• Title/Summary/Keyword: 선천성심기형

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Clinic Analysis of Heart Surgery -110 cases- (심장수술 110례 임상고찰)

  • Chang, Hoon;Shin, Sung-Aia;No, Joong-Kee;Rho, Jun-Ryang
    • Journal of Chest Surgery
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    • v.34 no.8
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    • pp.597-603
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    • 2001
  • Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

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Retroperitoneal Bronchogenic Cyst Located in the Presacral Space: A Case Report (전천추 부위에 생긴 후복강 기관지 낭종: 증례 보고)

  • A Yeon Kim;Seon Jeong Min;Hyunchul Kim;Jung-Ah Choi
    • Journal of the Korean Society of Radiology
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    • v.82 no.1
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    • pp.207-211
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    • 2021
  • Bronchogenic cysts are rare congenital anomalies that are most frequently found in the mediastinum along the tracheobronchial tree, especially in the posterior aspect of the superior mediastinum. Bronchogenic cysts have also been reported in intrapulmonary, intrapericardial, abdominal, and retroperitoneal locations. Herein, we report a case of a retroperitoneal bronchogenic cyst in the presacral space. The patient was diagnosed based on a post-operative histopathological examination.

Simultaneous Repair of Secondary Anterior Chest Wall Deformity and Secundum Atrial Septal Defect -1 Case Report- (심방중격결손을 동반한 이차성 전흉벽기형의 동시교정 -1예 보고-)

  • 김용희;정종필
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1247-1250
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    • 1997
  • A 13-year-old boy presented with anterior chest wall depression and dyspnea on exertion(NYHA II). He underwent Ravitch operation for pectus excavatum 7 years ago. A preoperative echocardiographic study revealed secundum atrial septal defect. He had no other abnormality of laboratory test, except FVC and FEVI were decreased into 2.03 L(7 %) and 1.82 L(71 %). He underwent repair of secondary anterior chest wall deformity and secundum atrial septal defect. We used unique method, raising sternum at right angle to secure good operative field for open heart surgery. Acute respiratory insufficiency was developed on postoperative day 1. Mechanical ventilation was applied which could be weaned on postoperative day 6 and thereafter hospital course was uneventful without any other sequale. He was discharged on postoperative day 19.

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A case of Hallermann-Streiff syndrome with aphakia (무수정체안을 동반한 Hallermann-Streiff 증후군 1예)

  • Lee, Myung Chul;Choi, Im Jeong;Jung, Jin Wha
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.646-649
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    • 2008
  • Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.

Histidine-tryptophan-ketoglutarate Versus Blood Cardioplegic Solutions: A Prospective, Myocardial Ultrastructural Study (선천성 심장기형의 수술 후 Histidine-tryptophan-ketoglutarate 심정지액과 혈성 심정지액의 전자현미경적 심근 구조의 비교 관찰)

  • Kim, Si-Ho;Lee, Young-Seok;Woo, Jong-Soo;Sung, Si-Chan;Choi, Pil-Jo;Cho, Gwang-Jo;Bang, Jung-Heui;Roh, Mee-Sook
    • Journal of Chest Surgery
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    • v.40 no.1 s.270
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    • pp.8-16
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    • 2007
  • Background: We performed a prospective clinical study to evaluate the ultrastructural integrity of the myocardium after using Histidine-Tryptophan-Ketoglutarate (HTK) solution in comparison with blood cardioplegic solution during congenital heart surgery. Material and Method: Twenty two patients with acyanotic heart disease, who were scheduled for elective open heart surgery, were randomized into two groups. The HTK Group (n=11) received HTK cardioplegic solution; the blood group (n=11) received conventional blood cardioplegic solution during surgery. The preoperative diagnoses included ventricular septal defect (n=9) and atrial septal defect (n=2) in each group. A small biopsy specimen was taken from the right ventricle's myocardium, and this was processed for ultrastructural examination at the end of 30 minutes of reperfusion. Semiquantitative electron microscopy was carried out 'blindly' in 4 areas per specimen and in 5 test fields per area by 'random systematic sampling' and 'point and intersection counting'. The morphology of the mitochondrial membrane and cristae were then scored. The interstitial edema of the myocardium was also graded. Result: The semiquantitative score of the mitochondrial morphology was $19.65{\pm}4.75$ in the blood group and $25.25{\pm}5.85$ in the HTK group (p=0.03). 6 patients (54.5%) in the blood group and 3 patients (27.3%) in the HTK group were grade 3 or more for the interstitial edema of the myocardium. Conclusion: The ultrastructural integrity was preserved even better with HTK solution than with conventional blood cardioplegic solution.

Long-term Results of Modified Lecompte Procedure for the Anomalies of Ventriculoarterial Connection (심실대혈관 연결 이상에 대한 변형된 Lecompte 술식의 장기 성적)

  • 임홍국;한국남;김웅한;이정렬;노준량;김용진
    • Journal of Chest Surgery
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    • v.37 no.9
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    • pp.727-734
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    • 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.

