• Journal of Chest Surgery
• /
• v.37 no.4
• /
• pp.364-368
• /
• 2004

Congenital pulmonary vein stenosis is a rare anomaly and related to high mortality due to progressive pulmonary hypertension and heart failure in infancy. Aggressive anti-failure medication and surgical treatment is recommended. Surgical options are balloon dilatation, endovascular stent, pneumonectomy, lung transplantation, patch grafting, and sutureless repair. We report a case of congenital pulmonary vein stenosis with normal anatomical connection successfully treated with sutureless technique and using pulmonary vasodilators, such as Sildenafil, lloprost and iNO postoperatively.

• Journal of Chest Surgery
• /
• v.10 no.1
• /
• pp.143-147
• /
• 1977

Atrial septal defect is the most common of the congenital heart disease in the adult. Since the first description of atrial septal defect by Rokitansky in 1857, this anomaly has been studied by many workers in past one century. In 1953, Lewis had first corrected the atrial septal defect under direct vision with deep hypothermia, and Gibbon [1954] had first done the atrial septal defect under direct vision with extracorporeal circulation. In our college [May 2’ 1977], we have first repaired the A.S.D. under direct vision with artificial heart-lung machine and, the defect was 4x5cm in size which was closed by Dacron patch. This patient was 12 year old girl who is well now. [postoperative 13 days]

• Journal of Chest Surgery
• /
• v.22 no.6
• /
• pp.990-995
• /
• 1989

Since 1984, 24 patients underwent repair of atrioventricular septal defect. Nineteen had a partial defect and 5 had a complete atrioventricular septal defect. There were 9 men and 15 women, ranging in age from 1 to 50 years [mean age, 13.3 years]. Four patients had a Downs syndrome. Additional congenital heart defects were present in 11 patients. One patient had palliative operation prior to total correction. In partial defects, the primum atrial septal defect was closed with Xenomedica patch and the mitral valve was repaired with simple closure of the septal commissure. Central incompetence from annular dilatation was repaired by a local annuloplasty. In complete defect, the septal defects were closed with two patches except one. Operative mortality was 5% in partial defects and 60% in complete defects and low cardiac output was the commonest etiology. In a mean follow-up period of 27.9 months [range, 4 to 63 months] there were no late death and no instances of late-onset complete heart block. One patient required reoperation [MVR] for residual mitral regurgitation. The majority of patients were asymptomatic and mean postop. NYHA functional class was 1.2.

• Journal of Chest Surgery
• /
• v.16 no.3
• /
• pp.289-294
• /
• 1983

The report is concerned to our experience of 12 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Chosun University Hospital during the period between Nov, 1979 and April, 1983. 1. There were 4 cases of congenital anomaly and 8 cases of acquired heart disease. 2. There were 6 male and 6 female patients with a mean age of 20 years. [range 9 to 33 years]. 3. The cases induced 2 ventricular septal defect, 2 atrial septal defects and 8 acquired valvular heart diseases. 4. The surgical managements were 2 primary repair for atrial septal defect and 2 patch closure for ventricular septal defect, 1 triple valve replacement [AVR MVR TVR], 1 aortic valve replacement, 4 double valve replacement [AVR MVR] and 2 open mitral commissurotomy for pure mitral stenosis. 5. The average cardiopulmonary bypass time was 61.5 minutes for congenital heart disease and 201.4 minutes for acquired valvular heart disease and the average aortic cross clamping time was 36.75 minutes for the former and 165.6 minutes for the latter. 6. Postoperatively, there were 1 Alopecia, 1 Electric burn and 1 wound infection as complication. 7. Overall operative mortality was 8.3%. 7. All patients received valve replacement were recommended anticoagulation with persantin.

• Journal of Chest Surgery
• /
• v.19 no.1
• /
• pp.116-121
• /
• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.22 no.2
• /
• pp.53-57
• /
• 2022

Congenital metabolic disorders are rare inherited disorders resulting from a defect in biochemical and metabolic pathways affecting proteins, fats, carbohydrates metabolism or impaired organelle function. Depending on the abnormality of biochemical metabolism, various precursors and their abnormal metabolites can accumulate in the body and the final products which are critical in normal physiology can be deficient, resulting in disease. Congenital metabolic disorders present complicated medical conditions involving several human organ systems, including nervous system, eyes, liver, and kidneys. Various proteins and lipids are involved in the development and homeostasis of the skin, so many congenital metabolic disorders present abnormal changes in skin and hair. In this review, congenital metabolic diseases related to amino acid and lipid metabolism accompanying skin abnormalities will be discussed.

• Journal of Chest Surgery
• /
• v.27 no.7
• /
• pp.624-627
• /
• 1994

Congenital coronary arteriovenous fistula is a rare cardiac defect that causes coronary arterial flow to drain into the right cardiac chambers, the pulmonary artery, the coronary sinus, or the left cardiac chambers. The most frequently involved vessel is the right coronary artery. We experienced a case that had a coronary arteriovenous fistula associated with valvular heart disease. With the cardiopulmonary bypass done under hypothermia, mitral valve replacement was accomplished and the fistulas of both proximal and distal portions of the right coronary artery were closed with 3-0 prolene. Postoperative course was uneventful.

• Journal of Chest Surgery
• /
• v.28 no.6
• /
• pp.640-643
• /
• 1995

Bochdalek hernia is the congenital posterolateral defect in the diaphragm caused by a failure of the pleuroperitoneal canal to close at 8 weeks, gestation. Infants with hernia diagnosed at birth have poor prognosis. Survival rate depends on pulmonary growth and development, preoperative stability and postoperative care. We experienced two cases of Bochdalek hernia, one in right was repaired with patch closure using sheet and the other in left was repaired with simple closure.

• Journal of Chest Surgery
• /
• v.19 no.4
• /
• pp.672-677
• /
• 1986

From April, 1984 to August, 1986, 214 cases of cardiovascular surgeries had been performed at Yeungnam University Hospital consisting 158 open heart surgeries and 56 non-open heart surgeries. The leading cardiac anomaly of open heart surgeries was ventricular septal defect which was 43% of congenital heart diseases, and most of remaining non-open heart surgeries were ligating patent ductus arteriosus. We had observed 33 postoperative complications such as wound problems, transient arrhythmia, postpericardiotomy syndrome, bleeding requiring reoperation and so on. 3 cases of surgical mortality were present [2 in congenital heart diseases and 1 in acquired heart disease], which resulting 1.9% of surgical mortality rate in the open heart surgeries.

• Journal of Chest Surgery
• /
• v.24 no.5
• /
• pp.475-479
• /
• 1991

Tetralogy of Fallot associated with atrioventricular canal defect is rare congenital anomaly. Because of complexity of the surgical corrections of two associated anomalies, the mortality of surgery has been high. We have experienced a case of the tetralogy of Fallot with atrioventricular canal defect in a 9-year-old boy of Down`s syndrome, and the anomalies were totally corrected with good result. Single Dacron patch was placed to close the ventricular septal defect and the pericardial patch for atrial septal defect. The right ventricular outflow tract was widened by infundibulectomy and pulmonary valvulotomy followed by Goretex patch in right ventricular outflow tract.