• Journal of Chest Surgery
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• v.23 no.3
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• pp.430-437
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• 1990

We clinically evaluated 121 cases of ventricular septal defect which we operated from April, 1986 to December, 1989 at Inha General Hospital, Seong-Nam, Department of Thoracic and Cardiovascular Surgery, College of Medicine, Inha University. These patients were occupied 54.8% of all congenital heart diseases operated on its same period. Of the 121 patients, 63 patients were male[52.1%] and 58 patients were female[47.9i]. The two most common symptoms were frequent upper respiratory infection and dyspnea on exertion. By Kirklin s anatomical classification, type I constituted 34.7%, type II 61.98%, type III 0.03% and type IV not occupied. Associated cardiac anomalies were found in 34 cases, and PDA was most common associated anomaly, occupied in 22 cases. On the cardiac catheterization data, there were statistically significant correlation between VSD size[cm2 /BSAm2] and systolic pulmonary arterial pressure[sPAP], pulmonary to systemic flow ratio[Qp/Qs] & pulmonary to systemic pressure ratio[Pp/Ps] respectively, Type II [r=0.53, p<0.01] was more correlated than type I [r=0.49, p<0.05] between VSD size and Qp /Qs. We could not found the correlationship between age and Qp/Qs [Type I; r=0.16, Type II; r=-0.15] All cases were operated under cardiopulmonary bypass and 58 cases[46.3%] were operated through the right atrial approach, and 34 cases[28.1%] through the pulmonary arterial approach. Operative mortality rate was 4.13%[5 cases].

• Journal of Chest Surgery
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• v.49 no.5
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• pp.379-382
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• 2016

With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute. We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return.

• Journal of Genetic Medicine
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• v.16 no.1
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• pp.1-9
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• 2019

Noonan syndrome (NS) and NS-related disorders (cardio-facio-cutaneous syndrome, Costello syndrome, NS with multiple lentigines, or LEOPARD [lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth and sensory neural deafness] syndrome) are collectively named as RASopathies. Clinical presentations are similar, featured with typical facial features, short stature, intellectual disability, ectodermal abnormalities, congenital heart diseases, chest & skeletal deformity and delayed puberty. During past decades, molecular etiologies of RASopathies have been growingly discovered. The functional perturbations of the RAS-mitogen-activated protein kinase pathway are resulted from the mutation of more than 20 genes (PTPN11, SOS1, RAF1, SHOC2, BRAF, KRAS, NRAS, HRAS, MEK1, MEK2, CBL, SOS2, RIT, RRAS, RASA2, SPRY1, LZTR1, MAP3K8, MYST4, A2ML1, RRAS2). The PTPN11 (40-50%), SOS1 (10-20%), RAF1 (3-17%), and RIT1 (5-9%) mutations are common in NS patients. In this review, the constellation of overlapping clinical features of RASopathies will be described based on genotype as well as their differential diagnostic points and management.

• The Journal of Internal Korean Medicine
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• v.45 no.1
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• pp.100-110
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• 2024

This case report demonstrates the effect of modified Bangibokryeong-tang (Fangji Fuling Decoction), a traditional herbal prescription in treating dyspnea and palpitations, symptoms that often lead to anxiety and reduced quality of life of cardiac patients. A female patient in her late 70s with congenital valve malformation and atrial fibrillation presented with dyspnea and palpitations, each rated at a severity of 8 on a numeric rating scale (NRS). After voluntarily discontinuing Western medication, she received modified BGBRT for 25 days, leading to significant symptom relief and NRS improvement to 0-2. The patient reported maintaining an improved condition and showed a significant increase in vitality. This improvement was sustained for 7 months, but the symptoms recurred; thus, modified BGBRT was restarted. Modified BGBRT showed substantial effects on persistent cardiac-origin dyspnea and palpitations. Thus, this TKM has the potential to manage symptoms and enhance the quality of life of cardiac patients.

