• Journal of Chest Surgery
• /
• v.10 no.2
• /
• pp.299-314
• /
• 1977

Clinical experience on 16 cases of open heart surgery under the extracorporeal circulation with mild or moderate hypothermia and partial hemodilution technique at the National Medical Center during the period from June 1976 to October 1977. Nine of sixteen were congenital heart disease and seven were acquired heart disease. The age of the patient ranged between 6 and 48 years. The body weight varied from 18.5kg to 60kg and body surface area 0. 79-1.70m2. The average priming volume of pump oxygenator was 2080 ml, which was consisted fresh ACD blood, buffered Hartmann`s solution, Mannitol, 50% dextrose in water and Vit. C. The average hemodilution rate was 27%. The average flow 2.3 L/min/m2 or 80 ml/min and the duration of perfusion varied from 31 min to 270 min with average of 107 min. The perfusion was carried out under the mild or moderate hypothermia using core cooling alone in 10 cases, core cooling and local myocardial cooling with $0-4^{\circ}C$ physiologic saline in 2 cases. From a hemodynamic point of view, the blood pressure dropped down around 80 mmHg after the initiation of perfusion follwed by increase to safety level and stable during the perfusion. The central venous pressure remained within normal limits. In most cases, hemoglobin and hematocrit decreased during and after the perfusion. Hemogiobin level was decreased, average of 20.6 %, hematocrit 18.6%, pletelets 55% postoperatively. Plasma hemoglobin increased moderately, from preperfusion average valve of 7.79 mg % to post-perfusion value of 54.7 mg %. Electrolytes changes during cardiopulmonary bypass showed definite hypokalemia but changes of Na, Ca were not definite. Arterial blood gas analysis during cardiopulmonary bypass suggested that the metabolic acidosis which was accompanied by respiratory alkalosis which was corrected postoperatively. As the opera tive complication, transient hemoglobinuria in 4 cases and neurological signs in 2 cases were all cured. There were 2 death cases and operative mortality rate was 12.5%.

• Clinical and Experimental Pediatrics
• /
• v.58 no.10
• /
• pp.392-397
• /
• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of Chest Surgery
• /
• v.14 no.3
• /
• pp.260-267
• /
• 1981

Left atrial myxoma is a rare disease and its recurrence is reported to be always possible whenever primary excision is incomplete. Cardiac Myxoma is rare disease of the heart, and it has a little chance of recurrence. We experienced a patient of recurrent left atrial myxoma who was 31 years old, had been gotten excision of Lt. atrial myxoma when she was 28 years of her age. She was gotten mitral valve replacement simultaneously during 2nd operation with difficulty. So we report this case with the review of the literatures. [KTCS 1981;3:260-267]Surgical Treatment of Acute Pyogenic Pericarditis followed the Sepsis Ki Woo Shin, M.D.,* Ho Wan Lee, M.D.* and Dong Jun Lee, M.D.* Two cases of acute pyogenic pericarditis are, one case, 12-year-old male patient, followed the bacteremia of pneumonia and other case, 9 year old female patient, followed the bacteremia of osteomyelitis. After the confirmed diagnosis by pericardial aspiration, the emergency pericardial window was made to relief the severe cardiogenic symptoms. The general symptoms were improved immediately, but 40 days and 15 days after pericardiostomy, in each case, the sign and symptoms of cardiac compression were seen with recurrent cardiac tamponade. Pericardiectomy with median sternotomy was performed in each case and thereafter the patients were discharged without any problems.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.14 no.2
• /
• pp.156-162
• /
• 2014

A 25-month-old boy was referred to the hospital due to large head detected on routine physical examination. At visit, dysmorphic facial appearances, including broad nose, prominent forehead, and coarse face, were noted. Nasal obstruction with nasal voice, prominent adenoids, and bilateral middle ear effusions were detected. His abdomen was distended, and liver and spleen were palpated about 3 finger and 2 finger breadths, respectively. He was operated for bilateral inguinal hernias. The motion of both elbow joints was mildly limited on supination and pronation. Urinary level of glycosaminoglycan was elevated and the enzyme activity of iduronate sulfatase in leukocytes was decreased. The mutational analysis of the gene iduronate 2-sulfatase (IDS) revealed c.263G>A (p.Arg88His) mutation. His developmental scale showed delayed development and there was cardiac valvular involvement (tricuspid regurgitation and mitral valve prolapse). After the diagnosis of Hunter syndrome, enzyme replacement therapy started on a weekly basis without progression of any clinical features. Here we report a case of early diagnosed Hunter syndrome detected by large head on routine examination. Thus, it is important to associate Hunter syndrome in the patient with large head especially, if there is the history of bilateral inguinal hernia and prominent adenoids to increase the possibility of early diagnosis and treatment.

