• Journal of Chest Surgery
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• 제16권1호
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• pp.18-29
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• 1983

Thirty-two patients with a cyanotic cardiac malformations having more complex intracardiac defects than ones in a tetralogy of Fallot underwent complete intracardiac repairs in a-full-year period from July 1981 to June 1982. Twenty-two patients [68.8%] died within 30 days after surgery: Transposition of the great arteries, seven of 10 patients; Double-outlet right ventricle, four of 6 patients; Tricuspid atresia, four of 6 patients; Single ventricle, all of 4 patients; Pulmonary atresia, two of 3 patients; Double-outlet left ventricle, none of 2 patients; and Truncus arteriosus, one of a single patient. All deaths occurred with a low cardiac output syndrome or a failed off-bypass, and they were almost always accompanied with other grave postoperative complications. The complex intracardiac anatomy itself was one of the risk factors by making a complete intracardiac repair of the defects difficult in a small heart. The reconstruction of the right ventricular outflow carried a difficulty in balancing an adequate relief of the obstruction with an avoidance of making too much pulmonary valvular insufficiency as well. On the other hand, the presence of an elevated pulmonary arterial pressure and a high pulmonary vascular resistance was also the factors affecting the postoperative surviv als. The importance of detailed knowledge of intracardiac anatomy and hemodynamics from the careful preoperative evaluation of the patient was discussed along with the necessity of technical refinement of the correction.

• Korean Journal of Radiology
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• 제22권11호
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• pp.1894-1908
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• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• Journal of Chest Surgery
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• 제28권3호
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• pp.237-246
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• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8 kg [mean weight, 4.0 1.4 kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1 $\pm$ 3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients [86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septum was resected whenever necessary. There were seven early deaths [16.8 % , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis, stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily. We concluded that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are reasonable.

• Anatomy and Cell Biology
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• 제51권4호
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• pp.266-273
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• 2018

The ganglion cardiacum or juxtaductal body is situated along the left recurrent laryngeal nerve in the aortic window and is an extremely large component of the cardiac nerve plexus. This study was performed to describe the morphologies of the ganglion cardiacum or juxtaductal body in human fetuses and to compare characteristics with intracardiac ganglion. Ganglia were immunostained in specimens from five fetuses of gestational age 12-16 weeks and seven fetuses of gestational age 28-34 weeks. Many ganglion cells in the ganglia were positive for tyrosine hydroxylase (TH; sympathetic nerve marker) and chromogranin A, while a few neurons were positive for neuronal nitric oxide synthase (NOS; parasympathetic nerve marker) or calretinin. Another ganglion at the base of the ascending aorta carried almost the same neuronal populations, whereas a ganglion along the left common cardinal vein contained neurons positive for chromogranin A and NOS but no or few TH-positive neurons, suggesting a site-dependent difference in composite neurons. Mixtures of sympathetic and parasympathetic neurons within a single ganglion are consistent with the morphology of the cranial base and pelvic ganglia. Most of the intracardiac neurons are likely to have a non-adrenergic non-cholinergic phenotype, whereas fewer neurons have a dual cholinergic/noradrenergic phenotype. However, there was no evidence showing that chromogranin A- and/or calretinin-positive cardiac neurons corresponded to these specific phenotypes. The present study suggested that the ganglion cardiacum was composed of a mixture of sympathetic and parasympathetic neurons, which were characterized the site-dependent differences in and near the heart.

• Journal of Chest Surgery
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• 제28권7호
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• pp.658-665
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• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8kg[mean weight, 4.0$\pm$l.4kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1$\pm$3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients[86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septurn was resected whenever necessary. There were seven early deaths[16.8% , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis,stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily.We conclude that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are resonable.

• Journal of Chest Surgery
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• 제16권4호
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• pp.431-443
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• 1983

A hundred and fifty-one patients with congenital heart disease less than 24 months old underwent intracardiac repairs from January 1982 to July 1983, which consists 24.2% of all the patients with congenital heart diseases operated during the same period. There were 98 patients[64.9%] with acyanotic congenital heart disease and 53 patients[35.1%] with cyanotic congenital heart disease, and 55 patients[36.4%] were less than 1 year of age. Twenty-two patients died within 30 days after surgery and 3 patients died after postoperative 30th day: Ventricular septal defect, four of 90 patients; Tetralogy of Fallot, five of 23 patients; Transposition of great arteries, nine of 17 patients; Tricuspid atresia, four of 5 patients; Pulmonary atresia, all of 2 patients; Single ventricle, one of single patients. Over all mortality was 16.6% and mortality of acyanotic congenital heart disease, cyanotic congenital heart disease and patients less then 1 year of age was 4.1%, 39.6% and 20.0% respectively. Still the mortality of cyanotic congenital heart disease is high. Careful preoperative evaluation of the detailed intracardiac anatomy and hemodynamics of the patients and proper selection of surgical treatment yield better clinical results.

