• Title/Summary/Keyword: Congenital defect

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Noonan Syndrome with Double-Chambered Right Ventricle and Atrial Septal Defect -1 Case Report- (Noonan 증후군에 동반된 DCRV와 심방중격결손증 -1례 보고-)

  • Park, Young-Woo;Lee, Seock-Yeol;Jeong, Yoon-Seop;Youm, Wook
    • Journal of Chest Surgery
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    • v.33 no.5
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    • pp.419-421
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    • 2000
  • Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

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Clinical analysis of ostium secundum atrial septal defect (이차공형 심방중격결손증의 임상적 고찰)

  • 이종태
    • Journal of Chest Surgery
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    • v.17 no.4
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    • pp.607-613
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    • 1984
  • Thirty seven patients with ostium secundum atrial septal defect, operated from January, 1976 to September, 1984 at the Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital, were given clinical assessment. The following results were obtained. 1.Ostium secundum atrial septal defect was comprised of 18% of congenital heart disease. Their mean age was 15.7\ulcorner.42. Sex ratio [male:female] was 1:1.1. 2.Most frequent clinical symptom was dyspnea on exertion occurred in 26 patients [76.5%]. Only one patient had no symptom [2.9%]. 3.Pre-operative EKG findings revealed RVH in 61.8%, ICRBBB in 29.4%, and RAD in 41.2%. 4.Mean value of systolic pulmonary arterial pressure in patients over 20 years old was 37.8\ulcorner4.4mmHg and it was 28.1\ulcorner10.2mmHg in patients under 20 years old, but the difference between two groups was not statistically significant. 5. In large defect group [>3cm in diameter], Qp/Qs was significantly increased than small defect group [<3cm in diameter], but systolic pulmonary arterial pressure and Rp/Rs were not different between two groups. 6. Overall operative mortality was 5.4%.

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Congenitally Corrected Transposition of the Great Arteries [SLL] with Pulmonary Atresia, Ventricular Septal Defect,Patent Ductus Arteriosus, and Atrial Septal Defect -One case report- (폐동맥 폐쇄, 심실중격 결손, 동맥관 개존 및 심방중격 결손을 동반한 선천성 교정형 대동맥 전위증의 치험)

  • Kim, Yeong-Hak;Ji, Haeng-Ok
    • Journal of Chest Surgery
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    • v.23 no.5
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    • pp.953-961
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    • 1990
  • Congenitally corrected transposition of the great arteries is a rare congenital heart anomaly, in isolation, has no hemodynamic consequences. It is usually associated with one or more of a variety of intracardiac lesions, ventricular septal defect, valvular or subvalvular pulmonary stenosis, and deformity of the systemic atrioventricular valve with insufficiency. This report describes a successful two stage operation for congenitally corrected transposition, [SLL] type, with ventricular septal defect, pulmonary atresia, persistent ductus arteriosus, and atrial septal defect. A 9 years old patient underwent modified Blalock-Taussig operation because of severe pulmonary hypoplasia. 2 years later a corrective operation, direct closure of ASD and PDA, VSD closure with Dacron patch, Enlargement of left pulmonary artery with pericardial patch and Relief of ROTO with Rastelli procedure could be successfully performed without complication.

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Clinical Study of 156 Cases of Secundum Atrial Septal Defect closed by Direct Suture (직접봉합으로 치료한 단독 이차공 심방중격결손증 156례 보고)

  • 송정근
    • Journal of Chest Surgery
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    • v.28 no.4
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    • pp.335-339
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    • 1995
  • Secundum atrial septal defect occupies about 10 to 15% of congenital heart diseases, and the surgical accomplishment is outstanding, so that the operative mortality is getting near to zero percent. But, the methods of correction, direct closure versus patch closure are still controversial and there is no absolute method about it. Some surgeons prefer direct closure technique for its simplicity and lesser thrombogenicity but others, afraid of arrhythmia and suture detachment after closure of large defect, prefer patch closure. Usually most surgeons use direct suture technique in small and moderate sized defects and patch closure in large defects. In our hospital, 156 cases of isolated secundum atrial septal defect were closed directly by double continuous over and over suture using 5-0 polypropylene[prolene , regardless their sizes and the amounts of shunt flow. There were no operative mortality and no serious complications such as heart block, suture detachment and embolism.

