The mean pressure of the pericardial cavity was 2.4mmHg and the amount of the pericardial fluid was 13cc/m2 of body surface for the group A and 17.7cc for the group B. And the cell count was 138$\pm$l16/1 in group A and 230$\pm$135/1 in group B and the pH was 7.83$\pm$0.40 in group A. 7.80$\pm$0.52 in group B. Pericardial fluid revealed satisfactically significant alkaline pH than plasma. The fundamental electrolyte, Wa+, K+, Cl and glucose were identical to the hematologic values of the same patient, but the protein concentration was 2.Bg/dL for group A and 3.Ig/dL for group B heart disease and those were remarkable low concentration compared to the hematologic value of the same patient. LDH and amylase were identical to the value of the serum of the same patient, but the concentration of LDH of group B was slightly higher than that of the group A.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.518-521
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• 2010

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

• Journal of Veterinary Clinics
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• v.31 no.5
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• pp.403-406
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• 2014

A 6-year-old female Maltese (body weight, 3.1 kg) without clinical signs was referred for further evaluation of the cause of cardiac murmur. Thoracic radiography revealed right-sided cardiomegaly. Echocardiography showed marked hypertrophic remodeling of the right ventricular free wall and an anomalous muscular bundle and fibrous nodule near the subinfundibular portion of the right ventricular outflow tract (RVOT), indicating a double-chambered right ventricle (DCRV). The turbulent flow from the anomalous muscular bundle to the main pulmonary artery was 4.6 m/sec, in addition to the tricuspid valvular regurgitation of 4.4 m/sec and main pulmonary artery flow of 1.1 m/sec. The dog is receiving atenolol (0.5 mg/kg) to minimize the deleterious cardiac effects of the high afterload, even though she remains asymptomatic. This report describes a case of DCRV, a rare congenital heart disease in dogs in South Korea.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.18-27
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• 2006

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. Material and Method: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. Result: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3$\∼$33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7$\∼$11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114$\pm$37 minutes, and the average aortic cross clamping time was 55$\pm$22 minutes. The average stay of intensive care unit was 5$\pm$3 days, the mean mechanical ventilator time was 38$\pm$45 hours and the hospital stay after operation was 12$\pm$5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2$\pm$ 10.3$\%$ to 60.5$\pm$ 15.8$\%$ within 1 month and to 59.8$\pm$13.9$\%$ within 1 year after operation, the SF (Shortening Fraction) increased from 23.6$\pm$4.7$\%$ to 38.6$\pm$8.4$\%$ within 1 month and to 37.4$\pm$7.9$\%$ within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8$\pm$25.6 mm/$m^{2}$ to 90.3$\pm$ 19.2 mm/$m^{2}$ within f month and to 79.3$\pm$ 15.8 mm/$m^{2}$ within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy Conclusion: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important. But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.167-172
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• 2003

Purpose : Pulmonary vascular hypertension is a common problem in congenital heart disease, the most common cardiac condition in childhood. However, the mechanisms responsible for this pathologic change, treatment, and prevention are poorly understood. Therefore, we studied the gene expression of vascular endothelial growth factor(VEGF) by using a hypoxic model of the pulmonary artery smooth muscle cells. Methods : The main pulmonary artery and its proximal branches of a 6 wk old Fischer rat were excised. They were cut into multiple small pieces and suspended in DMEM medium supplemented with 20% fetal bovine serum and incubated in 5% $CO_2$-95% air atmosphere. The smooth muscle cells were confirmed by immunostaining with smooth muscle myosin and ${\alpha}$-smooth muscle actin antibodies. The VEGF gene expression in the hypoxic group was compared with the one in control the group as well as the one in the starved group by RT-PCR and Northern blot hybridization. Results : There was no statistically significant difference among the control, hypoxic and starved groups. Conclusion : There are few studies of pulmonary vascular hypertension at the molecular level in Korea. Therefore, we studied the expression of VEGF gene in hypoxic pulmonary vascular smooth muscle cells. Further studies will be needed to find the difference between newly born and adult rats, or human and rat pulmonary vascular smooth muscle cells in gene expression. We hope that the study will lead to a better understanding of pulmonary vascular hypertension.

• Neonatal Medicine
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• v.18 no.2
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• pp.395-398
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• 2011

Sick sinus syndrome (SSS) is a disorder characterized by sinus node dysfunction. Although the condition is most common in the elderly, it can occur in children including neonates and its recognition and treatment are important. The diagnosis of SSS is based on the presence of sinus bradycardia, sinus arrest or exit block, combinations of sinoatrial and atrioventricular nodal conduction disturbances, and atrial tachyarrhythmias documented in the Holter recordings. In most children with SSS, previous history of congenital heart malformation or cardiac surgery is noted. SSS is also seen in the children including neonates without heart disease or other contributing factors, however SSS is most often idiopathic. The treatment of SSS depends on the basic rhythm problem, but generally involves the placement of a cardiac pacemaker. We report a case of SSS in extremely low birth weight infant without congenital heart disease and suggest that the treatment system is necessary for preterm infants with SSS.

• Journal of Preventive Medicine and Public Health
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• v.34 no.4
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• pp.427-436
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• 2001

Objective : To estimate the prevalence of congenital heart disease from the 1990 student heart disease screening program. Methods : The heart disease screening program for elementary students was conducted in Kyonggi-do, in 1998. The subjects of the present study comprised the 40,402 students who attended the schools in the catchment area of a collaborative university hospital and who participated in the primary examination. The congenital heart disease (CHD) patients were initially identified through a questionnaire about prior medical history, and further through diagnostic tests & medical examinations in the secondary & the tertiary examinations. Certain assumptions were used in the estimation of the number of CHD cases among non-participants of the secondary & tertiary examinations. The overall prevalence of CHD was estimated by adding the CHD detection rates of the participants and the estimated prevalence of the non-participants. Results : Among the 40,402 primary participants, 1,655 were referred further, of whom 79.1% (1,309) participated in the secondary examination. Of these, 121 were referred to the tertiary examination, with a participation rate at this last stage of 80.2%. The positive predictive value (PPV) of the screening tools was the highest when the results of both EKG and the questionnaire were positive. Because 85.9% of the detected cases had a past history of CHD, PPV was higher when the selection criteria in the questionnaire included past CHD history than when it didnt. The CHD defection rate among the participants was 1.76 cases/1,000 and the presumed number of cases among the non-participants was 31; giving an estimated final CHD prevalence of 2.52 cases/1,000 (95% CI : 2.06-3.06). Among the identified cases of CHD, VSD (52.8%) was the most common, followed by PDA (9.7%), TOF (9.7%) & PS (9.7%). Conclusion : Because the characteristics of the non-participants differed from those of the participants, the estimation of prevalence was influenced by the participation rate. Of the detected cases, 85.9% had a past history of diagnosis or operation for CMD. These findings suggested that the prevalence estimated in this study may be an underestimation of the actual condition. Therefore, a birth cohort study is required in order to more accurately estimate the prevalence and the effects of the program.