• Journal of Chest Surgery
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• v.56 no.3
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• pp.155-161
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• 2023

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m², p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.466-471
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• 1998

Purpose: The safety and efficacy of minimally invasive techniques in congenital heart surgery were tested in this study. Materal and method: Between July 1997 and November 1997, a total of 46 children were underwent minimally invasive cardiac operations at Seoul National University Children's Hospital. Age and body weight of the patients averaged 34.6${\pm}$41.8 (Range: 1∼148) months and 14.5${\pm}$9.9(Range: 3.0∼40.0) kg, respectively. Twenty eight patients were male. Preoperative surgical indications included 15 atrial septal defects, 25 ventricular septal defects, 1 foreign body in aorta, 3 partial atrioventricular septal defects, 1 total anomalous pulmonary venous connection(cardiac type), and 1 tetralogy of Fallot. After creating a small lower midline skin incision starting as down as possible from the sternal notch, a vertical midline sternotomy extended from xyphoid process to the level of the second intercostal space, where one of the T-, J-, I- or inverted C-shaped lower lying mini-sternotomy was completed with a creation of unilateral right or bilateral trap door sternal opening. A conventional direct aortic and bicaval cannulation was routine. Result: A mean length of skin incision was 6.1${\pm}$1.0(range: 4.0∼9.0) cm. A mean distance between the suprasternal notch and the upper most point of the skin incision was 4.0${\pm}$1.1 (range: 2.0∼7.0) cm. Mean cardiopulmonary bypass time, aortic cross-clamp time, and the operation time were 62.9${\pm}$20.0(range: 28∼147), 29.8${\pm}$12.8(range: 11∼79), and 161.1${\pm}$34.5 (range: 100-250) minutes. A mean total amount of postoperative blood transfusion was 71.0${\pm}$68.1 (range: 0∼267) cc. All patients were extubated mean 11.3${\pm}$13.8(range: 1∼73) hours after operation. A mean total amount of analgesics used was 0.8${\pm}$1.8(range: 0∼9) mg of morphine. The mean duration of stay in intensive care unit and hospital stay were 35.0${\pm}$32.2 (range: 10∼194) hours and 6.2${\pm}$2.0(range: 3∼11) days. There were no wound complications and hospital deaths. Conclusion: This short-term experience disclosed that the minimally invasive technique can be feasibly applied in a selected group of congenital heart disease as well as is cosmetically more attractive approach.

• Journal of Chest Surgery
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• v.34 no.11
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• pp.831-842
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• 2001

Background: Patients with cardiac diseases who have structural defects in their heart bring about metabolic insult such as preoperative acid-base imbalance. Cardiac operation requires many nonphysiologic procedures such as extracorporeal circulation, hypothermia, and hemodilution. We studied the acid-base status of surgical heart diseases pre-operatively, during extracorporeal circulation, and post-operatively and researched the treatment indications of acid-base disturbances. Material and Method: From January 1997 to May 1999, fifty two cases of open heart surgery were carried out under extracorporeal circulation, which divided into a set of pediatric and adult groups, congenital and acquired groups, non-cyanotic and cyanotic groups, The $\alpha$ -stat arterial blood gas analysis was done in each group during the preoperative period, during the operation with extracorporeal circulation, and during the postoperative period. Result: Before surgery, all patients present metabolic acidosis, PaO2 was low in adult group and acquired group and compensatory respiratory alkalosis was noted in cyanotic group. During extracorporeal circulation, adult group revealed alkalosis and normal in acquired group. Pediatric group presents low Pa$CO_2$, metabolic acidosis and respiratory alkalosis. Congenital group and non-cyanotic group showed non-compensatory alkalosis trend and non-compensatory respiratory acidosis were observed in cyanotic group during extracorporeal circulation. Postoperative acid-base status of adult group was recovered to normal and the standard bicarbonate was increased in the acquired group. All of the pediatric, congenital non-cyanotic, and cyanotic groups revealed the lack of buffer base.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.848-855
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• 2008

Purpose : The objective of this study was to ascertain the incidence of congenital heart disease in the western part of Gyeongnam Province in Korea. Methods : We enrolled 849 children (M:F=390:459) who were diagnosed with congenital heart disease (CHD) and received echocardiography during January 1997 to December 2005 in Gyeongsang National University Hospital. The subjects were limited to patients born from 1997 to 2004. The CHDs were classified into acyanotic types and cyanotic type. Further, acyanotic type CHDs were divided into shunt type and valvular types. Results : Numbers of initially diagnosed patients from 1997 to 2004 were as follows: 113, 128, 116, 104, 129, 97, 84, and 78. The median age of initially diagnosed CHD patients was 1 month in the acyanotic type and 0.5 months in cyanotic type. Also, an annual frequency among CHD types and gender ratio were no significant difference in every year. Incidences of CHD from 2000 to 2004 in the western part of Gyeongnam Province were 8.9, 12.7, 10.9, 10.0, and 9.7 per 1,000 live births, respectively. Conclusion : From 1997 to 2001, 100-130, and from 2002 to 2004, less than 100 patients were diagnosed with CHD. This showed a decrease in the numbers of CHD patients in the last three years. However, the Incidence of CHD was 0.89-1.24% during the last five years. Therefore, the decrease in the annual frequency of CHD was due to the recent decline in birth rate in Korea.