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Pulmonic stenosis with atrial septal defect in a Siamese cat

  • Kim, Ji-Youn;Lee, Sung-Wook;Lee, Seung-Gon;Suh, Sang-Il;Hyun, Changbaig
    • Korean Journal of Veterinary Research
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    • v.57 no.1
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    • pp.63-66
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    • 2017
  • A 6-month-old mature intact female Siamese cat presented with exertional dyspnea. Diagnostic studies revealed pleural effusion, grade 4/6 left basal systolic murmur, deep S-wave in electrocardiograph leads I, II, and III, cardiomegaly with pleural effusion on radiography, pulmonic systolic (~5.8 m/sec) and tricuspid (3.6 m/sec) regurgitant jets, atrial septal defect, and a hypoplastic right outflow tract. Based on these results, the case was diagnosed as pulmonic stenosis with atrial septal defect. To the best of our knowledge, this is the first case report describing pulmonic stenosis with atrial septal defect in a cat in Korea.

Evaluation of Pulmonary Perfusion Scan in Heart Disease (폐주사(肺走査) - 심장질환(心臟疾患)의 폐관류주사(肺灌流走査) 소견(所見) -)

  • Lee, J.T.;Kim, C.K.;Park, C.Y.;Choi, B.S.
    • The Korean Journal of Nuclear Medicine
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    • v.7 no.2
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    • pp.27-34
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    • 1973
  • Pulmonary perfusion scan with radioactive $^{113m}In$-iron hydroxide particle was performed in the 25 cases of heart disease which had been diagnosed by cardiac catheterization prior to surgery from July, 1972 to July, 1973 at the Department of Radiology and Nuclear Medicine, Yonsei Medical College. It consists of 7 mitral stenosis, 2 mitral insufficiency, 1 aortic insufficiency, 3 atrial septal defect, 5 ventricular septal defect, 2 patent ductus arteriosus, 1 transposition of great vessel and 4 Tetralogy of Fallot. Findings of pulmonary perfusion scan in relation to hemodynamic data of cardiac catheterization were examined. 1) Out of 10 cases of acquired valvular heart disease, In 6 cases of mitral stenosis and 1 case of aortic insufficiency, radioactivity was increased at both upper lung. This finding is noted when pulmonary wedge or venous pressure was elevated above 22 mmHg and arterial systolic pressure above 33 mmHg. 2) Out of 15 cases of congenital heart disease. In almost all cases of atrial septal defect and ventricular septal defect except 2 cases, radioactivity was even at both entire lung. In 2 cases of patent ductus arteriosus, radioactivity was decreased especially at the left lung. It is observed that in acyanotic congenital heart disease, radioactivity of lung is not related with pulmonary arterial pressure. In 3 cases of Tetralogy of Fallot, radioactivity was even at both entire lung and in 2 of them, extrapulmonary radioactivity of liver or kidney which depends on size of defect and volume of right to left shunt reversible, was noted.

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Postoperative Follow up Study in ECD (심내막상 결손증 술후 추적 관찰에 관한 연구)

  • 류지윤
    • Journal of Chest Surgery
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    • v.27 no.3
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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].

