• Journal of Chest Surgery
• /
• v.37 no.9
• /
• pp.727-734
• /
• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

• Journal of Chest Surgery
• /
• v.38 no.3 s.248
• /
• pp.214-220
• /
• 2005

Extended transseptal approach can provide an excellent view of the mitral valve but the safety of this approach is controversial because this incision requires transaction of the sinus node artery, which in most cases and can result postoperative arrhythmia. The purpose of this study was to evaluate perioperative and longterm conduction disturbances and the cardiac rhythms of patients who underwent an extended transseptal approach for mitral valve surgery. Material and Method: Postoperative cardiac rhythms were analyzed in the 164 consecutive patients who received mitral valve replacements with a extended transseptal approach between March 1992 and July 2003. Result: Of the 84 patients in normal sinus rhythm, 34 ($39\%$) had developed transient junctional rhythm and atrial fibrillation after operation, lasting less than 72 hours in most of cases. No intractable arrhythmias occurred. Most of these arrhythmia were not detected at the time of discharge and only 8 patients ($9\%$) had atrial fibrillation at discharge. Postoperative PR intervals increased for 1 week, then decreased within 2 weeks postoperatively, and returned to normal range by 6 months postoperatively. During the postoperative period, 4 of the 78 patients with preoperative atrial fibrillation developed normal sinus thythm. Conclusion: The post-operative arrhythmias were temporary and showed no significant complications after extended transseptal approach for the mitral valve surgery.

• Clinical and Experimental Pediatrics
• /
• v.49 no.4
• /
• pp.417-423
• /
• 2006

Purpose : The objectives of this study were to assess ventricular function by tissue Doppler imaging in children who were receiving chemotherapy or who had received chemotherapy, and to apply repeated tissue Doppler imaging to make an early assessment in cardiac toxicity studies. Methods : This study was conducted on 23 oncology patients on-treatment or off-treatment from April 2005 to July 2005 at Dongsan Medical Center, Keimyung University. All patients(group 1) were divided into two groups, fractional shortening(FS) over 29 percent(group 2) and FS under 28 percent (group 3) in the first category. These same patients were also divided into the following groups : group treated with anthracyclin(group 4) and group treated without anthracyclin(group 5). Deceleration time(DT), isovolumic relaxation time(IVRT), FS, peak early diastolic(E), and peak late diastolic (A) velocity of transmitral flow were measured by M-mode and pulsed wave Doppler. Systolic(Sm), peak early diastolic(Em), and peak late diastolic(Am) velocity in apical 4-chamber and 2-chamber views were measured by tissue Doppler imaging. The author calculated a modified Tei index, E/A, E/Em ratio by using measured values. Results : Twenty three patients were enrolled : 12 boys and 11 girls. The average age of patients was 8 years and 4 months. Thirteen out of 23 patients were in the group treated with anthracyclin (group 4) and 6 had FS under 28 percent(group 3). E/Em ratio showed a significant difference between group 1 and control group($6.46{\pm}1.85$ vs $7.06{\pm}1.64$, P<0.05). Other parameters had no difference statistically. Conclusion : This study showed that the change of cardiac function developed earlier in diastolic function than in systolic function, as E/Em ratio reflecting the mean LV diastolic pressure showed a significant difference between the control group and chemotherapy groups. Echocardiography using tissue Doppler imaging is a non-invasive, comfortable and reliable method for post-chemotherapy follow up.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.2 no.1
• /
• pp.46-58
• /
• 1999

Purpose: The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis. Method: We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed. Result: Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis. Conclusion: This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

• Tuberculosis and Respiratory Diseases
• /
• v.45 no.1
• /
• pp.153-168
• /
• 1998

Background: The existing data indicate that obstructive sleep apnea syndrome contributes to the development of cardiovascular dysfunction such as systemic hypertension and cardiac arrhythmias, and the cardiovascular dysfunction has a major effect on high long-term mortality rate in obstructive sleep apnea syndrome patients. To a large extent the various studies have helped to clarify the pathophysiology of obstructive sleep apnea, but many basic questions still remain unanswered. Methods: In this study, the influence of obstructive sleep apnea on systemic blood pressure, cardiac rhythm and urinary catecholamines concentration was evaluated. Over-night polysomnography, 24-hour ambulatory blood pressure and ECG monitoring, and measurement of urinary catecholamines, norepinephrine (UNE) and epinephrine (UEP), during waking and sleep were undertaken in obstructive sleep apnea syndrome patients group (OSAS, n=29) and control group (Control, n=25). Results: 1) In OSAS and Control, UNE and UEP concentrations during sleep were significantly lower than during waking (P<0.01). In UNE concentrations during sleep, OSAS showed higher levels compare to Control (P<0.05). 2) In OSAS, there was a increasing tendency of the number of non-dipper of nocturnal blood pressure compare to Control (P=0.089). 3) In both group (n=54), mean systolic blood pressure during waking and sleep showed significant correlation with polysomnographic data including apnea index (AI), apnea-hypopnea index (AHI), arterial oxygen saturation nadir ($SaO_2$ nadir) and degree of oxygen desaturation (DOD). And UNE concentrations during sleep were correlated with AI, AHI, $SaO_2$ nadir, DOD and mean diastolic blood pressure during sleep. 4) In OSAS with AI>20 (n==14), there was a significant difference of heart rates before, during and after apneic events (P<0.01), and these changes of heart rates were correlated with the duration of apnea (P<0.01). The difference of heart rates between apneic and postapneic period (${\Delta}HR$) was significantly correlated with the difference of arterial oxygen saturation between before and after apneic event (${\Delta}SaO_2$) (r=0.223, P<0.001). 5) There was no significant difference in the incidence of cardiac arrhythmias between OSAS and Control In Control, the incidence of ventricular ectopy during sleep was significantly lower than during waking. But in OSAS, there was no difference between during waking and sleep. Conclusion : These results suggested that recurrent hypoxia and arousals from sleep in patients with obstructive sleep apnea syndrome may increase sympathetic nervous system activity, and recurrent hypoxia and increased sympathetic nervous system activity could contribute to the development of cardiovascular dysfunction including the changes of systemic blood pressure and cardiac function.

