• Journal of Chest Surgery
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• v.30 no.11
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• pp.1069-1076
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• 1997

There remains controversy regarding the appropriate surgical treatment for coarctation of the aorta because of relatively high rate of recoartation and high mortality in the cases associated with complex anomalies. We evaluated 31 consecutive patients who underwent surgical repair of coarctation of the aorta from May 1992 through June 1996. Nineteen patients(61.3%) were neonates and 26(83.9%) were under three months. Nine patients did not have major associated anom lies(Group I), 15 patients had ventricular septal defect(Group II), and 7 patients had major complex anomalies(Group III). 35.5% of the patients had arch hypoplasia. Surgical procedures performed were as follows: extended end-to-end anastomosis in 17 patients, combined resection-flap procedure in 7 patients, and subclavian flap aortoplasty in 7 patients. Residual coarctation occurred in 7(25%) of 28 patients; 2 after subclavian (lap aortoplasty(2/6, 33.3%), none after combillrd resection-flap procedure(0/7, 0%), and 5 after extended end-to-end anastomosis(5/15, 33.3%). Higher incidence of residual coarctation was noticed in the group with arch hypoplasia. The incidence of postoperative coarctation at a mean follow-up of 20.5 months in survivals was 12.0%(3/25); 2 cases after subclavian flap aortoplasty(2/6, 33.3%), none after combined resection-flap procedure(017, 0%), and one after endtoend anastomosis(1/12, 8.3%). The mortality rate related to coarctation repair was 9.7%(3 patients all in Group III). This study revealed that isolated coarctation of aorta and coarctation with ventricular septal di3fect(groups I & ll) can be repaired with low mortality, but repair of coarctation with complex anomaly had a high operative mortality Also the patients with arch hypoplasia had higher incidence of post-operative residual coarctation.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.705-711
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• 2002

The advantages of mitral valve reconstruction have been well established and so mitral valve reconstruction is now considered as the procedure of choice to correct mitral valve disease. This is the report of intermediate-term results of 38 cases that performed mitral valve reconstruction for valve insufficiency(the total number of mitral valve reconstruction were 49 cases, but 11 cases that performed mitral valve replacement due to incomplete reconstruction were excluded). Material and Method : From March 1991 to March 2001, 38 patients underwent mitral valve repair due to mitral valve regurgitation with or without stenosis. Mean age was 47.6$\pm$14.7 years(range 15 to 70 years) : 11 were men and 27 were women. The causes of mitral valve regurgitation were degenerative in 14, rheumatic in 21, infective in 2 and the other was congenital. Result : According to the Carpentier's pathologic classification of mitral valve regurgitation, 3 were type 1 , 16 were type II and 19 were type III. Surgical procedures were annuloplasty 15, commissurotomy 19, leaflet resection and annular plication 9, chordae shortening 11, chordae transfer 5, new chordae formation 2, papillary muscle splitting 2 and vegetectomy 2. These procedures were combined in most patients. There were 2 early death and the causes of death were respiratory failure, renal failure and sepsis. There was no late death. Valve replacement was done in 6 patients after repair due to valve insufficiency or stenosis 3 weeks, 1, 3, 51, 69, 84months later respectively. These patients have been followed up from 1 to 116 months(mean 43.0 months). The mean functional class(NYHA) was 2.36 pre-operatively and improved to 1.70. Conclusion : In most cases of mitral valve regurgitation, mitral valve reconstruction when technically feasible is effective operation that can achieve stable functional results and low surgical and late mortality.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.793-795
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• 2004

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.873-877
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• 2005

Jeune's asphyxiating thoracic dystrophy is a rare, complex malformation with a broad spectrum of clinical expression. The degree of chest wall deformity is the most important prognostic factor and the only part which is correctable. A 11 month-old male infant was diagnosed as having Jeune's syndrome and received right side lateral thoracic expansion surgery. But because respiratory distress symptom was sustained postoperatively, we performed left side procedure 3 months after the initial operation. Respiratory distress symptom got worse after fracturing the left titanium plate which was inserted to fix the expanded thoracic wall and reimplantation was performed. The patient was discharged 6 months after the initial operation. He was readmitted and received ventilator care for respiratory failure and died 10 months after the initial operation.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.417-422
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• 2008