• Journal of Chest Surgery
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• 제56권6호
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• pp.456-459
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• 2023

Penetrating chest trauma may result in significant intracardiac injury. A traumatic ventricular septal defect is a rare complication that requires surgical management, particularly if heart failure ensues. We report a case of delayed repair of an outlet-type ventricular septal defect and perforation of the aortic and pulmonary valve leaflets following a stab wound. This report highlights diagnostic and surgical considerations and also presents an opportunity to review the conotruncal anatomy, which may be relatively unfamiliar to many adult cardiac surgeons.

• Applied Microscopy
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• 제22권2호
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• pp.118-126
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• 1992

The pathway and time course of fucose-containing glycoprotein synthesis and intracellular translocation in osteoclasts of the mice maxillary alveolar bone were investigated by electron microscopic radioautography. Male Balb-C mice weighing 17gm were anesthetized with Nembutal and injected via the external jugular vein with 2.5 mCi of $L-[6-^{3}H]-fucose$ (specific activity 16.8 mCi/mmol) in 0.1 ml of sterile saline solution. At 5, 10, 20, 35 minutes and 8 hours after administration of the $^{3}H-fucose$, animals were killed by intracardiac perfusion of 30ml of 2% glutaraldehyde in a modified Tyroid solution, pH 7.4. The maxillae were then removed and further fixed in Karnovsky fixative for an additional 3-4 hours. After rinsing in 0.1M cacodylate buffer for 10 minutes, the maxillae were demineralized for 2 weeks at $4^{\circ}C$ in ethylene diamine tetra acetate containing 2% glutaraldehyde. The first interdental areas were mesiodistally sectioned into slices of 1mm thickness and postfixed in osmium tetroxide. Tissues were then dehydrated and embedded in Poly Bed. To prepare electron microscopic radioautography, the dipping method of Kopriwa (1973) was employed. Thin sections were coated with a crystalline monolayer of ILford $L_4$ photographic emulsion. After exposure for 4 months at $4^{\circ}C$, the sections were developed Kodak Microdol-X and Phenidon (for compact grains), fixed in 30% sodium thiosulfate, stained with uranyl acetate and lead citrate and examined in the electron microscope (JEOL 1200 EX). At 5, 10 and 20 minutes after injection, $^{3}H-fucose$ was concentrated in Golgi cisternae of the osteoblasts. By 35 minutes the labels were observed over small vesicles in the suprannclear area of osteoclasts. At 8 hours, numerous silver grains were located on the ruffled border and cell membrane of osteoclasts. These results indicate that fucose molecules are added in the Golgi apparatus and small vesicles appear to be responsible for translocation of the glycoproteins to the marginal portion of osteoblasts. The glycoproteins are distributed on the osteoclast cell surface and especially over the ruffled border.

• Journal of Chest Surgery
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• 제32권7호
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• pp.628-636
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• 1999