• Journal of Chest Surgery
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• v.30 no.4
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• pp.373-377
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• 1997

Between January, 1991 and May 1995, mitral valve repair was undertaken on 32 patients under 15 years for congenital mitral regurgitation. Mean age was 24.0$\pm$26.1 months(range 3 months to 15 years), 16 patients were male and 16 patients were female associated cardiac anomalies were found in 26 patients (81%), and ventricular septal defects were noted in 18 patients(56%). In regards to pathologic findings, there were annular dilatation(n:7), leaflet prolapse(n=18), cleft leaflet(n=5) and restricted valve motion (n=2). The method of repair consisted of annuloplasty(Modif ed Devega type) in 14, repair of redundunt leaflet in 6, closure of cleft in 5, triangular resection in 2 and splitting of papillary muscle in one. There was no operative mortality and two late deaths occurred as a result of heart failure and sepsis. Tro patients required replacement of the mitral valve after 3 months and 7 months respectivehy because of recurrent mitral regurgitation. Actuarial survival was 92.5% at 46 months and actuarial freedom from reoperation was 95% at 12 months and 92.5% at 46 months. Actuarial freedom from valve repair failure was 68% at 12 months and 61.8% at 46 months. Although valve repair failure rate was high, we believe that mitral valve repair should be of rcrred to children because of low mortality and low reoperation rate.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.3
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• pp.388-393
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• 2000

Cleft lip and palate is one of the most common congenital defects in oro-maxillo-facial region. Because most patients undergo surgical repair in early life, the sagittal jaw relationships used to be deteriorated gradually from palate surgery up to adulthood. Also, the maxillary lateral incisor may be absent or atypical-shaped in the cleft site and may not erupt or erupt ectopically, so multidisciplinary dental cares are needed for cleft lip and palate patients. The effects of the cleft lip and alveolus seem to be limited to that part of the dentofacial complex that surrounds the cleft area. In the maxillary arch, the anterior part of the non cleft segment has a tendency to be rotated forward. On the other hand, the cleft segment has a tendency to rotated slightly medially ; hence, the tendency for canines to be edge-to-edge and sometimes in crossbite. Lip and alveolus surgery adequetely correct these problems, with little untoward effect on the skeletal maxillary-mandible relationships. In this report, the patient has a repaired lip and cleft alveolus on the left side with congenital missing on '62, '22, oronasal fistula, and skeletal class III malocclusion which is not affected by lip surgery. Dental treatments for this patient including orthodontic(space supervision, functional regulator in mixed dentition, fixed therapy in permanent dentition) and prosthodontic(removable obturator with key and keyway attachment and Konus crown) therapy were performed to improve the patient's functions and esthetics.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.37-44
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• 2010

Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.

• Journal of the Korean Society of Radiology
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• v.81 no.6
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• pp.1523-1528
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• 2020

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

• The Journal of Korean Academy of Prosthodontics
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• v.54 no.3
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• pp.280-285
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• 2016

Mandible defects could be caused by congenital malformations, trauma, osteomyelitis, tumor resection. If large areas are included for reconstruction, those are primarily due to tumor resection defects. The large jaw defect results in a problem about mastication, swallowing, occlusion and phonetics, and poor esthetics causes a lot of inconvenience in daily life. It is almost impossible to be a part underwent mandibular resection completely reproduced, should be rebuilt artificially. This case is of a patient who was diagnosed with squamous cell carcinoma pT1N0M0, stage I in February 2004 and received surgery (combined mandibulectomy and neck dissection operation (COMMANDO) in oromaxillofacial surgery) in March 2004, by implant assisted removable partial denture. We could obtain good retention and stability through sufficient coverage and implant holding. Follow up period was about four years. Mandibular left third molar regions have been observed to have resorption of surrounding bone, and periodic check-ups are necessary conditions.

• Neonatal Medicine
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• v.18 no.2
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• pp.359-364
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• 2011

Purpose: Aplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin, and it can be associated with other congenital anomalies. This study was done to evaluate clinical course and outcome of ACC in neonates. Methods: Based on the medical records, we retrospectively reviewed 8 neonates diagnosed with ACC at Cheil General Hospital and Women's Health Care Center, Kwandong university College of Medicine from January 2004 to December 2010. We classified ACC by Frieden's classification and analyzed the patient's demographic data, clinical course and outcome. Results: Among 8 patients with ACC, 5 patients were classified to group 1; scalp ACC without anomalies and 3 patients to group 7; ACC localized to extremities without blistering. Defect size was from 0.3 cm to 1.5 cm, limited in the superficial skin or subcutaneous tissue without associated anomalies. Defects were healed under conservative treatment with mild scar formation within four months. Conclusion: ACC could be diagnosed easily through physical examination in neonates. All cases showed good clinical outcome without surgical treatment. However because of small numbers and small sized defects of cases, further study including lesions of large size is needed.