Background: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. Material and Method: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was $3.2{\pm}4.9$ years (range: 7 days$\sim$45 years) and the mean body weight was $12.5{\pm}8.7\;kg$ (range: $2.4\sim76.3\;kg$). Result: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was $6.3{\pm}5.2$ years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. Conclusion: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV outflow.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.54-59
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• 2014

Adrenoleukodystrophy is an X-linked genetic disease resulting from mutations in the ABCD1 gene. Cerebral adrenoleukodystrophy is one of the phenotypes of adrenoleukodystrophy and shows progressive demyelination of brain white matter and adrenal insufficiency. We report a nine year old male who presented with rapidly progressive cognitive and neurologic deterioration. He had abnormal findings in brain imaging and elevated very long chain fatty acid level in serum. Mutation analysis of ABCD1 revealed a c. 1252C>T (p.Arg418Trp) mutation which was previously known but not reported in Korea.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.890-893
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• 2002

Ebsteins anomaly is a rare congenital cardiac malformation that is the downward displacement of the septal and posterior leaflets of the tricuspid valve into the ventricular apex, aresulting in the formation of an atrialized portion of the right ventricle. In most patients, symptoms such as cyanosis and progressive heart failure occur during the adolescence or young adulthood. The associated anomalies include ventricular septal defect, transposition of the great arteries, and malformation of the mitral valve. An 8-months-old male with Ebsteins anomaly, ventricular septal defect, and duplication of the mitral valve orifice successfully underwent operation with VSD patch closure. Postoperatively, cardiac size was significantly reduced and tricuspid regurgitation was trivial in echocardiography He has been doing well without any cardiac medication during the 18 months follow up after operation.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.268-271
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• 2007

Purpose : Premature narrowing of the foramen ovale is rare but serious clinical entity. Prenatal narrowing or obstruction of the foramen ovale shows symptoms such as right heart failure, fetal hydrops, triscupid regurgitation, left heart obstructive disease, and supraventricular tachycardia. This study aimed to assess the prenatal diagnosis and postnatal clinical course of restrictive foramen ovale in utero in otherwise normal heart. Methods : The subjects were five patients diagnosed with restrictive foramen ovale in utero from January 2001 to June 2005 at Chungnam National University Hospital. The diagnostic criteria was defined when the maximum diameter in a 4-chamber view is less than 2.5 mm and there is a continuous doppler velocity at the foramen ovale of more than 0.6m/s. Results : At the time of diagnosis of restrictive foramen ovale, gestation age was 34~37 wks, and chief complaints were fetal arrhythmia(2 cases), pericardial effusion, Ebstein anomaly and subaortic stenosis. Two cases which were diagnosed fetal hydrops and supraventricular tachycardia delivered by emergent cesarian section. Five cases were found to have right heart dilatation on echocardiogram after birth, but right heart dilatation became normalized at day 7 after birth and the clinical courses were not eventful. Conclusion : Identifying an obstructed foramen ovale in the fetus warrants the further search for additional cardiac and extracardiac anomalies, which may alter the prognosis. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.285-291
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• 1996

We have reviewed 157 cases of coarctation of aorta in the age of infancy and children from March 1973 to december 1994. Patients were 9) males and 64 females, and their age ranged from one month to fifteen years (23.8 $\pm$ 41.6months) with 113 infant cases. In the infantile age, congestive heart failure was the most common chief complaint (781113), and above that age, asymptom was most common (15/44). Isolated coarctation was present in 31 patients, and 9 patients had additional atrial septal defect (group I), 73 patients had associated ventricular septal defect (group II), and 44 patients had associated complex intracardiac lesions(group III). Aortic arch hypoplasia was present in 17 patients. , The operations perf'ormed were subclavian flap angioplasty in 71 patients, resection and anastomosis in 32 patients, patch aortoplasty in 26 patients, resd extended end-!o-end anastomosis in 27 patients, and direct angioplasty with resection of web in 1 patient. The early mortality was 17.2% (27/157) and re-coarctation rate was 18.0% (281157). The most common complication was respiratory problem in infants and postoperative hypertension in children.