• Journal of Chest Surgery
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• 제7권1호
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• pp.101-108
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• 1974

Repair of incomplete endocardial cushion defect was accomplished in two cases in September 1973 in this department. In each case a low atrial septal defect and a cleft of mitral septal cusp were found. Interrupted suture repair on the cleft mitral cusp and closure of the atrial septal defect with dacron patch were done successfully. Rigg-Kyvsgaard bubble oxygynator and Sigma motor were utilized for extracorporeal circulation. Follow up study, six months after discharge, revealed satisfactory results.

• Journal of Chest Surgery
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• 제13권3호
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• pp.237-242
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• 1980

This is a case report of spontaneous regression of elevated pulmonary vascular resistance after the age of 6 years in a patient with partial endocardial cushion defect. The patient was first evaluated and considered to be highly risky for surgical correction because of obstructive type of pulmonary hypertension and presisting congestive heart failure at the age of 6 years, during which time medical treatment for congestive heart failure and sufferring from respiratory infection were only provided. Finally the patient was reevaluated at the age of 16 years, in January of 1980 when cardiac catheterization was revealed markedly reduced pulmonary vascular resistance and pulmonary hypertension as well. The patient was operated upon with uneventful postoperative recovery. So we report this case with review of the literatures regarding natural history regarding natural history of partial endocardial cushion defect.

• Journal of Chest Surgery
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• 제39권8호
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• pp.633-636
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• 2006

부분 심내막상 결손증은 일차공 결손과 승모판 전엽에 열공을 동반하는 질환으로 완전형 심내막상 결손증에 비해 임상 증상이 심하지 않으나, 승모판 부전이 진행할수록 심비대, 심부전, 폐동맥 고혈압 등이 더욱 쉽게 발현되어 그 예후가 좋지 않고, 따라서 고령에서는 보기 힘든 질환이다. 본원으로 30년 전부터 증상이 있던 67세 부분 심내막상 결손증 환자가 내원하였다. 심도자 검사상 폐동맥압이 45/22 mmHg, 평균 압력은 32 mmHg로 약간의 폐동맥 고혈압이 있었고, 수술 소견상 $3.5{\times}2.5\;cm$ 크기의 일차공 결손과 심한 석회화를 동반한 승모판 전엽의 열공이 보여 일차공 결손 첨포 봉합과 승모판막 치환술을 시행하였다. 수술 후 11일째부터 심한 방설 차단이 발생하여, 영구 심박동기를 삽입하였고, 수술 27일 후 퇴원하였다. 저자들은 임상적으로 보기 드문 고령에서의 부분 심내막상 결손증을 수술적으로 교정하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제17권4호
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• pp.657-665
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• 1984

Thirty-seven patients had undergone repair of a endocardial cushion defect between 1977 and Aug. 1983 in Seoul National University Hospital. Twenty eight had a partial defect, one intermediate defect and eight complete endocardial cushion defect. Tricuspid cleft was found in 4 cases and mitral cleft was in all p-ECD. Seven patients were of type C anatomy in c-ECD. Four patients had associated major anomalies, including three TOF in c-ECD, one coarctation in p- ECD. In p-ECD patients, the septal defect was closed with patch in all cases and the atrioventricular valvular insufficiency was corrected with MVR in 4 cases, TVR in 1 case and simple interrupted sutures in remainders. In c-ECD patients the septal defect was closed with single patch except one case. The atrioventricular valve was repaired with simple interrupted sutures except one MVR and TVR case. The operative mortality was 14.2% in p-ECD, 44.4% in c-ECD, but recent 3 years [1980-1983] mortality was 8.7% in p-ECD, 20% in c-ECD. More than grade III systolic regurgitant murmur was oted postoperatively in 4 cases of c-ECD and 3 cases of p-ECD. The operative risk factors were preoperative NYHA classification, cyanosis, Rp/Rs, systolic pressure of main pulmonary artery and the degree of regurgitation of atrioventricular valves. The causes of death were low cardiac output syndromes, pulmonary complications and arrhythmias.

• Journal of Chest Surgery
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• 제27권3호
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• pp.196-201
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• 1994

Endocardial cushion defect is a rare congenital heart disease. From September 1985, we experienced 20 cases of endocardial cushion defects and postoperative follow up was taken.We reviewed preoperative clinical data, echocardiography, cardiac cath data, operative method & time. After operation, we performed echocardiography and examined mitral valve function & integrity of patch closure sites. Postoperative follow up was taken regularly & follow up period was from 2 month to 91 months. Several postoperative complications were overcome with adequate treatment. There were 3 cases of early death and mortality rate was 15% [3/20].