• Journal of Chest Surgery
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• v.25 no.7
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• pp.693-701
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• 1992

Extracorporeal cardiopulmonary bypass[CPB] has been associated with a wide variety of hematologic derangements, including a transient deformation and hemolysis of red blood cells[RBCs], which is supposed to be due to mechanical trauma and/or metabolic alterations. Since membrane integrity is, in part, maintained by energy requiring process, inadequate function of erythrocyte glycolytic pathway, which is inevitalble during CPB, may cause depletion of high energy phosphate pool and result in hemolysis. The authors performed an investigation to assess whether administration of Fructose-l, 6-diphsphate [FDP], which has been known to enhance intracellular glycolytic activities, could counteract erythrocyte hemolytic events caused by CPB. Sixty pateints with cyanotic congenital heart diseases, who underwent open heart surgery under CPB longer than 60 minutes, were randomly divided into two groups depending on whether use of FDP[Group FDP] or not[Group Control]. The age, sex, CPB time, preoperative hemoglobin level, disease entities were all similar[Table 1], and membrane type oxygenators were used in all patients. In Group, FDP, a dose of 250mg/kg body weight of FDP was administered by intravenous dripping every 12 hours from the morning of the operation to postoperative 48 hours, To demonstrate the degree and pattern of hemolysis of erythrocyte, reticulocyte count, indirect /direct bilirubin, haptoglobin, plasma hemoglobin, lactate dehydrogenase were measured every 12 hours from the time of cessation of CPB to 48 hours and RBC morphologic study, osmotic fragility test were done every 24 hours. All parameters revealed less hemolytic in group FDP [Fig. 1~5], though the differences between two groups were not significant, except plasma hemoglobin, lactate dehydrogenase changes. A pattern of sequential changes of plasma hemoglobin, lactate deh-ydrogenase showed the highest level at the time of CPB stop and abrupt decrease in following 24 hours in both groups, and statistically significant differences were demonstrated in group FDP at least for the first 12 hours postoperatively[p<0.05]. The authors conclude that they can expect the benificial effect of FDP on the maintenance of membrane stability of RBC probably by energy enhancement during the shock status of CPB, but FDP could not completely prevent the damaging effect on RBC by cardiopulmonary bypass

• Journal of Chest Surgery
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• v.36 no.7
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• pp.483-488
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• 2003

There are still debates in the literature on the relative benefits of blood cardioplegia and crystalloid cardioplegia in pediatric cardiac surgery. We performed a clinical trial to compare the myocardial protective effect between HTK solution and blood cardioplegic solution in congenital heart surgery. Material and Method: 15 patients who underwent HTK solution cardioplegia (group 1) and 15 patients who underwent blood cardioplegia(group 2) were included in this study. Preoperative and postoperative serial serum cardiac enzyme levels (troponin I, CK-MB, LDH) were measured in all patients. Clinical data were analyzed and compared between the two groups. Result: There were no differences in age and body weight between the two groups. Operative diagnosis included ventricular septal defect (VSD, n＝4), atrial septal defect (ASD, n＝1), tetralogy of Fallot (TOF, n＝4), and other complex heart diseases (n＝6) in group 1, VSD (n＝7), ASD (n＝5), and TOF (n＝3) in group 2. Cardiopulmonary bypass times were 99.1$\pm$48.1 minutes in group 1, and 69.3$\pm$27.3 minutes in group 2 (p＝0,02). Aortic clamping times were 52.1$\pm$23.6 minutes in group 1, and 37.9$\pm$20.5 minutes in group 2 (p＝0.07). There was no mortality and spontaneous defibrillation was possible in all patients. No differences were observed in the serial enzyme levels between the two groups. There were no differences in the duration of inotropic support and ventilator time between the two groups. Conclusion: HTK solution provided comparable myocardial protection compared with blood cardioplegic solution. A single high dose of HTK solution may be safely and conveniently used for an extended periods as well in congenital heart surgery.