• Journal of Chest Surgery
• /
• v.55 no.3
• /
• pp.233-238
• /
• 2022

Background: This study investigated the distribution of valve incompetence in patients with chronic venous disease (CVD) and its correlation with the clinical category of the clinical, etiological, anatomical, and pathophysiological (CEAP) classification. Methods: In total, 1,386 limbs with clinically suspected CVD were categorized according to the CEAP classification and consecutively underwent duplex ultrasonography between April 2017 and December 2020. Results: There were 362 limbs in male patients and 1,024 limbs in female patients. The limbs were classified as C0s-C1 (608 limbs, 43.8%), C2 (727 limbs, 52.5%), or C3-C6 (51 limbs, 3.7%). The prevalence of saphenous vein incompetence in CEAP C0s-C1 limbs was 43.6%. The saphenofemoral junction (SFJ) was competent in 37% of CEAP C2-C6 limbs. The CEAP C3-C6 category was not correlated with reflux patterns of the saphenous vein system (Cramer's V=0.07), incompetent SFJ (Cramer's V=0.07), deep vein reflux (Cramer's V=0.03), or the distribution of incompetent segments in the great saphenous vein (GSV) (Cramer's V=0.11). Conclusion: Duplex ultrasonography is necessary to formulate a proper treatment plan for limbs categorized as CEAP C0s-C1. The SFJ was competent in more than one-third of CEAP C2-C6 limbs with GSV reflux; as such, flush ligation of the GSV may be unnecessary in these patients. The CEAP C3-C6 category showed no correlations with reflux patterns of the saphenous vein system, SFJ reflux, deep vein reflux, or the distribution of incompetent segments in the GSV.

• Journal of Yeungnam Medical Science
• /
• v.40 no.2
• /
• pp.187-192
• /
• 2023

Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

• Korean Journal of Veterinary Research
• /
• v.43 no.2
• /
• pp.307-310
• /
• 2003

An atrial septal defect (ASD) is congenital heart disease with a communication between the atria, which allows blood to shoot from the atrium with pressure. A 3-month-old female Miniature Schunauzer was referred to the Veterinary Teaching Hospital of Konkuk University for the evaluation of systolic heart murmur. At presentation, the mucous membrane was cyanotic. On physical examination, an ejection-type systolic murmur was auscultated at the pulmonic area. In addition, thoracic radiography showed enlargement of main pulmonary artery and right atrial/ventricular enlargement. Echocardiography revealed dilated right atrium and atrial septal defect. However, mitral and tricuspid valve were still intact and well tolerating. The presence of an ASD was confirmed by identifying flow across the defect with color Doppler imaging. Doppler echocardiography provides a means of non-invasive documentation and quantification of ASD. Complete blood count and serum chemistry were not remarkable. Although large defect was confirmed between the two atrium, the patient did not show any obvious clinical signs of heart failure at this time.

• Journal of Chest Surgery
• /
• v.19 no.2
• /
• pp.280-287
• /
• 1986

Between March 1978 and August 1985, 29 cases at various congenital heart diseases were reoperated because of remnant shunt of residual anomalies at Seoul National University Hospital. They were consisted of 10 cases of Tetralogy, 4 simple VSD, 6 complicated VSD. 3 partial ECD, and 5 other rare congenital anomalies. The interval between the initial and the second procedure ranged from 1 day to 122 months [mean; 26.9 months]. In 4 cases of them, the second procedure was done during initial hospitalization within 3 weeks post-operatively. The primary operation intended to be corrective surgery except four whose primary operation was palliative or exploratory one even though it was done with extracorporeal circulation. The indication for second operation was mainly residual shunt or valvular obstruction due to patch detachment or inadequate relief of stenotic lesion. Others were paravalvular leak, valvuloplasty failure, prosthetic valve failure, and inadequate primary diagnosis. Four patients were dead [14.3%]; three complicated VSD`s and one Tetralogy. There were 7 cases of nonfatal complication with subsequent improvement except one [diffuse cerebral dysfunction].

• Journal of Chest Surgery
• /
• v.36 no.10
• /
• pp.759-765
• /
• 2003

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

• Journal of Chest Surgery
• /
• v.18 no.2
• /
• pp.293-298
• /
• 1985

Cor triatriatum is a rare congenital malformation of the heart in which a septum stretches in a transverse plane through the left atrium, thus creates two left atrial subchambers. The upper one connects with the pulmonary veins, and the lower connects with the left ventricles. Due to the rarity of, and difficulty in diagnosing car triatriatum, data on the surgery of the disease are of necessity and very limited. A case of cor triatriatum combined with atrial septal defect and persistent left superior vena cava was experienced in November, 1984 in Chonnam University Medical School. There was a transverse septum in the left atrium below atrial septal defect, all pulmonary veins were drained into the upper chamber of the left atrium which connected with the right atrium via atrial septal defect and the lower chamber via an oval opening[8mm] in the abnormal septum and the lower chamber was connected with the left atrial appendage, and the left ventricle via mitral valve. There was persistent left superior vena cava drained to left atrium and coronary sinus. The abnormal transverse septum within the left atrium was completely excised and the atrial septal defect was repaired with Woven Dacron patch. The post-operative course was not eventful and the patient was discharged to home with good result on the 15th postoperative day, and has been in good condition upto now.