• Title/Summary/Keyword: 선천성 심기형

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Lecompte Procedure in Complex Congenital Heart Diseases (선천성 복잡 심기형에서의 Lecompte 술식의 유용성 및 임상적용에 관한 연구)

  • Kim, Yong-Jin;Kim, Kyung-Hwan;Lee, Suk-Jae;Song, Hyun;Oh, Sam-Se;Lee, Jeong-Ryul;Rho, Joon-Ryang;Suh, Kyung-Phill
    • Journal of Chest Surgery
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    • v.31 no.7
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    • pp.660-667
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    • 1998
  • Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

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Prenatal diagnosis and clinical course of restrictive foramen ovale in otherwise normal heart (단순 조기 난원공 협착의 산전 진단과 임상경과)

  • Lee, Ji Joung;Lee, Min A;Rhee, Yun ee;Chang, Mea Young;Kil, Hong Ryang
    • 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.

Results of Mitral Valve Repair in Patients with Congenital Mitral Disease (선천성 승모판막 기형 환자에서 승모판막 성형술)

  • Jang, Hee-Jin;Lee, Jeong-Ryul;Rho, Joon-Ryang;Kim, Yong-Jin;Kim, Woong-Han
    • Journal of Chest Surgery
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    • v.42 no.2
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    • pp.175-183
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    • 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.

The Surgical Case Analysis of Congenital Heart Disease -A review of 1,060 Cases (선천성 심장병의 외과적 고찰)

  • Jo, Sam-Hyeon;O, Bong-Seok;Lee, Dong-Jun
    • Journal of Chest Surgery
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    • v.29 no.12
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    • pp.1316-1322
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    • 1996
  • From May 1977 to December 1994, 1.060 cases of open heart surge y for congenital heart disease were performed under cardiopulmonary bypass in Chonnam University Hospital They were divided into two groups; group A(677 cases, from May 1977 lo December 1989), group B(38B cases, from January 1990 to December 1994), to be compared and analized. Of the 1, 060 cases, there were 889 acyanotic (83.9%) and 171 cyanotic(16.1%). The operative mortality was 7.8% in group A and 5.7% in group B (total : 6.6%). The operative mortality for group A was 25% in cyanotic disease and 3.7% in acyanotic disease, respectively in group B, it was 23.8%, 3.2%. There were 19 patients (3.8%) and 78 patients(20.4%) with body weight under 10kg in group A and group B respectively. The operative mortality for these patients were 31.6% in A group and 16.1% in B group. In result, increasing trend in congenital heart disease in patients under 10 kg of body weight has been noted, whereas mortality has decreased. The operative mortality of tetralogy of Fallot has decreased in group B t an group A. These results suggest that the operative result have improved in recent years.

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Surgical Rrepair of Ebsteins Anomaly with Ventricular Septal Defect in the Infant (영아에서 시행한 심실중격결손이 동반된 Ebsein's 기형의 삼첨판막 성형수술)

  • 유지훈;박표원;성기익;박계현;이영탁;전태국
    • 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.

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery (우관상동맥의 폐동맥 이상 기시증(ARCAPA))

  • Esther Choi;Jeong Jun Park;Tae Jin Yoon;Young Hwoe Kim;Jae Kon Ko;In Sook Park;Dong Man Seo
    • Journal of Chest Surgery
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    • v.35 no.12
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    • pp.894-897
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    • 2002
  • Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital anomaly that has generally been found incidentally during autopsy or surgery. Sudden death may occur without antecedent symptoms in apparently healthy, asymptomatic patients and hence operation is recommended when the lesion is recognized. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary artery, only a few children with this anomaly have been reported to have undergone surgical treatment. This report describes a 2-year old patient whose diagnosis was made by echocardiography, confirmed by angiocardiography, and successfully corrected by reimplantation of the anomalous coronary artery into the aorta.

