• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.622-628
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• 2002

Purpose : The purpose of this study is to ascertain the clinical manifestations and associated cardiac anomalies of dextrocardia. Methods : Twenty-four pediatric patients, who were admitted to, or visited, Dongsan Medical Center Keimyung University and were diagnosed with dextrocardia between January 1996 and July 2001, were enrolled in this study. All patients received echocardiogram and abdominal ultrasonogram to identify structural cardiac anomalies and visceral position. Results : Among 24 patients, 7 cases were situs solitus(29.2%), 10 cases(41.6%) were situs inversus and 7 cases(29.2%) were situs ambiguous. Most were diagnosed within a week of life(87.5%). They were most commonly cyanotic(45.8%), but eleven cases(45.8%) had no specific symptoms or signs. Nineteen cases(79.2%) had accompanied cardiac anomalies, and pulmonary stenosis or atresia were the most common among them. All the cases of situs solitus and ambiguous had associated cardiac anomalies, but half of the situs inversus had that. Eleven cases were dead by cardiac or extracardiac anomalies during follow up and the mortality rate was higher in situs solitus or ambiguous group than situs inversus group. Conclusion : Dextrocardia accompanies different cardiac and extracardiac anomalies. It's very important to diagnose dextrocardia and associated cardiac or extracardiac anomalies in the early stages of life to improve prognosis.

• Journal of Yeungnam Medical Science
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• v.4 no.2
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• pp.75-81
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• 1987

Bone grafts are an integral and important aspect of craniofacial reconstruction. Ribs, tibia, and iliac bone have traditionally used as donor site but each of these has various problems, however using to the calvarial bone as a donor site has several advantage. These are as follows ; there are abundance of material, easy to reach the donor site through coronal incision, minimal pain of donor area, less functional inability, shorter hospitalization, no need of immobilization, hidden scar at donor site, no secondary deformity and appropriate curvature obtained properly selected. From march to December 1987, we experienced three cases of autogenous calvarial bone graft such as congenital saddle nose deformity, fibrous dysplasia on the right side frontal bone, and deviated nose. The results were very excellent without any significant complication. The detail technique of autogenous calvarial bone graft and its advantages compared with the traditional methods of bone grafts are discussed.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.22-32
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• 1990

Postoperative cardiac performance of cyanotic congenital heart disease is somewhat different from that of other cardiac diseases. For the evaluation of postoperative cardiac performance in the cyanotic congenital heart disease we measured cardiac output by thermodilution technique at 1, 4, 8, 12, 16, 20, 24, 36, 48 postoperative hours in 14 patients operated from Feb. 1989 to Nov. 1989 in The Department of Thoracic and Cardiovascular Surgery, Seoul National University Children`s Hospital. At the same time, we checked left atrial pressure [LAP], central venous pressure [CUP], and mixed venous oxygen saturation [SvO2] to detect correlation between them. Immediate postoperative cardiac index was 3.585 $\pm$ 0.945 L/min/m2, and it decreased maximally to 3.322$\pm$1.007 L/min/m2 at postoperative 16 hours. After then it increased and stabilized from 36 hours after operation, and its value was 4.426$\pm$1.358 L/min/m2. There were no correlations between cardiac index and left atrial pressure or central venous pressure. Between mixed venous oxygen saturation and cardiac index, there was no correlation in the early postoperative period but after postoperative 16 hours, there was significant correlation between them and correlation coefficients were 0.573 [16hrs], 0.743 [20hrs], 0.436 [24hrs], 0.560 [36hrs], 0.636 [48hrs], respectively. From these results, we concluded that in the corrective surgery of cyanotic congenital heart disease, cardiac performance was depressed in the early postoperative period. It improved from postoperative 16 hours, and stabilized from 36 hours after operation. During early postoperative period, mixed venous oxygen saturation should not be used as a predictor of cardiac performance but it could be used as a predictor of cardiac performance from 16 hours after operation.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.601-608
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• 1989