• Clinical and Experimental Pediatrics
• /
• v.52 no.4
• /
• pp.458-463
• /
• 2009

Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively. Results : The mean age at diagnosis was $9.08{\pm}2.67$ months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures. Conclusion : Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.

• Journal of Chest Surgery
• /
• v.38 no.4 s.249
• /
• pp.284-290
• /
• 2005

We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

• Journal of Chest Surgery
• /
• v.30 no.2
• /
• pp.131-136
• /
• 1997

The hemodynamic effects of thyroid hormones which is well established, affect myocardial contractility, heart rate, and myocardial oxygen consumption. The alterations in thyroid function test are frequently seen in patients with nonthyroidal illness and often correlate with the severity of the illness and the prosnosis. In this study, thyroid hormone changes were investigated in 20 patients who received cardiopulmonary bypass(CPB). All patients showed a state of biochemical euthyroidism preoperatively: The results were as follows : 1. Serum triiodothyronine(73) reached to its nadir(30.05 $\pm$ 17.5ng/dl, p(0.001) at 10 minutes after the start of CPB and remained low(p(0.05) throughout the study period. 2. Serum thyroxine(74) concentr tion slightly decreased after CPB, but maintained within normal range. 3. Serum free thyroxine(W4) concentration slightly increased after CPB, but maintained within normal range. 4. Serum thyroid stimulating hormone(TSH) concentration increased 10 minute after CPB, reached to its nadir(3.37 $\pm$ 0.81u1U/m1, p(0.001) at 2 hours after CPB. After then, serum TSH concentration decreased and reached its normal levels at 24 hours after CPB. 5. The patients whose postoperative recovery was uneventful(Group 1) had higher serum 73 levels than those who had postoperative complications(Group 2)(p<0.05). Group 1 showed elevating patterns of serum 73 in the fourth day after operation, whereas group 2 did not show such an elevating pattern. These findings are similar to the euthyroid sick syndrome seen in severely ill patients and indicate that patients undergoing open heart surgery have suppression of the pituitary-thyroid axis.

• Journal of Chest Surgery
• /
• v.30 no.10
• /
• pp.966-972
• /
• 1997

Between June 1994 to August 1996, 13 patients underwent emergency coronary artery bypass operations. There were 3 males and 10 females and ages ranged from 56 to 80 years with the mean of 65.5 years. The indications for emergency operations were cardiogenic shock in 12 cases and intractable polymorphic VT(ve'ntricular tachycardia) in 1 case. The causes of cardiogenic shock were acute evolving infarction in 6 cases, PTCA failure in 4 cases, acute myocardial infarction in 1 case, and post-AMI VSR(ventricular septal rupture) in 1 case. Pive out of 13 patients could go to operating room within 2 hours. However, the operations were delayed from 3 to 10 hours in 8 patients due to non-medical causes. In 12 patients, 37 distal anastomoses were constructed with only 3 LITA's(left internal thoracic arteries) and 34 saphenous veins. In a patient with post-AMI VSR, VSR repair was added. In a patient with intractable VT and critical sten sis limited to left main coronary artery, left main coronary angioplasty was performed. Pive patients died after operation with the operative mortality of 38.5%. Three patients died in the operating room due to LV pump failure, one patient died due to intractable ventricular tachycardia on postoperative second day, and one patient died on postoperative 7th day due to multi-organ failure with complications of mediastinal bleeding, low cardiac output syndrome, ARF, and lower extremity ischemia due to IABP. In 8 survived patients, 3 major complications (mediastinitis, PMI, UGI bleeding) developed but eventually recovered. We think that the aggressive approach to critically ill patients will salvage some of such patients and the most important factor for patient salvage is early surgical intervention before irreversible damage occurs.

• Journal of Chest Surgery
• /
• v.42 no.2
• /
• pp.175-183
• /
• 2009

Background: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement or pediatric mitral lesions can cause problems such as a lack of growth potential. There re only limited experiences with mitral valve repair at any institution, so the purpose of his study is to evaluate the outcomes of mitral valve repair n pediatric patients. Material and Method: Sixty-four consecutive children (28 males and 36 females) with a mean age of $5.5{\pm}4.7$ years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without trial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). Result: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there as no significant difference among the groups. The median follow-up was 4.6 years range: $0.5{\sim}12.2$ years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of $2.1{\pm}1.1$ procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there ere significant differences according to the repair strategies. Conclusion: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.