• Journal of Chest Surgery
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• v.18 no.4
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• pp.574-581
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• 1985

Surgical treatment for PDA has been pivotal in historical development of surgery for congenital heart disease. A clinical study on 36 cases of operated PDA were performed during period from Aug. 1981 to Jul. 1985 at the Department of Thoracic & Cardiovascular Surgery in Chonbuk University. The following results are obtained. 1. The 8 males and 28 females ranged in age from 2 yrs, to 24 yrs, [mean 11 yrs.] 2. Chief complaints of the patients were dyspnea on exertion in 61%, palpitation in 39%, frequent URI in 12%, and no subjective symptoms in 11%. 3. On auscultation, continuous machinery murmur heard in 94% and systolic in 14%. 4. Radiologic findings of chest P-A showed increased density of pulmonary vascularity in 94%, cardiomegaly in 69%, and within normal limits in 5% of the patients. 5. EKG findings of the patients revealed LVH in 69%, RVH in 6%, BVH in 6%, and within normal limits in 17%. 6. Of the 36 patients, cardiac catheterization was performed in 34 patients. The results showed mean Qp/Qs = 2.25, mean Pp/Ps=0.42, and mean systolic pulmonary arterial pressure=53mmHg. 7. Surgical methods were as followed: The 32 case of ductal ligation and one case of division & suture technique for PDA through the left posterolateral thoracotomy were done. And 2 cases of ductal ligation one suture closure through the pulmonary artery were performed under the cardiopulmonary bypass. 8. Intraoperative complication was ductal rupture with division 8< suture for PDA and transient hoarseness in 1, recanalization in 1, and urethral stricture in 1 case postoperatively. 9. One patient died due to ductal rupture intraoperatively and operative mortality was 2.8%.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.86-90
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• 2014

Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductase deficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiple malformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient with SLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second and third toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7 gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a review of the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This report describes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient with SLOS.

• Clinical and Experimental Pediatrics
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• v.65 no.4
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• pp.188-193
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• 2022

Background: Respiratory distress syndrome (RDS) is a common cause of hospitalization and death in preterm infants who require surfactant treatment and respiratory support. Purpose: This study aimed to compare the clinical outcomes of minimally invasive surfactant therapy (MIST) and the INtubation, SURfactant administration, and Extubation (INSURE) technique in preterm infants with RDS. Methods: In this clinical trial, 112 preterm infants born at 28-36 weeks of gestation and diagnosed with RDS randomly received 200-mg/kg surfactant by MIST or the INSURE method. In the MIST group, surfactant was administered using a thin catheter (5F feeding tube); in the INSURE group, surfactant was administered after intubation using a feeding tube and the tracheal tube was removed after positive pressure ventilation was started. Nasal continuous positive airway pressure was applied in both groups for respiratory support and the postprocedure clinical outcomes were compared. Results: The mean hospitalization time was shorter for infants in the MIST group than for those in the INSURE group (9.19±1.72 days vs. 10.21±2.15 days, P=0.006). Patent ductus arteriosus was less frequent in the MIST group (14.3% vs. 30.4%, P=0.041). Desaturation during surfactant administration occurred less commonly in the MIST group (19.6% vs. 39.3%, P=0.023). There were no significant intergroup differences in other early or late complications. Conclusion: These results suggest that surfactant administration using MIST could be a good replacement for INSURE in preterm infants with RDS since its use reduced the hospitalization time and the number of side effects.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.48-54
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• 2021

Genetic imbalances are a major cause of congenital and developmental abnormalities. We report the first case of a 3p26 microdeletion and 5q35.2q35.3 microduplication in a newborn with multiple congenital anomalies evaluated using chromosomal microarray analysis (CMA) and fluorescence in situ hybridization (FISH). The patient was born at 30 weeks and 2 days of gestation with a body weight of 890 g. He had symmetric intrauterine growth restriction, microcephaly, facial dysmorphism (hypertelorism, blepharophimosis, mild low-set ears, high-arched palate, and micrognathia), and right thumb polydactyly. Echocardiography revealed an atrial septal defect and patent ductus arteriosus. Furthermore, CMA revealed a concurrent microdeletion in 3p26 and a microduplication in 5q35.2q35.3. FISH analysis showed that these genetic changes resulted from a translocation mutation between chromosomes 3 and 5. The patient's mother had mild intellectual disability, short stature, and facial dysmorphism, while his father had a normal phenotype. However, parental FISH analysis revealed that the asymptomatic father carried a balanced translocation of chromosomes 3p26 and 5q35. CMA and FISH tests are useful for diagnosing neonates with multiple congenital abnormalities. Further parental genetic investigation and proper genetic counseling are necessary in cases of chromosomal abnormalities inherited from parental balanced translocations.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.959-965
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• 2006