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Surgical correction of congenital heart defects in adult (성인 선천성 심장기형의 외과적 치료)

  • 신현종
    • Journal of Chest Surgery
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    • v.22 no.1
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    • pp.95-105
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    • 1989
  • The records of 248 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular malformation during the period of 10 years from August, 1978 to July, 1988 were reviewed. During this period, the incidence of congenital cardiovascular malformation in adult was 18.2% of 1376 total heart disease operated on and 25.5% of 986 congenital heart defects. Among them, there were 200 patients in acyanotic group and 48 patients in cyanotic group. Male versus female ratio was 1:1.28. The oldest patient was 59 years old female who had atrial septal defect. The mean age was 24.4 years old. The distribution of the lesions showed a large preponderance of atrial septal defects [37.19o] followed in frequency by ventricular septal defects [918.1%], patent ductus arteriosus [17.3%], tetralogy of Fallot [16.1%], and a variety of other complex malformations[3.2%]. In the pediatric age group, relative frequency was different from that of this adult group, showing ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus and atrial septal defects in order of incidence. The hospital mortality and late mortality were 6.0% and 1.7% respectively. The causes of hospital death were low cardiac output in 10 patients, arrhythmia in 2, air embolism in 1, sepsis in 1 and respiratory failure in l. Clinical improvement upto NYHA functional class I or II postoperatively has been achieved and sustained in all patients following repair except the patients of late death and receiving reoperation. This result confirms that congenital heart defects in the adults can be corrected with a good outcome and an aggressive operative approach seems justified.

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Surgical Treatment of Atrial Septal Defect: Secundum Type (심방중격결손증의 외과적 요법)

  • 안광필;이영균
    • Journal of Chest Surgery
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    • v.8 no.2
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    • pp.81-88
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    • 1975
  • Up to October 31, 1975, 34 cases of atrial septal defect, secundum type, operated in this department, were presented. This is 23.2% of all congenital heart diseases, operated utilizing cardiopulmonary bypass, in this department during this period [34 out of 146]. Out of 34 cases, 32 cases are pure ostium secundum type and one case is sinus venosus and another one is ostium secundum type with partial anomalous pulmonary venous drainage. Six cases of endocardial cushion defect and 3 cases of trilogy of Fallot are excluded in this report. All 34 cases are repaired under direct vision utilizing extracorporeal circulation. Among 34 cases of atrial septal defect, 16 cases are male, and 18 cases are female. Their ages range between 3 to 48 years, but over 59% of the cases are below the ages of 20 years. Thirty-two cases are repaired by direct sutures while 2 cases are repaired with Teflon patches. The average perfusion time is 30 minutes; the shortest 12 and the longest 81 minutes. The number of the defect is single in 31 cases, double in 2 cases, triple in one case. But the associated defect except the main defect are so small as can be closed by simple direct suture. The size of the defect is average $12cm^2$; the smallest 0.7 and the largest $25cm^2$. The surgical mortality is 2 cases [5.6%] and other cases are found to be excellent in the follow up studies.

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Coexistent Atrial Septal Defect with Mitral Valve Disease: a report of 6 cases (승모판막질환을 합병한 심방중격결손증의 수술요법6례 보고)

  • Jo, Sung-Rae;Chae, Hurn;Rho, Joon-Rhyang
    • Journal of Chest Surgery
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    • v.12 no.4
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    • pp.339-345
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    • 1979
  • Mitral valve disease is the most common disease of the acquired heart diseases, and atrial septal defect is also one of common congenital heart diseases. Coexistence of these two lesions is rare, but of great hemodynamic interest. Among 443 cases with mitral valve disease and 90 cases with atrial septal defect experienced in the Department of Thoracic Surgery, Seoul National University Hospital, there were 6 cases with atrial septal defect complicated by mitral valve disease. 1. Of the 6 patients, four were female and two were male. The age was ranged from 18 to 46. 2. Atrial septal defect was ostium secundum type in all cases, and the mitral valvular lesions were regurgitation in four and stenosis in two. Type II ventricular septal defect was also combined in one of the cases. 3. The atrial septal defect was corrected by, primary closure and the mitral valve was replaced with the prosthetic or bioprosthetic valve in all cases. The combined ventricular septal defect was closed using Teflon felt patch. 4. The operative result was good except in one who was expired of bacterial endocarditis 4 months after hospital discharge.

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