• Journal of Chest Surgery
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• v.10 no.1
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• pp.106-112
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• 1977

Transposition of the great arteries is one of the commonest forms of severe congenital heart disease and produces severe cyanosis threatening survival from the day of birth. Anatomical anomalies which the aorta arises from the infundibulum of the right ventricle and the pulmonary artery arises from the outflow tract of the left ventricle make the deranged circulation. Survival is possible only if additional anomalies are present which allow mixing of the pulmonary and systemic circulations. Preoperative diagnosis as TGA was taken on the 15 day old female via the preoperative examination and the right cordioangiography. As palliative treatment for cyanosis, Blalock-Hanlon operation was performed in this patient. The results were good as 54 mmHg changed from 27 mmHg of $PO_2$ in aorta, but sudden cardiac arrest was developed in postoperative 12 hours. In order to confirm the cause of death and the cardiac anomalies, autopsy was performed on the date of death. The diagnosis of the autopsy showed; [1] Transposition of the Great Arteries. [2] Patent Ductus Arteriosus. [3] Patent Foramen Ovale. [4] Ventricular Septal Defect, 2 Muscular Type. [5] Double Ureter, Right. [6] Artificial Atrial Septal Defect. [7] Total Collapse of the left lung and Intraparenchymal hemorrhage of right lung.

• Journal of Chest Surgery
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• v.23 no.6
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• pp.1263-1269
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• 1990

Two patients with uncorrectable cyanotic cardiac anomalies underwent total cavopulmonary shunt[modified Fontan operation]. Case I was a 14 years old male with dyspnea and cyanosis after birth. Aortogram showed TGA combined with overriding of aorta, pulmonary stenosis, complete atrioventricular septal defect, interruption of inferior vena cava, and situs inversus totalis. We had performed total cavopulmonary shunt using with 16 mm Gortex Graft in single atrium to bypass the hepatic vein to pulmonary artery. Postoperatively, patient sustained low PaCO2 and low cardiac output and then expired at 19th postoperative day. The cause of death of the patient would be low cardiac output. Case II was a 6 years old female with dyspnea and cyanosis after birth. Aortogram showed tricuspid atresia[Type IIb], transposition of great arteries, atrial septal defect, ventricular septal defect and pulmonary stenosis, We had performed total cavo-pulmonary shunt using intraatrial baffle[tunnel] with Goretex patch. The postoperative course of this patient was good without event.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1455-1460
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• 1992

Anomalous drainage of the right superior vena cava into the left atrium is a very rare congenital cardiac anomaly. Recently a patient with this venous anomaly was surgically corrected and forms the basis of this report. Patient findings were as follows: The patient has no other symptom but cyanosis which prompted cardiac evaluation Chest PA and electrocariogram were usual. Cross-sectional echocardiogram showed normally connected heart without intracardiac defect, Inferior vena cava drained normally into right atrium and coronary sinus was not dilated. Contrast, given into the right atrium, appeared in the left atrium This rare venous anomaly was confirmed by surgery. Surgical correction consisted of division of superior vena cava above the junction of left atrium and reanastomosis into right atrial appendage. Postoperative digital subtracion angiography confirmed the successful repair. She has doing well for 6months since operation. Systemic venous anomalies without intracardiac defect are very rare. However this anomalies should be considered in the differential diagnosis of cyanosis. The successfully corrected case is reported and literature is reviewed.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.310-315
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• 1983

The combined anomaly of pulmonary stenosis with atrial or ventricular septal defects is usually associated with decreased pulmonary blood flow and right to left shunt, and result in generalized cyanosis. Non-cyanotic pulmonary stenosis patients have generally been considered to have isolated pulmonary stenosis with intact septa. We are going to report a case of pulmonary stenosis with septal defects who have no frank cyanosis at rest because of the predominant intracardiac shunt from left to right. Recently, we managed surgically a case of pulmonary valvular stenosis combined with secundum type atrial septal defect, type II ventricular septal defect, and patent ductus arteriosus. The clinical manifestations of this patient were exertional dyspnea, frequent upper respiratory infection, chest discomfortness and lethargy since late childhood and these had been progressively aggravated. Pulmonary valvular stenosis, atrial septal defect and ventricular septal defect were closed through simply right atriotomy and patent ductus arteriosus through pulmonary arteriotomy. Immediate postoperative course was uneventful and one year follow up is excellent.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.322-330
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• 1983

TAPVC represents 1 to 4% of all congenital cardiac defects. Generally severe and refractory cardiac failure develops in the majority of patients in the early infancy. In a small minority of patient, they are relatively asymptomatic in infancy, but symptoms will develop of necessity In the later life. Our three cases had dyspnea on exertion and cyanosis and had a history of frequent respiratory infections. All 3 cases had the typical showman configuration on roentgenograms of the chest. Current surgical therapy has greatly altered the unfavorable course of these patients after institution of extra-corporeal circulation. We have experienced three cases from June, 1973 to May, 1983, the patients were undergone complete repair with extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery in the Yonsei University. The ages ranged from 12 years to 14 years, all cases were supracardiac type of TAPVC. Only one case died due to bleeding from anastomosis site between LA and pulmonary venous trunk. In the remaining two patients with complete corrections of TAPVC, there was excellent relief of symptoms such as dyspnea and cyanosis. Their postoperative course have been good during follow-up.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.806-811
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• 1985