Mid-term results of IntracardiacLateral Tunnel Fontan Procedure in the Treatment of Patients with a Functional Single Ventricle (기능적 단심실 환자에 대한 심장내 외측통로 폰탄술식의 중기 수술성적)

  • 이정렬;김용진;노준량
    • Journal of Chest Surgery
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    • v.31 no.5
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    • pp.472-480
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    • 1998
  • We reviewed the surgical results of intracardiac lateral tunnel Fontan procedure for the repair of functional single ventricles. Between 1990 and 1996, 104 patients underwent total cavopulmonary anastomosis. Patients' age and body weight averaged 35.9(range 10 to 173) months and 12.8(range 6.5 to 37.8) kg. Preoperative diagnoses included 18 tricuspid atresias and 53 double inlet ventricles with univentricular atrioventricular connection and 33 other complex lesions. Previous palliative operations were performed in 50 of these patients, including 37 systemic to pulmonary artery shunts, 13 pulmonary artery bandings, 15 surgical atrial septectomies, 2 arterial switch procedures, 2 resections of subaortic conus, 2 repairs of total anomalous pulmonary venous connection and 1 Damus-Stansel-Kaye procedure. In 19 patients bidirectional cavopulmonary shunt operation was performed before the Fontan procedure and in 1 patient a Kawashima procedure was required. Preoperative hemodynamics revealed a mean pulmonary artery pressure of 14.6(range 5 to 28) mmHg, a mean pulmonary vascular resistance of 2.2(range 0.4 to 6.9) wood-unit, a mean pulmonary to systemic flow ratio of 0.9(range 0.3 to 3.0), a mean ventricular end-diastolic pressure of 9.0 (range 3.0 to 21.0) mmHg, and a mean arterial oxygen saturation of 76.0(range 45.6 to 88.0)%. The operative procedure consisted of a longitudinal right atriotomy 2cm lateral to the terminal crest up to the right atrial auricle, followed by the creation of a lateral tunnel connecting the orifices of either the superior caval vein or the right atrial auricle to the inferior caval vein, using a Gore-Tex vascular graft with or without a fenestration. Concomitant procedures at the time of Fontan procedure included 22 pulmonary artery angioplasties, 21 atrial septectomies, 4 atrioventricular valve replacements or repairs, 4 corrections of anomalous pulmonary venous connection, and 3 permanent pacemaker implantations. In 31, a fenestration was created, and in 1 an adjustable communication was made in the lateral tunnel pathway. One lateral tunnel conversion was performed in a patient with recurrent intractable tachyarrhythmia 4 years after the initial atriopulmonary connection. Post-extubation hemodynamic data revealed a mean pulmonary artery pressure of 12.7(range 8 to 21) mmHg, a mean ventricular end-diastolic pressure of 7.6(range 4 to 12) mmHg, and a mean room-air arterial oxygen saturation of 89.9(range 68 to 100) %. The follow-up duration was, on average, 27(range 1 to 85) months. Post-Fontan complications included 11 prolonged pleural effusions, 8 arrhythmias, 9 chylothoraces, 5 of damage to the central nervous system, 5 infectious complications, and 4 of acute renal failure. Seven early(6.7%) and 5 late(4.8%) deaths occured. These results proved that the lateral tunnel Fontan procedure provided excellent hemodynamic improvements with acceptable mortality and morbidity for hearts with various types of functional single ventricle.

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The Results of Extracardiac Fontan Operation in the Patients with Heterotaxy Syndrome (이소성증후군에서의 심장외도관 폰탄 수술의 결과)

  • Lim Hong Gook;Kim Soo-Jin;Lee Chang-Ha;Kim Woong-Han;Hwang Seong Wook;Lee Cheul;Oh Sam-Sae;Baek Man-Jong;Na Chan-Young;Kim Jae Hyun;Seo Hong Joo;Jung Sung Chol;Kim Chong Whan
    • Journal of Chest Surgery
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    • v.38 no.8 s.253
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    • pp.529-537
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    • 2005
  • Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

Association of ionized magnesium, total magnesium, gestational age, and intraventricular hemorrhage in preterm babies (미숙아에서 이온화 마그네슘, 총 마그네슘과 재태 연령과의 관계 및 뇌실 내 출혈과의 관계)

  • Kim, Tae-Yeon;Lee, Hyun-Hee;Sung, Tae-Jung
    • Clinical and Experimental Pediatrics
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    • v.52 no.10
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    • pp.1140-1146
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    • 2009
  • Purpose:The pathophysiology of magnesium, the second highest common compound in humans, is still unclear, especially in preterm babies. We accessed the association between total magnesium (tMg), ionized Mg (iMg), and gestational age (GA) and that between serum magnesium (sMg) and intraventricular hemorrhage (IVH) in preterm babies. Methods:In all, 119 inborn preterm infants admitted between July 2006 and February 2008 were divided into the IVH group (19) and the control group (100) and were prospectively analyzed. TMg, iMg, pH, total calcium (tCa), and ionized Ca (iCa) levels were determined immediately after delivery or within 3 hours after birth, and their correlation with GA were investigated. Results:TMg was not correlated with GA, tCa, iCa, and pH. IMg was correlated with tMg (r=0.288, P=0.002) and iCa (r=0.212, P=0.021); however, it was not correlated with GA and pH. Mean GA and birth weight were significantly lower (P=0.002) and smaller (P=0.030) in the IVH group. Mean sMg was higher in the IVH group ($2.5{\pm}0.9mg/dL$) than in the control group ($2.1{\pm}0.6mg/dL$) (P=0.021). SMg was a risk factor even after logistic regression analysis (OR, 2.798; 95% C.I., 1.265-6.192; P=0.011). Conclusion:In less than 37-week-old preterm babies, tMg and iMg were similar, regardless of GA. High sMg may be a risk factor for IVH in premature babies, regardless of their exposure to antenatal magnesium.

Valve Replacement in Children (소아심장판막치환술)

  • 김재현;이광숙;윤경찬;유영선;박창권;최세영
    • Journal of Chest Surgery
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    • v.32 no.4
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    • pp.341-346
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    • 1999
  • Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

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