• Child Health Nursing Research
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• v.6 no.2
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• pp.249-261
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• 2000

The purpose of this study were to identify the quality of life for the mothers of hospitalized chronic pediatric patients, and to explore the factors affecting the QOL of those mothers. The subjects were 201 mothers whose children had hospitalized at one University hospital with chronic diseases. Data were collected from the December, 1997 to December, 1998. We used a revised QOL instrument consisting of 34 items, 5 point likert scale based on the Noh's QOL instrument. The revised QOL consists of six subscales, those are physical status and function, self esteem, emotional status, economic status, relationship with family members, and relationship with neighborhood. Data were analyzed by t-test, ANOVA, Pearson's correlation using SPSS-PC. The results were as follows: 1. The mean score of quality of life for the mothers of chronic pediatric patients was 100.31, and item mean was 2.95. In subscale analysis, item mean of economic status was the lowest, and that of relationship with family members was the highest. 2. Correlations between characteristics of chronic pediatric patients, their mothers and QOL of mothers were as follows; 1) Total QOL of mothers had a significant positive relationship with progressing time relapse after diagnosing and age of mothers. There was a significant negative relationship between the total QOL of mothers and number of hospitalization of their children. 2) QOL on self esteem and economic status had a significant relationship with age of pediatric patients, the time relapse after diagnosis, and age of mothers. Total number of family members and QOL on economic status showed a significant positive relationship. QOL on emotional status, economic status, and relationship with family members of mothers showed negative correlations with the number of hospitalization of their children. 3. Followings were the result of difference in QOL among different demographic cha- racteristics of the subjects. 1) QOL on economic status of mothers was significantly higher when fathers of pediatric patients had jobs. 2) Total QOL score, QOL on emotional status, and QOL on relationship with neighborhood were significantly higher when mothers of pediatric patients had spouses. 3) QOL on self esteem of mothers was significantly higher when mothers had religion. 4. Followings were the result of difference in QOL among different diagnosis of the children. 1) Total QOL score of mothers whose children had congenital heart disease was higher than that of mothers whose children had leukemia and cancer. 2) QOL on emotional status, economic status, and relationship with family members of mothers whose children had congenital heart disease were higher than those of mothers whose children had leukemia, cancer, and epilepsy.

• Tuberculosis and Respiratory Diseases
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• v.64 no.1
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• pp.28-32
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• 2008

Pulmonary arterial thrombosis develops during hypercoagulable states, intra-arterial tumorous conditions, and congenital heart disease accompanied by pulmonary hypertension. Thrombosis in the main pulmonary arterial stump after pneumonectomy can also occur. Herein, we report a very rare case of pulmonary arterial thrombosis in a patient with pulmonary hypertension and a lung destroyed by tuberculosis. He presented with aggravated dyspnea without fever or purulent sputum. His chest computerized tomography scan showed left main pulmonary arterial thrombosis as a convex shape, with the ipsilateral distal arteries and arterioles showing parenchymal destruction. After excluding pulmonary thromboembolism and hypercoagulable disorders, we diagnosed pulmonary arterial thrombosis and treated him with an anticoagulant.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.548-553
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• 1992

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.337-343
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• 2002

A tracheal bronchus, an aberrant bronchus arising directly from the trachea, is an infrequent congenital anomaly. The incidence of this anomaly ranges from 0.5 to 5%. It usually originates from the right lateral wall of the trachea at the level <2 cm above the tracheal bifurcation. These patients usually are asymptomatic, but some patients may experience recurrent pneumonia, chronic bronchitis, bronchiectasis, or asthmatic episodes. A tracheal bronchus may be associated with other anomalies such as a tracheal stenosis, pulmonary agenesis, pulmonary sequestration, congenital heart disease, a pulmonary venous anomaly and Down's syndrome. This anomaly is usually diagnosed incidentally during bronchoscopy in patients with respiratory problems. Here we report a case of a 20-year-old man with a past history of bronchial asthma, which was incidentally diagnosed as a tracheal bronchus during a medical examination prior to military service, and was associated with a bilateral superior vena cava anomaly.