Animal Experiments of Heart Transplantation for Complicated Congenital Heart Disease in Neonate (신생아의 복잡심장기형에서 심장이식을 위한 동물실험)

  • 박영환;윤치순;정원석;김명옥;조범구
    • Journal of Chest Surgery
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    • v.32 no.6
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    • pp.504-509
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    • 1999
  • Background: Heart transplantation is considerated for a selected certain group of complicated congenital heart disease in neonates because corrective surgery is very difficult and has high mortality. Precise planning of transplantation is necessary to adequately fit the donor heart to the recipient. Material and Method: We have performed 4 neonatal pig heart transplantations to test the technical feasibility. Experiment 1: The transplantation was performed using the same technique as the adult heart transplantation. Experiment 2: The transplantation for hypoplastic left heart syndrome was simulated as we reconstructed the whole aortic arch with donor aorta. Experiment 3: The heart transplantation was done with radical pulmonary artery reconstruction. Experiment 4: The experiment was performed for a long term survival. Result: Preoperative planning was very important for adequate fitting. All animals could be weaned from cardiopulmonary bypass, however, two animals died due to bleeding at pulmonary artery and left atrium. Conclusion: We concluded that the neonatal heart transplantation can be applied in some complicated Further using animal model is mandatory.

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Arterial Switch Operation in 1140gm LBW Premie Baby with TGA, IVS (1140gm의 미숙아에 대한 동맥치환술)

  • Park Soon Ik;Lee Seung Hyun;Park Jeong-Jun;Kim Young Hwee;Koh Jae Kon;Park In-Sook;Seo Dong-Man
    • Journal of Chest Surgery
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    • v.38 no.11 s.256
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    • pp.773-775
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    • 2005
  • Cardiac surgery in the neonate with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight premie with congenital heart disease continue to challenge the intellectual and technical skills of those who care for them. We report a case of successful arterial switch operation in 1140g premie with TGA, IVS after 4 week care 1317gm.

Complete Repair of Coarctation of the Aorta and a Ventricular Septal Defect in a Low Birth Weight Neonate (저체중 미숙아에서 시행만 심실중격결손을 동반한 대동맥축착증 완전 교정술)

  • Kwak, Jae-Gun;Jun, Jae-Hyun;Yoo, Jae-Suk;Kim, Woong-Han
    • Journal of Chest Surgery
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    • v.41 no.4
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    • pp.480-483
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    • 2008
  • Even though some authors have reported on the advantages of early total correction of complex heart disease, for low birth weight premature neonates, most surgeons prefer a multi-step approach to early total correction due to the many problems, such as the technical problems, the cardiopulmonary bypass management and etc. We report here on a successful case of early one-stage total repair of coarctation of the aorta and a ventricular septal defect in a 1,250 gram premature neonate.

An analysis of change in pulmonary arterial pressure and mixed venous oxygen tension after correction of congenital heart disease associated with pulmonary hypertension (고폐동맥압을 동반한 선천성 심기형 환자에서 술후 폐동맥압과 혼합정맥혈 산소분압의 변화에 관한 분석)

  • Kim, Gi-Bong;Kim, Yong-Jin
    • Journal of Chest Surgery
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    • v.22 no.6
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    • pp.894-900
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    • 1989
  • It has been suggested that mixed venous $O_{2}$ tension is a predicor of cardiac output especially in a critically ill patient after an open heart surgery. From April 1988 through September 1989, we monitored mixed venous $O_{2}$ tension and pulmonary arterial pressure in 48 patients with acyanotic congenital heart disease at postoperative 1 hour, 6 hour, 12 hour, 24 hour, and 48 hour respectively. They were divided into Group I, with severe pulmoary hypertension, and Group II, without severe pulmonary hypertension. In Group I, mixed venous $O_{2}$ tension and cardiac index showed significant increase with time (p<0.05), but the ratio of pulmonary-aortic systolic pressure didn't show significant change. The increase was significant only 24 hour after operation, and so this low cardiac performance in early postoperative period should be considered when postoperative management is being planned in the risky patient. In Group II, all of the three variables didn't show any significant change with time. The correlation coefficient between mixed venous $O_{2}$ tension and cardiac index was significantly different from zero in both Group I (p<0.001) and group II (p<0.05) at each imeperiod, but the ratio of pulmonary-aortic systolic pressure didn't correlated well with the other 2 variables. Our study showed that serial determination of mixed venous $O_{2}$ tension in acyanotic congenital heart disease could be used as a guide in estimating the cardiac index postoperatively.

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