• Journal of Chest Surgery
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• 제17권1호
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• pp.118-124
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• 1984

Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

• Journal of Chest Surgery
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• 제34권11호
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• pp.854-857
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• 2001

양대혈관 좌심실기시는 양대혈관이 좌심실에서 기시하는 드문 선천성 심장기형으로 정확하게 진단하기가 어렵다. 환아는 생 후 2개월에 대동맥축착교정술 및 폐동맥교약술을 받았던 3세된 남아로 대동맥하 심실중격결손, 단일관상동맥을 동반한 양대혈관 좌심실기시로 진단되어 심실중격결손의 첩포봉합, 난원공개존의 봉합 폐동맥 교약부 절제, 그리고 폐동맥간을 좌심실에서 분리 후 우심실로 전위시켜 우심실 유출로 재건술을 받았다. 본 술식은 술 후 좋은 혈역학적 특성을 보였고, 자가조직을 사용함으로써 전위된 폐동맥의 성장잠재성과 판막 기능의 보존으로 술 후 폐동맥 폐쇄부전과 관련된 우심실기능부전의 가능성이 줄어드는 장점이 예상되므로 문헌 고찰과 함께 보고한다.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.610-614
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• 2003

폐고혈압의 원인은 크게 심박출량의 증가나 혈관 수축 또는 폐쇄에 의한 폐혈관저항의 증가로 볼 수 있고 수동적으로도 높아질 수 있다. 다른 심기형이나 혈관기형을 동반하지 않고 개별 정맥의 협착으로 인해 유발되는 경우는 매우 드물다. 저자들은 출생시 식도 기관루 제거와 식도 연결을 시술한 환아에서 반복되는 청색증과 호흡곤란으로 일반외과 3회 입원 후 식도 협착으로 풍선 확장술을 시행하였던 10개월 영아에서 심초음파를 통해 폐동맥 고혈압을 진단하고 심혈관도자술을 통해 개별 폐정맥들의 협착을 원인으로 밝혔기에 보고하고자 한다.

• Journal of Chest Surgery
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• 제12권2호
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• pp.119-126
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• 1979

This is one case report of the extremely rare congenital cardiac malformation, Double-outlet of left ventricle in corrected transposition of great arteries. 11-year-old boy complained acrocyanosis and exertional dyspnea, the parents noticed cyanosis since birth. Physical examination revealed acrocyanosis, clubbed fingers and toes, G-III pansystolic murmur on 2nd and 3rd ICS, LSB. Right heart catheterization revealed significant $O_2$ jump in ventricular level. Right and left ventriculography showed the both catheters arriving in the same ventricle i.e. anterior chamber, morphological left ventricle was in right and anterior position, simultaneous visualization of aorta and pulmonary artery and aorta locating anterior and right side of pulmonary artery. Echo cardiogram surely disclosed interventricular septum. Conclusively it was clarified that the patient has Double-outlet of left ventricle and corrected transposition of great arteries [S.L.D.]. Operation was performed to correct the anomalies under extracorporeal circulation with intermittent moderate hypothermia. Right-sided ventriculotomy disclosed the following findings. 1. Right-sided ventricle was morphological left ventricle. 2. Left-sided ventricle was morphological right ventricle. 3. Right side atrioventricular valve was bicuspid. 4. Left side atrioventricular valve was tricuspid. 5. Aortic valve was superior, anterior and right side of pulmonary valve. 6. Subpulmonary membranous stenosis. 7. Non-committed ventricular septal defect. We made a tunnel between VSD and aorta with Teflon patch so that arterial blood comes through VSD and the tunnel into aorta. After correction the patient needed assisted circulation for 135 min. to have adequate blood pressure. Postoperatively by any means, adequate blood pressure could not be maintained and expired in the evening of operation day.

• Advances in pediatric surgery
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• 제14권1호
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• pp.1-11
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• 2008

The aim of this study is to analyze the outcomes of the esophageal atresia with tracheoesophageal fistula over the last 2 decades. The records of 51 patients born between 1987 and 2006 were reviewed. Twenty-seven patients were male. Mean values of the age, gestational age and birth weight were 2.9 days, 296 days and 2.7kg, respectively. All patients had Gross type C anomalies. Thirty-one patients (60.7 %) had one or more associated congenital anomalies and the most common anomaly was cardiac malformation. In 48 cases, primary anastomosis was done and staged operation was done in one case. Circular myotomies in the proximal esophagus were performed in 9 cases. Postoperative complication developed in 26 cases (54 %): pulmonary complication in 12 cases, anastomotic leakage in 10 and anastomotic stricture in 10, recurrent trachoesophageal fistula in one and tracheomalasia in 2 cases. Reoperation was carried out in 2 patients with anastomotic leaks, the remaining leaks were managed non operatively. Three of the strictures were reoperated upon and the others were successfully managed by balloon dilatations. Overall mortality rate was 15.6 %. Mortality rate of the second 10 years (8 %) period decreased significantly compared to that of the first 10 years (23 %) period.

