• Clinical and Experimental Pediatrics
• /
• v.46 no.3
• /
• pp.308-311
• /
• 2003

Congenital chloride diarrhea is a serious autosomal recessive disease, and defect of intestinal electrolyte absorption that involves, specifically, $Cl^-/{HCO_3}^-$ exchange in the distal part of the ileum and colon. The clinical feature is dominated by profuse, watery diarrhea containing high concentrations of chloride(>90 mmol/L) and sodium. The chloride loss results in severe dehydration with a hypochloremic alkalosis. The molecular pathology involves an epithelial $Cl^-/{HCO_3}^-$ exchanger protein. Mucosal ion transport is affected to differing degrees and the severity of the disease may thus vary. Recently, a gene defect on chromosome 7 has been identified. However, there was a deficit in replacement of fluid and electrolyte, abdominal distension remained and the character of stools was watery. We report a case of congenital chloride diarrhea in a premature female who presented with watery diarrhea containing high concentrations of chloride and abdominal distension.

• Journal of Chest Surgery
• /
• v.32 no.6
• /
• pp.584-587
• /
• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Journal of Chest Surgery
• /
• v.16 no.3
• /
• pp.331-336
• /
• 1983

Cor triatriatum is a rare congenital malformation of the heart, in which a septum stretches in a transverse or oblique plane through the left atrium, thus separating it into two compartments. The upper one connects with the pulmonary veins, and the lower one connects with the left ventricle. Due to the rarity of, and great difficulty in-diagnosing, cor trlatrlatum, data On the surgery of this disease are of necessity very limited and so accurate pre-operative diagnosis was very difficult to make. `We experienced a case of the cyanotic congenital heart disease which was diagnosed as a large atrlal septal defect with streaming venous blood from inferior vena cava to left atrium through atrial septal defect in August, 1982. We found that there were transverse septum in the left atrium through atrlal septal defect, the pulmonary venous drainage were located in the upper chamber of the left atrium, and the lower chamber was connected with the left atrial appendage, mitral valve and `left ventricle. But our case had not any opening in this transverse septum and the right atrium was connected with the upper chamber of the left atrium through the upper part of the atrlal septal defect, and was communicated with the lower chamber of the left atrium through the lower part of the atrlal septal defect. We excised the transverse septum and repair this atrial septal defect with Woven Dacron patch accompanying with the drainage of coronary sinus to right atrium. The post-operative course was not eventful and he was discharged with good result on the post-operative 8th day, and has been in good .condition up to now for longer than 10 months.

• Journal of Physiology & Pathology in Korean Medicine
• /
• v.21 no.2
• /
• pp.561-566
• /
• 2007

After researching on infantile diseases in Hyungsang medicine, the writer got the conclusions as follows. The infants who are excess of the Yang energy need to nourish the Eum- blood. The main causes of the infantile disorders are congenital defect and malfunction of internal organs by nature, as results of these they suffer from mental disorders or being undergrown. And after birth they get ill from internal injury or external affections, mainly epilepsy by retention of undigested food, fever, cough, asthma, nasal obstruction, dermatopathia, and affection by cold, etc. In Hyungsang medicine Dam-body is apt to get ill from deficiency of Eum-blood and bangkwang-body from deficiency of Yang-energy. And infants are hare to be moderate in food, so they become to diseases of the Spleen and stomach, especially infants with Yangmyung type get to epilepsy, cough, skin disorders, and obese for the reasonof overeating. Among main infantile symptoms congenital defects, infantile mental disorders, and convulsive diseases come from congenital defect and malfunciton of internal organs, so it must be treated the symptoms following the reasons. Above all infantile mental disorders are treated not to separate the spirit from the body. And fever, cough and asthma, affection by cold, skin diseases, poor appetite, and obese come from deficiency of Kidney or the deficiency and excess of the Spleen and stomach. In order to prevent from infantile diseases right antenatal training, taking medicine rightly, exercise and eating good habits are needed to give guidance. Seeing through the clinical cases in Hyungsang medicine, we come to know that the infantile mental disorders come out primarily for the reasons of the congenital defect, and the infantile epilepsy come from malfunction of internal organs, and the nasal obstruction and skin diseases come from deficiency of Kidney or the deficiency and excess of the Spleen and stomach.

• Korean Journal of Veterinary Research
• /
• v.56 no.3
• /
• pp.193-195
• /
• 2016

The purpose of this report is to introduce persistent left cranial vena cava (PLCVC) with persistent right aortic arch (PRAA) and patent ductus arteriosus (PDA). Case 1 was a Cocker Spaniel with PRAA and case 2 was a Maltese with PDA. PLCVC was enclosed at the sites of PRAA and PDA surgery; therefore, it was lifted dorsally during PDA and PRAA surgery. Surgery to repair congenital heart defects including PRAA and PDA is recommended for dogs that do not die of PLCVC at a young age.

• Journal of Chest Surgery
• /
• v.54 no.3
• /
• pp.224-227
• /
• 2021

Here, we report the rare case of a 13-year-old girl with a congenital diaphragmatic hernia (also known as Bochdalek hernia), which was revealed to be an extralobar pulmonary sequestration that was treated using laparoscopic and video-assisted thoracic surgery sequestrectomy and repair of the diaphragm defect after detection of a supradiaphragmatic mass connected with the retroperitoneum. The patient showed no postoperative complications at a 1-month follow-up examination.