배경: 최근에 완전방실중격결손의 해부학적 구조에 대한 이해가 증가됨에 따라서 본원에서는 수술방법에 있 어서 여러 가지 변형을 시도하게 되었다. 대상 및 방법: 1997년 5월부터 1998년 7월까지 8명의 완전방실중 격결손 환자를 수술하였다. 남녀 각각 3명, 5명이었으며 나이는 2개월에서 28개월까지 분포하였고 평균 몸무 게는 6.0$\pm$2.2 Kg이었다. 심장의 해부학적 구조에 따라서 3명의 환아에서는 심실중격결손을 일차적으로 봉합 하여 수술을 단순화하였다(Group I). 좌심실유출로 협착의 가능성이 있는 2명의 경우에는 통상적인 방법대로 2개의 포편을 이용하였다(Group II). 그리고 불균형 완전방실중격결손의 경우에는 심실중격결손을 막는 포편 을 우심실에 치우쳐서 막고 심방중격을 새로 만들거나 좌측 방실판막의 부족한 판막부위를 자가 심낭막으로 메꾸어 주거나 좌측 방실판막이 parachute valve 인 경우에는 2개의 판막입구를 만들어 주었다(Group III). 결과: 모든 환자가 수술후 만족스러운 혈류역학을 보였으며 7명에서는 평균 3$\pm$1 일만에 인공호흡기 이탈이 가능하였으며 1명의 환자는 심장의 문제없이 폐질환으로 24일만에 인공호흡기를 이탈하였다. 수술후 합병증 으로 III군에서 판막성형을 시행한 좌측 판막이 찢어지면서 판막역류가 점진적으로 심해진 1명에서 2주만에 재수술을 하였다. 조기 및 만기사망은 없었으며 수술후 심장초음파검사에서 약간의 좌측 방실판막협착이 III 군에서 1명 있었으며(평균압력차 6.5 mmHg), 좌측 방실판막역류가 7명(87.5 %)의 환아에서 Grade I 이하였으 며, 우측 방실판막역류는 모든 환아에서 Grade I 이하였다. 결론: 완전방실중격결손의 수술시 해부학적인 구 조에 따라서 선택적으로 일부 환아에서는 심실중격결손을 일차적으로 봉합함으로써 수술을 단순화할 수 있 으며 우심실이 발달된 불균형 완전방실중격결손에서도 변형수술방법을 통하여 양심실성 교정을 하여 좋은 결과를 얻을 수 있었다.

• Journal of Chest Surgery
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• 제30권9호
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• pp.862-868
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• 1997

서울대학교병원 흉부외과에서는 1985년부터 1994년까지 총 9례의 숀 증후군 환자를 치험하였다. 남자 환 아가 떼이었고 여자 환아가 1례이었다. 첫 수술을 시행한 연령은 깨월에서 11세까지로 평균 연령은 33.0 $\pm$ 31.0 개월이었다. 선천성 승모판 협착증 및 대동맥 축착증은 모든 환아에서 존재하였고 승모판 상부 링 및 대동맥하 협착은 각각 4례의 환아에서 발견되었다. 2례의 환아에 있어서는 숀 증후군의 4가지 병변 모두를 가지고 있었다. 3례의 환아에 있어서는 한번에 완전 해부학적 교정술을 시행하였으며 나머지 2례의 환아에 있어서는 개흉술 하에 대동맥 축착증을 교정하고 나중에 정중 흉골 절개 하에 나머지 병변을 교정하는 단계적 수술을 시행하였다. 9례의 환아에 있어서 총 45개의 수술적 치료를 시행하여 1례당 평균 수술적 치료 는 5.0 $\pm$ 0.7개이었다 세번째 수술을 시행한 환아는 2례이었으며 각각 지속적인 승모판 협착으로 승모판 치 환술을 시행한 환아와 지속적인 대동맥 축착으로 대동맥 축착 성형술을 시행한 환아이었다. 한번에 완전 교정술을 시행한 환아와 1차 단계적 수술을 시행한 환아에 있어서 수술 사망률은 없었으나 2차 단계 \ulcorner수술을 시행한 6례중 2례가 사망하여 수술 사망률은 22.2%이었으며 사망한 2례 모두 1차 대동칵 축착증 교정술후 계속되는 심부전 및 인공 호흡기 이탈이 되지 않아 각각 1차 수술 후 9일과 15일 후에 완전 교정술을 시행했던 환아들로 모두 좌심실 형성부전에 의한 심부전으로 사망하였다. 세번째 수술시 사망한 환아는 없었다. 생존한 7례 모두 11개월에서 12년까지(평균 6.7 $\pm$ 3.6년)장기 추적 조사하였으며 모두 뉴욕 심장 협회 활 동도 1군에 속해 있었으며 판막 치환술로 인하여 쿠마딘을 복용하고 있는 환아와 마지막 추적조사시 흉부 X선 사진상 약간의 심비대가 있어 디곡신을 복용하고 있는 환아를 제외하고는 어떤 약도 복용하고 있지 않았 다 결론적으로 숀 증후군은 수술 사망률 및 이환율이 높은 질환이긴 하나 각각의 해부학적 병변의 특징을 잘 파악하여 적절한 치료를 한다면 좋은 장기성적을 기대할 수 있다는 것이다.