Purpose : The purpose of this study was to investigate the effect of prophylactic indomethacin on reduction of patent ductus arteriosus(PDA) and intraventricular hemorrhage(IVH) in extremely low birth weight infants(ELBWI). Methods : Retrospective review of 84 ELBWI who were admitted to our neonatal intensive care unit from June 2004 to April 2006 was performed. Patients were divided into prophylactic group(n=28) and control group(n=56), where prophylactic indomethacin were given within 6 hours after birth. Clinical outcomes were compared between these groups. Results : There were no significant differences in gestational age, birth weight, incidence of hemodynamically significant PDA and severe IVH, and mortality between prophylactic group and control group. However, there were more frequent indications for therapeutic indomethacin, higher incidence of intestinal perforation, and longer time to achieve full enteral feeding in prophylactic group than control group. The incidence of other adverse events attributed to indomethacin prophylaxis did not differ between two groups. Conclusions : Prophylactic indomethacin may not prevent hemodynamically significant PDA and severe IVH in ELBWI. On the contrary, it may be associated with increased risk of adverse events. Further efforts should be investigated to decrease PDA and severe IVH in ELBWI.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.434-440
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• 2009

Background: Surgical closure of a patent ductus arteriosus (PDA) can be considered when conservative medical treatment is ineffective or contraindicated. Low weight and earlier gestational age neonates who are treated with conservative medical therapy generally showed a higher failure rate. The morbidity of surgical PDA closure in such extremely low birth weight (ELBW) neonates is also high. Here we present the early results of a new technique for approaching the PDA through a dorsal minithoracotomy. Material and Method: From March 2006 to November 2008, 24 premature neonates underwent surgical PDA closure. The procedures were performed in the newborn intensive care unit via a 2 cm long dorsal minithoracotomy with the baby in the prone position with the left hemithorax elevated 30$^{\circ}$. Bimanual cotton swab blunt dissection completed the extrapleural accesstothe PDA and then two clips were applied. Tube thoracostomy was avoided if there was no meaningful pleural laceration. Result: The infants mean gestational age was 26.5$\pm$2.1 weeks (range: 23 to 30 weeks) and the average age at operation was 11$\pm$11 days. The mean body weight at operation was 933$\pm$271 grams (range: 570 to 1,700 grams). Eight patients expired, but there was no procedure-related death. Postoperative echocardiography revealed two cases of residual shunt but none of these shunts were detected on the follow up echocardiogram that was performed on the post operative 5 and 59 days. Conclusion: We concluded that the technique described here is an effective procedure in view of the satisfactory operative exposure and the low rate of complications.

• Journal of Chest Surgery
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• v.33 no.10
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• pp.785-791
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• 2000

Left ventricular dysfuction is common in immediate postoperative periods after surgical correction of heart diseases with chronic left ventricular volume overload. We speculated postoperative changes of left ventricular volume and unction in patients with patent ductus osus(PDA) who had underwent surgical repair at ages older than 16 years. Factors influencing postoperative left ventricular volume and function were also analyzed. Material and Method: From August 1989 to August 1999 thirty-siz adult patients with PDA 28 females and 8 males. were enrolled in this study. Their age ranged from 16 years to 57 years(mean :32 years). Types of surgical repair were division with primary closure in 22, division with patch closure in 6, internal obliteration using cardiopulmonary bypass in 4 and ligation in 4. Aortic clamping was combined during surgical repair in 22(61%) and cardiopulmonary bypass was used in 8(22%) Two-dimensional echocardiography studies were performed in 34(94%) preoperatively and in 25(66%) immediate postoperatively to assess postoperative changes of left ventricular internal dimensions. left ventricular volume and ejection fraction. Duration of postoperative follow-up ranged from 1 month to 99 months (mean:22 months) and 10 patients underwent 16 echocardiographic evaluation during this period Result : Preoperative and postoperative left ventricular systolic dimensions(LVIDs) were 42$\pm$8.0mm and 42$\pm$8.3mm left ventricular diastolic dimensions(LVIDd) were 64$\pm$10.0mm and 56$\pm$7.4mm left ventricular end systolic volumes(LVESV) were 62$\pm$19cc (z=1.87$\pm$0.06) and 59$\pm$24cc(z=1.78$\pm$0.08) left ventricular end diastolic volumes(LVEDL) were 169$\pm$40cc(z-1.17$\pm$0.1) and 112$\pm$29cc(z=0.85$\pm$0.1) and ejection fractions(EF) were 66$\pm$6.7% and 48$\pm$12.6% respectively. There were statistically significant differences between preoperative and postoperative values in LVDIDd(p=0.001) LVEDV(p=0.001) and EF(p=0.0001) while no significant difference is LVIDs and LVESV. Postoperative depression of ejcection fraction was significantly related with z-score of preoperative LVESV and LVEDV by univariateanalysis while LVEDV only was significant risk factor for postoperative LV dysfunction by multiple regressioin analysis ($\Delta$LVEF=-13.3-4.62$\times$LVEDV(z), p=0.001) During the follow-up periods ejection fractions become normalized in all except one patients. Conclusion ; Left ventricular function is usually deteriorated after the surgical correction of PDA in adult age and preoperative LVEDV is a major determinant of postoperative LV function.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.392-398
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• 2011

Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.430-438
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• 2002

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. We reviewed our recent surgical experiences on congenital heart defect (other than patent ductus arteriosus) in low birth weight babies. Material and Method: From September 1994 to February 2001, 31 consecutive infants weighing 2500 g or less underwent cardiac surgery with (OHS group n=12) or without cardiopulmonary bypass (CHS group n=19). A retrospective study was carried out to evaluate short-and intermediate-term outcome. Mean gestational age and age at operation were 36.9 weeks(range, 32.3-42weeks) and 32.1days (range, 0-87days) respectively. Mean body weight at birth and operation were 1972g (range, 1100-2500g) and 2105g (range, 1450-2500 g) respectively. There was no difference between the two groups in age and body weight. Defects included ventricular septal defect (VSD) (n=3), VSD with arch anomaly (n=2), total anomalous pulmonary venous return (n=2), transposition of the great arteries (TGA) (n=2), truncus arteriosus (n=2), and univentricular heart with cor triatriatum (n=1) in OHS group, and coarctation of aorta (n=7), tetralogy of Fallot (TOF) (n=3), TOF with pulmonary atresia (n=3), multiple muscular VSDs (n=1), double outlet right ventricle (n=1), pulmonary atresia with intact ventricular septum (n=2), tricuspid atresia (n=1), and TGA with multiple VSD (n=1) in CHS group. 13 patients (41.9%) were intubated pre-operatively. Result: There were 4 early deaths(＜30 days); 1 (8.3%) in OHS group and 3 (15.8%) in non-OHS group. All these early deaths were related to the pulmonary artery banding(PAB). There was no operative mortality in infants undergoing complete repair and palliative operations other than PAB. Delayed sternal closure was required in 3 patients. Prolonged postoperative mechanical ventilation (>7 days) was required in 7 patients(58.3%) in OHS and 7(38.8%) in CHS group. Late mortality occurred in 3 patients, two of which were non-cardiac. A patient in OHS group was documented to have neurologic sequelae. All the survivors except two are in NYHA class 1. Conclusion: Complete repair and palliative operations other than PAB can be performed in low birth weight infants with low operative mortality and an acceptable intermediate-term result. However, about a half of the patients required long-term postoperative mechanical ventilation.

• Journal of Chest Surgery
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• v.9 no.2
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• pp.220-238
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• 1976

Six cases of congenital heart disease were operated on by means of cardiopulmonary bypass between December, 1975 and April, 1976. Two cases of ventricular septal defects (VSD), two cases of VSD, associated with ruptured aneurysm of sinus Valsalva, two cases of atrial septal defects (ASD) and one case of pulmonic stenosis with patent ductus arteriosus were operated. Sarns roller pumps and Bentley Temptrol oxygenators were used for extracorporeal circulation. Pump oxygenator was primed with Ringer's lactate solution, 5% dextrose in water, mannitol, and ACD blood. Flow rate ranged from 2.0 to $2.4L/M^2/min$. Bicarbonate was added to the oxygenator with estimated amount as 15 mEq/L/hr. Venous catheters were introduced into superior and inferior vena cava, and oxygenated blood was returned to the body through aortic cannula inserted into ascending aorta. Moderate hypothermia ($30^{\circ}C$) was induced by core cooling. Aorta was cross clamped for 15 minutes and released for 3 minutes, and repeated clamping when necessary. Atrial and ventricular septal efects were closed by direct sutures. Aneurysms of sinus Valsalva ruptured into the right ventricle were repaired through right ventriculotomy by d:rect closure with Dacron patch reinforcement. Cardiopulmonary bypass time varied from 66 to 209 minutes, and aorta cross clamping time ranged from 13 to 56 minutes. Postoperative bleeding was minimal except one case who needed for evacuation of substernal hematoma. Intra- and postoperative urinary output was satisfactory. Acid-base balance, partial pressure of $O_2$, electrolytes, and hematological changes during intra- and post-perfusion period remained at the acceptable ranges. No mortality was experienced.