• 한국동물생명공학회지
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• 제36권1호
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• pp.17-24
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• 2021

In this study, the potential toxicity of isoprocarb was demonstrated using zebrafish embryos. We treated isoprocarb (0, 29, and 58 mg/L) to the zebrafish embryos for 72 h then, we estimated morphological changes and apoptotic cell numbers. The increasing extent of apoptosis from the anterior to posterior region of developing zebrafish larvae was correlated with toxicity in the overall development process, including growth and normal organ formation. The appearance of abnormalities in the isoprocarb-treated groups in comparison to normal developing zebrafish larvae was verified using quantitative image analysis based on ImageJ software program. The vascular system comprising a complex interconnection of blood vessels was visualized in vessel-fluorescent transgenic zebrafish (fli1:eGFP). The main vasculature was malformed on isoprocarb treatment, and this was also related to cardiac defects. Taken together, normal embryonic development in zebrafish was interrupted owing to the acute toxicity of isoprocarb.

• Neonatal Medicine
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• 제18권2호
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• pp.395-398
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• 2011

굴기능부전증후군은 매우 드물지만 신생아에서도 발생할 수 있으며 소아 환자의 경우 대부분 선천심장병이 있거나 심장수술 후에 발생한다. 그러나 이와 같은 심질환의 선행 요인이 없는 신생아에서도 발생할 수 있기 때문에 이에 대한 인지가 필요하다. 굴기능부전증후군은 대부분 특발성으로 발생하며 증상도 다양하고 24시간 심전도 검사상에서도 다양한 형태로 관찰된다. 본 증례에서는 초극소저체중출생아에서 선천심장병 없이 발생한 굴기능부전증후군에 대해 보고하였다. 환아는 심전도상 굴느린맥과 함께 심방세동이 동반된 느린맥과 빠른맥이 반복되어 관찰되었으며 무호흡, 산소포화도의 저하, 청색증의 징후를 나타냈다. 증상이 있는 굴기능부전증후군의 경우 대부분 심장박동조율기의 삽입이 필요하나 본 환아는 당시 체중 750 g으로 심장박동조율기의 삽입에 실패하여 약물 치료를 시작하였다. Isoproterenol과 atropine으로 치료하면서 환아의 체중이 1,500 g을 넘어서자 증상과 심전도상의 호전을 보였다. 향후 본 환아에서처럼 심장박동조율기의 삽입이 어려운 소아 환자의 경우에 대한 치료 체계에 대해 많은 연구가 필요하다.

• Journal of Chest Surgery
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• 제31권2호
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• pp.216-219
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• 1998

Ebstein 기형은 복잡 심기형중 하나로 치료 또한 비정상적인 삼첨판막의 교정 또는 판막치환 등으로 방법이 다양하다. 이질환의 병리소견은 삼첨판의 중격엽과 후엽이 하방전위되어서 심방화된 심실을 형성하는 것으로 수술적 목표는 삼첨판의 기능이상을 교정하고 심방화된 우심실을 주름성형하는 것이다. 본원에서는 매우 심한 삼첨판막 폐쇄 부전증이 동반된 Carpentier type A의 Ebstein기형으로 진단된 12개월된 여아에 대해 심방화된 우심실을 수직방향으로 주름성형하고 판막을 새로이 형성된 판륜에 재부착시키는 이른바 변형적 Carpentier방법으로 성공적으로 수술하였다. 수술후 환아는 심장의 크기가 매우 줄어들었고 심초음파상 미미한 정도의 삼첨판 폐쇄부전증만 남아 술후 14일째 퇴원하였다.

• Clinical and Experimental Pediatrics
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• 제58권7호
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Journal of Chest Surgery
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• 제30권12호
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• pp.1184-1189
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• 1997

활로 4징증은 청색증이 있는 선천성 심장기형 중에서 가장 흔한 질환이다. 마산삼성병원 흉부외과에서는 1984년 4월부터 1993년 12월 까지 활로 4징증으로 완전교정을 받은 39례를 경험하였다. 그 관찰 결과는 다음과 같다. 남자가 14명, 여자가 25명이며 연령은 최소 2세에서 최고 36세로 평균 13.33$\pm$7.40세이었다. 심실중격결손증은 제 1형이 10례(25.6%), 제 2형이 19례(48.8%), 제 1형과 제 2형이 복합된 경우 10례 (25.6%)이었다. 우심실 유출로 폐쇄는 누두부와 폐동맥 판막이 함께 협착이 있는 경우가 19례(48.7%)로 가장 많고, 누두부 협착과 폐동맥 판막 및 판륜 협착이 함께 있는 경우 10례(25.6%), 누푸부 협착만 있는 경우 1례, 폐동맥 판막 협착만 있는 경우가 1례 있었다. 우심실 유출로 재건술은 첨포확장술이 33례(84.6%)에서 필요했고, 이중에 단일 판첨을 내재한 첨포를 사용한 2례를 포함하여 10례에서 경판륜 첨포확장술을 시행하였다. 수술후 36명의 생존자중 23례에서 2 dimensison 및 도플러 심에코를 시행하였다. 가장 흔한 합병증은 부정맥이었고 특히, 완전우각차단이 30 례(76.9%)에서 있었다. 수술 사망률은 7.7%이었으며, 사망원인은 저심박출증이었다.