• Korean Journal of Radiology
• /
• v.22 no.11
• /
• pp.1894-1908
• /
• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• Journal of Chest Surgery
• /
• v.11 no.2
• /
• pp.213-226
• /
• 1978

One hundred cases of open heart surgery were done at this Department in 1977. There were 65 congenital anomaly and 35 acquired diseases. Out of 65 cases of congenital malformation 35 acyanotic and 30 cyanotic cases were found. Fifteen cases of ventricular septal defect and 29 tetralogy of Fallot were noted eight patients expired out of 65 congenital anomaly [12.3%] , 4 out of 35 acyanotic [11. 4%] and 4 among 30 cyanotic anomaly[13.3%]. Among 35 cases of acquired heart disease 3 atrial myxoma [2 left and one right] and 32 valvular lesions were noted. In two cases open mitral commissurotomy, and in 30 valve replacement were done. Twenty-two single valve and 8 double valve replacement were done. Seven patients expired out of 30 patients [23.3%]. Among 22 single valve replacement cases 2 and among 8 double valve 5 died. ~ In eighteen mitral valve replacement cases 2 deaths occurred. One mitral insufficiency patient who expired suffered from severe pulmonary` hypertension [PA=120/67mmHg], tricuspid insufficiency and a large ventricular septal defect. The patient underwent mitral valve replacement, tricuspid annuloplasty and patch closure of ventricular septal defect. Over all mortality rate for 100 open heart surgery cases was 15%. Since 1977 open heart surgery cases were done routinely in this institution and cases are increasing rapidly. With present rapid improvement of economical status and introduction of medical insurance system, open heart surgery will be firmly established in Korea in the very near future.

• Journal of Chest Surgery
• /
• v.12 no.1
• /
• pp.33-42
• /
• 1979

The present study reports 41 cases of congenital and acquired heart diseases, who received open heart surgery under extracorporeal circulation [ECC] by Sarns Heart-Lung-Machine [HLM] at the Department of Thoracic and Cardiovascular Surgery, Hanyang University Hospital during the` period between July 1975 and February 1979. The priming of pump oxygenator was carried out by the hemodilution method using Hartman`s solution, whole blood, and fresh human plasma. The rate of hemodilution was in the average of 50.8 ml/kg. ECC was performed at the average perfusion flow rate of 85.0 ml/kg/min [2.43 L./ kg/2] and at moderate hypothermia. In the total cardiopulmonary bypass, arterial pressure ranged between 55 mmHg and 90 mmHg, but generally maintaining over 70 mmHg. Patient age ranged between 2 and 54 year old, in congenital heart diseases, between 2 and 28, in acquired heart diseases, between 17 and 54 Sex ratio of male to female was 20:21. The cases include a case of pulmonary valvular stenosis, 4 cases of atrial septal defect, 9 cases of ventricular septal defect, 9 cases of tetralogy of Fallot, 5 cases of pentalogy of Fallot, 3 cases of atypical multiple anomalies 7 cases of mitral stenosis or insufficiency, a case of myxoma in left atrium, and a case of ruptured aneurysm of Valsalva`s sinus. The surgical managements were 16 valvulotomy for pulmonary valvular stenosis, 2 Teflon patch graft closure and 5 simple suture closure of atrial septal defect, 16 Teflon patch graft closure and 5 simple suture closure of ventricular septal defect, 12 pericardial patch graft for infundibular stenosis of right ventricle, one anastomosis between left superior vena cava and right atrium, 2 open mitral commissurotomy, 5 mitral valve replacement using Starr-Edward`s ball valve, porcine xenograft by Hancock, by Carpentier-Edward, or Angell-Shiley, one removal of left atrial myxoma, and a repair of ruptured aneurysm of Valsalva`s sinus. Four [9.7%] out 41 cases expired postoperatively and the rest of 37 cases survived with satisfactory results. The causes of death were one coronary embolism in tetralogy of Fallot, 2 postoperative lower cardiac output in atypical multiple anomalies, and one right heart failure in large: ventricular septal defect with pulmonary hypertension.

• Journal of Chest Surgery
• /
• v.24 no.2
• /
• pp.206-211
• /
• 1991

Interruption of the aortic arch may be defined as discontinuity of the aortic arch in which either an aortic vessel or a patent ductus arteriosus supplies the descending aorta. This anomaly is a rare congenital malformation that usually occurs with severe associated intracardiac congenital anomalies, such as ventricular septal defect, patent foramen ovale and abnormal arrangement of the brachiocephalic arteries. Rarely, transposition of the great vessel, truncus arteriosus are coexistent. We experienced a case of the interrupted aortic arch [Type A] associated with VSD, PDA and patent foramen ovale in a 16 years old female. One stage total correction was done under profound hypothermia with total circulatory arrest. Aortic continuity was established using patent ductus arteriosus with anterior wall of main pulmonary artery, which was anastomosed obliquely to anteromedial side of the ascending aorta. Ventricular septal defect was closed using Dacron patch and patent foramen ovale was closed directly. Postoperative course was uneventful, except mild hoarseness.