Pulmonary arteriovenous fistula is a rare congenital vascular malformation in the lung, resulting from erroneous capillary development, with incomplete formation or disintegration of the vascular septa that would normally divide the primitive connection between the venous and arterial plexus. The pathogenesis of its symptom is that unoxygenated and desaturated arterial blood enter into the pulmonary venous system directly. Recently we have experienced a case of multiple pulmonary arteriovenous fistula in a 15 year old male patient, who presented the symptom of cyanosis and dyspnea on exertion. The operation revealed well circumscribed and multilobulated aneurysmal lesion in left lower lobe with its subpleural and posterolateral basal location, and another aneurysmal lesion in inferior lingular segment of left lung. There was no abnormal connection between the fistula and systemic circulation. The left lower lobectomy was performed along with local extirpation of the inferior lingular segment of left lung. Both lesions showed angiomatous dilatation of the various sized vessels embedded in the parenchyma microscopically. Postoperative clinical course disclosed much improvement in symptoms and in the value of blood gas analysis. The patient was discharged without any complication.

• Journal of Chest Surgery
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• v.23 no.3
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• pp.507-513
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• 1990

We experienced an intraatrial baffle repair for unroofed coronary sinus syndrome in TOF. The patient was a 32-month old female, with complaints of cyanosis, exertional dyspnea and growth retardation. Physical examination showed cyanosis of lip, clubbing of finger, growth retardation and systolic murmur[0 /Gr VI] on left sternal border. With an aid of noninvasive and invasive diagnostic procedure, the patient was diagnosed as TOF combined with unroofed coronary sinus syndrome and LSVC connecting to left atrium. After patch closure of VSD, infundibulectomy and pulmonary valvotomy, the intraatrial baffle redirection of anomalous LSVC to right atrium was undertaken. The Dacron baffle was constructed along the roof of the left atrium to the plane of the atrial septum. The intraatrial septum was then reconstructed with Dacron which was sutured to residual septal tissue. After surgery, the systemic oxygen saturation was elevated to 95.5%, as compared with preoperative value 61%. The postoperative course was uneventful.

• Journal of Dental Anesthesia and Pain Medicine
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• v.21 no.5
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• pp.471-474
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• 2021

Methemoglobinemia is rare. It is classified into two types: congenital methemoglobinemia and acquired methemoglobinemia. Methemoglobin is incapable of binding oxygen, leading to complications such as cyanosis, dyspnea, headache, and heart failure. In the present case, a 35-year-old man with congenital methemoglobinemia underwent general anesthesia for thyroidectomy. The patient was diagnosed with hemoglobin M at 7 years of age. Ventilation was performed with FiO₂ 1.0. Arterial blood gas analysis showed that the pH was 7.4, PaO₂ 439 mmHg, PaCO₂ 40.5 mmHg, oxyhemoglobin level of 83.2%, and methemoglobin level of 15.5%. The patient had a stable course, although cyanosis was observed during surgery.

• The Korean Journal of Rehabilitation Nursing
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• v.7 no.2
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• pp.127-139
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• 2004

Purpose: This study was to investigate the degree of body image, self esteem and quality of life, to identify general and disease of characteristics on influencing this factors with the intention of providing basal data for developing nursing intervention to promote body image, self esteem and quality of life. Method: Subjects of this study were 91 grown-up congenital heart patients over 18 years in 2 tertiary hospitals. The data on body image, self esteem and quality of life were collected through questionnaire from March to April 2004. The data were analyzed by descriptive statistics, t-test, Pearson's correlation, ANOVA and tukey test using SAS for Windows 8.1 program. Result: 1) The mean scores of body image, self esteem and quality of life were 63.01, 25.29 and 496.79. 2) Body image was correlated with age(p=.0239), educational level(p=.0182), diagnosis(p=.0066), number of operation(p=.0148), cyanosis(p<.0001), complication(p=.0096) and NYHA level(p=.0378). 3) Self esteem was correlated with education level(p=.0026), economic level(p=.0240), number of operation(p=.0113) and cyanosis (p=.0006). 4) Quality of life was correlated with age(p=.0432) and diagnosis(p=.0020), number of operation (p=.0063), duration of last operation(p=.0225), cyanosis(p<.0001), complication(p=.0090) and NYHA level(p<.0001). 5) There was significantly positive relationship between body image, self esteem and quality of life. Subjects with more positive body image had higher self esteem(r=.7897, p<.05) and subjects with higher self esteem had higher quality of life(r=.6091, p<.05).

• Journal of Chest Surgery
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• v.47 no.4
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• pp.398-401
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• 2014

Severe tricuspid regurgitation resulting from a flail leaflet is a rare cause of neonatal cyanosis. We report a neonate with profound cyanosis and severe tricuspid regurgitation caused by a rupture of the papillary muscle supporting the anterior leaflet, without other structural heart defects. Ductal patency could not be established. The repair of the tricuspid valve was performed after initial stabilization by using extracorporeal membrane oxygenation.