We sought the relationship between the pulmonary artery development and cardiac performance from the analysis of the 36 cyanotic congenital heart disease patients [mainly TOF] who were operated and indwelled the pulmonary artery and left atrial pressure monitoring catheters for the postoperative care at the department of Thoracic and Cardiovascular Surgery SNUH in 1988. They were pre-evaluated of the pulmonary artery index from the cineangiographic films and post-operatively, calculated the cardiac index from the arterial and mixed venous blood gas and also measured the mean left atrial pressure and total inotropic supporting amount after operation. The post-operative cardiac indices were 3.46 * 1.03 1/min/M2 [immediate postoperative], 3.31 [ 1.08 [postoperative 6 hrs], 3.29 [ 1.01 [12 hrs], 3.54 * 1.02 [24 hrs], 3.92 * 1.14 [48 hrs], respectively. We divided the patients the group A and group B from the size of the pulmonary artery index, that is, group A was below 200 mm2/M2 and group B above 200 mm2/M2 of the pulmonary artery index. The cardiac indices and left atrial pressure between the group A and B were not different in the view of the statistical significance but the total post-operative inotropic amount of group A was more than group B and it was significant statistically. We concluded that the smaller the pulmonary artery index is, the more postoperative supportive treatment [for example, inotropics] for the enhancement of cardiac performance is needed, and indirectly, which means that the pulmonary artery index was correlated with the postoperative cardiac performance in cyanotic congenital heart disease.

• Neonatal Medicine
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• v.16 no.1
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• pp.71-75
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• 2009

There are few cases of partial trisomy of 9q, known as partial 9q trisomy syndrome with low birth weight, microcephaly, hypotelorism, beaked nose, small lip, long finger, hypertrophic pyloric stenosis, ventricular septal defect, and mental retardation. We report partial trisomy of 9q derived from a paternal chromosome, which has different features of other syndromes, including prematurity, atrial and ventricular septal defect, patent ductus arteriosus, persistent left superior vena cava, congenital hydronephrosis, and scrotal hernia.

• Childhood Kidney Diseases
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• v.15 no.1
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• pp.86-89
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• 2011

Imperforate hymen is, with an incidence of 0.1%, a rare female anomaly, which can appear with symptoms such as lower abdominal pain, primary amenorrhea, dysuria, anuria, caused by retention of menstrual blood after the onset of menstruation. Generally urinary retention is caused by psychological conditions, drug effect, infection or congenital anomaly causing acute urinary obstruction. We experienced a patient with symptoms of acute urinary retention, suggesting acute urinary obstruction. The cause for the retention turned out to be an imperforated hymen, which should therefore be mentioned in the literature as a possible cause in cases suspected of urinary retention.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.499-503
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• 2008

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.81-87
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• 2011

This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19 % of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8 %). But 47.6 % were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arterious (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8 %), anorectal malformations were the most frequent, 70 % Vertebral and limb abnormalities accounted for 11.9 % and urogenital malformations 9.5 % of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8 % and 1.8% had full VACTERL. Almost 12 % of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8 % of patients in our study, only 21.42 % needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.11 no.2
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• pp.19-27
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• 1989

Hemifacial microsomia is characterized underdevelopment of the TMJ, mandibular ramus, and associated muscles of mastication. The Maxilla and malar bones on the affected side frequently are underdeveloped. The contiguous parotid gland may be hypoplastic. Preauricular sinus tracts and tags may exist, along with underdevelopment of the associated external ear, and affected facial nerve and muscles of facial expression may also show dysfunction. Children exhibiting the more classic signs will be identified at birth. Little is known about the etiology of hemifacial microsomia. We have corrected surgically a 22-year-old woman with hemifacial microsomia. We have performed leveling Le Fort I osteotomy with iliac bone graft on the maxilla, reverse-L osteotomy and iliac bone graft on the right mandibular ramus, vertical ramus osteotomy on the left side, onlay bone graft on the right mandibular body, and augmentation genioplasty. The postoperative course was uneventful and restoration of facial asymmetry was achieved.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.145-149
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• 2006

The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation or Lecompte operation is the preferred surgical procedure, but its long-term results are not optimal because of a warped left ventricular outflow tract through a space-occupied intraventricular tunnel and a contrived right ventricular outflow tract. We performed a half-turned truncal switch operation as an alternative surgical procedure in a 3-year-old boy (weighing 9.6 kg) with this anomaly. Postoperative echocardiography showed laminar flow through straight and nonobstructive aortic and pulmonary ventricular outflow tracts.