• Journal of Chest Surgery
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• 제17권2호
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• pp.244-249
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• 1984

Surgical treatment of partial endocardial cushion defect was accomplished in Feb. 1984 in this department. The 5 year old male patient had history of frequent upper respiratory tract infection and since his age of 3 years dyspnea on exertion and palpitation were noted but there were no cyanosis and clubbing. A thrill was palpable on the apex and grade IV/IV harsh systolic ejection murmur and diastolic murmur was audible on it. Liver was palpable about 3 finger breadths and no ascites. Chest X-ray revealed increased pulmonary vascularity, moderate cardiomegaly [C-T ratio; 0.69], and enlarged left atrium. EKG showed first degree heart block, RVH, LVH, and LAD. Echocardiogram showed paradoxical ventricular septal movement and abnormal diastolic movement of the anterior leaflet of mitral valve. Right heart catheterization resulted left to right shunt [Qp:Qs:2.1:1 ] and moderate pulmonary hypertension [60/40 mmHg]. Left ventriculogram showed mitral regurgitation [Grade III/IV] and filling of left atrium and right atrium nearly same time. Operative findings were: 1.Primum type atrial septal defect [3x2 cm] 2.Cleft on the anterior leaflet of mitral valve. 3.No interventricular communication and cleft of tricuspid valve leaflet. The mitral cleft was repaired with 4 interrupted sutures. The primum type atrial septal defect was closed with Dacron patch intermittently at endocardial cushion and continuously remainder. The post operative course was uneventful and discharged on 22nd postoperative day in good general conditions.

• Journal of Chest Surgery
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• 제18권2호
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• pp.283-287
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• 1985

Seven patients had undergone repair of endocardial cushion defect from Jan. 1977 to Dec. 1984 at National Medical Center. Most patients had no associated anomalies except one who had PFO, and mortality case was absent. Five patients had partial ECD and two had complete ECD [Rastelli type A]. In P-ECD patients, the atrial septal defect was closed with patch in all cases and mitral cleft was approximated with 2-3 direct stitches. In two cases of C-ECD, atrial and ventricular septal defect was closed with single patch in one case and atrial septal defect was closed with patch but ventricular septal defect was closed with patch but ventricular septal defect was closed it direct suture in the other case. Atrioventricular cleft was approximated with 2-3 direct sutures. Postop. transient A-V block was noted in 2 cases but returned to regular sinus rhythm after 2 to 6 months.

• Journal of Chest Surgery
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• 제21권3호
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• pp.574-582
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• 1988

Endocardial cushion defects is a rare congenital heart disease. We experienced two complete endocardial cushion defects[ECD] and three partial ones, which were successfully repaired between 1986 and 1987. In a patient of complete ECD, associated with secundum ASD, Pulmonary stenosis and Down`s syndrome, the atrial and ventricular septal defects were closed separately with bovine pericardium and Dacron patches respectively, and then pulmonary stenosis was relieved by transannular patch widening in addition to valvotomy and infundibulectomy. In another patient with complete ECD, small interventricular communication was closed with simple suture with pledget and primum ASD was closed with pericardial patch. In first patient of partial ECD, primum atrial septal defect was closed with pericardial patch. In second patient of partial ECD, associated with secundum ASD, direct closure of secundum ASD and patch closure of primum ASD were performed. In third patient of partial ECD, associated with patent foramen ovale[PFO], primum ASD was closed with bovine pericardial patch and PFO was closed directly. In all patient except third patient of partial ECD, mitral clefts were closed with three or four 5-0 prolene interrupted sutures. Transient A-V dissociation developed postoperatively in two patients and transient nodal rhythm developed postoperatively in other two patients. Heart failure in complete ECD with Down`s syndrome was overcome with medical treatment.

• Molecules and Cells
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• 제37권5호
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• pp.406-411
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• 2014

The initial step of atrioventricular (AV) valve development involves the deposition of extracellular matrix (ECM) components of the endocardial cushion and the endocardialmesenchymal transition. While the appropriately regulated expression of the major ECM components, Versican and Hyaluronan, that form the endocardial cushion is important for heart valve development, the underlying mechanism that regulates ECM gene expression remains unclear. We found that zebrafish crip2 expression is restricted to a subset of cells in the AV canal (AVC) endocardium at 55 hours post-fertilization (hpf). Knockdown of crip2 induced a heart-looping defect in zebrafish embryos, although the development of cardiac chambers appeared to be normal. In the AVC of Crip2-deficient embryos, the expression of both versican a and hyaluronan synthase 2 (has2) was highly upregulated, but the expression of bone morphogenetic protein 4 (bmp4) and T-box 2b (tbx2b) in the myocardium and of notch1b in the endocardium in the AVC did not change. Taken together, these results indicate that crip2 plays an important role in AV valve development by downregulating the expression of ECM components in the endocardial cushion.

• 한국임상수의학회지
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• 제35권6호
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• pp.286-289
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• 2018

A 4-year-old castrated male Maltese was referred to our hospital with heart murmur. The client did not recognize any symptoms. Auscultation revealed systolic murmur located at the left heart base. Radiographs showed right-sided cardiomegaly with the main pulmonary artery bulging and enlargement of pulmonary vessels. Echocardiogram revealed a small ventricular septal defect and a large atrial septal defect. There was also a common atrioventricular valve. Based on diagnostic imaging studies, the dog was diagnosed with transitional atrioventricular septal defect.