• Title/Summary/Keyword: congenital disease

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Clinical Experience of Open Heart Surgery under Extracorporeal Circulation -Review of Operation 131 Cases- (개심술에 의한 심질환의 외과적 치료 -131 례 수술경험-)

  • 유회성
    • Journal of Chest Surgery
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    • v.13 no.4
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    • pp.394-404
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    • 1980
  • During the period of June 1976 October 1980 131 cases of Open heart Surgery was performed at the National Medical Center in Seoul under the extracorpocal circulation. 77 cases were congenital heart disease and 54 were acquired heart disease. The age of the patients ranged between 2$\frac{1}{2}$ and 51 years. For all patients partial hemodilution technique and moderte hypothermia was used during extracorporeal circulation and cardioplegia was done for myocardial protection since April 1978. 41 of congenital cases were non-cyanotic group and 1 case died. 36 of congenital cases were cyanotic group and revealed very high mortiality rate (16 death, 39%). 53 of acquired cases were cases of valvular heart disease, 34 mitral (3 death), 1 aortic, 4 mitral with aortic 12 mitral with tricuspid (3 death), 2 triple valves (2 death), and revealed mortality rate of 15.1% (8 death). 1 of acquired cases were left atrial myxoma. There were 25 cases of operative death and over all motality rate was 19.1%.

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The Clinical Experience of 500 Cases in Open Heart Surgery (개심술 500례의 임상적 고찰)

  • 민용일
    • Journal of Chest Surgery
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    • v.20 no.1
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    • pp.148-155
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    • 1987
  • From May 1977 to June 1986, 500 cases of open heart surgery were performed under the cardiopulmonary bypass. There were 278 male and 222 female patients ranging from 6 months to 69 years. 363 cases[73.6%] were congenital heart diseases, and 137 cases[27.4%] were acquired heart diseases, which were 33 valvular diseases, 3 myxomas and 1 IVC obstruction. There were 363 congenital heart anomaly with 35 operative deaths[9.6%], consisting of 279 acyanotic cases with 12 deaths [4.3%] and 85 cases of cyanotic cases with 23 deaths[28.4%]. In 133 patients of acquired valvular disease, 124 valves were implanted and operative death of valvular disease was 12%. Finally the operative mortality was 9.6% in congenital anomaly, and 12.4% in acquired heart disease, overall mortality rate was 10.4%.

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Clinical analysis of 60 cases of open heart surgery (개심술 치험 60례 보)

  • Kim, Su-Seong;Kim, Yeong-Ho;Kim, Gong-Su
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.389-397
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    • 1984
  • Sixty cases of open heart surgery were performed in the Department of Thoracic and Cardiovascular Surgery of Chonbuk National University Hospital from July, 1983 to June, 1984. The patients were consisted of 40 [66%] congenital anomalies containing 26 [43%] patients of acyanotic group and 4 [23%] of cyanotic group, and 20 [34%] acquired heart diseases which involved one or more cardiac valves. The male patients were 42 and the female 18. In 20 valvular heart diseases, open mitral commissurotomy was done in 5 patients, mitral valvular replacement with tissue valve in 6, mitral valvular replacement with mechanical valve in 5, mitral valvular replacement with tricuspid annuloplasty in 2, mitral annuloplasty in 1, and mitral and aortic valvular replacements with mechanical valves in 1. The most frequency complication was low cardiac output syndrome occurred in 9, and the next was urethral stenosis, ARDS, and postoperative bleeding, etc. The perioperative mortality was 21% in congenital cyanotic heart disease, 12% in congenital acyanotic heart disease, and 5% in acquired heart disease.

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Clinical Experience of Open Heart Surgery - 168 cases - (개심술 168례에 관한 임상적 고찰)

  • Youm, Wook;Sung, Sang-Hyun
    • Journal of Chest Surgery
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    • v.21 no.1
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    • pp.48-54
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    • 1988
  • 168 cases of open heart surgery had been performed in Korea Veterans Hospital from Aug. 1984 to Nov. 1987. There were 150 cases of congenital heart disease and 18 cases of acquired heart disease. In congenital heart cases, 123 cases [82%] were acyanotic and 27 cases [18%] were cyanotic. Common congenital defects were VSD, TOF, ASD, PS in order of frequency. There were 11 cases of operative mortality; 5 cases [4%] in acyanotic group, 6 cases [22\ulcorner6] in cyanotic group. There was no mortality in valvular heart disease group. Overall mortality rate was 6.5%.

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Application of CRISPR-Cas9 gene editing for congenital heart disease

  • Seok, Heeyoung;Deng, Rui;Cowan, Douglas B.;Wang, Da-Zhi
    • Clinical and Experimental Pediatrics
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    • v.64 no.6
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    • pp.269-279
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    • 2021
  • Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

Congenital midline cervical cleft: An easily misdiagnosed disease

  • Kang, Byungkwon;Kim, Byungjun
    • Archives of Craniofacial Surgery
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    • v.21 no.6
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    • pp.372-375
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    • 2020
  • Congenital midline cervical cleft is a rare congenital disease. The disease is often misdiagnosed as a branchial cleft deformity, thyroglossal duct cyst, or other skin diseases. It has the following characteristics: skin defect at the midline of the anterior neck, a skin tag at the upper end of the lesion, and a blind sinus tract at the caudal aspect with or without mucoid discharge. Treatment is usually for aesthetic purposes; therefore, early surgical en bloc resection with Z-plasty or W-plasty is recommended to reduce recurrence and scar formation.

Congenital Lobar Emphysema -Report of A Case- (선천성 엽성 폐기종 -1례 보고-)

  • 손제문
    • Journal of Chest Surgery
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    • v.27 no.10
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    • pp.882-884
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    • 1994
  • Congenital lobar emphysema is one of the abnormal development of pulmonary parenchyme which may cause respiratory distress in infant. This disease is an unusual condition barely mentioned before 1949, and only 166 cases were reported till 1967 in English literature. This report describes a 13 months old girl who had a congenital left upper lobar emphysema. The left upper lobectomy was the operation for this case and the postoperative course was not eventful, and have been in good condition upto now.

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Surgical Treatment of Congenital Coronary Arteriovenous Fistula -A report of three cases- (선천성 관동정맥루 -수술치험 3례-)

  • 하현철
    • Journal of Chest Surgery
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    • v.27 no.8
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    • pp.700-704
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    • 1994
  • Congenital coronary arteriovenous fistula is relatively rare congenital heart disease which has formation of fistula between coronary artery and cardiac ventricle, atrium, or pulmonary artery, but that can be repaired with simple surgical procedure. We experienced three cases of congenital coronary arteriovenous fistula, which were repaired surgically under cardiopulmonary bypass, so we report these cases with review of the literatures.

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Congenital Heart Disease: a Pictorial Illustration of Putting Segmental Approach into Practice

  • Yeung, Tse Hang;Park, Eun-Ah;Lee, Ying Cheong;Yoo, Jin Young;Lui, Choi Yu
    • Investigative Magnetic Resonance Imaging
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    • v.19 no.4
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    • pp.205-211
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    • 2015
  • The human heart is a complex organ in which many complicated congenital defects may happen and some of them require surgical intervention. Due to the vast complexity of varied anatomical presentations, establishing an accurate and consistent nomenclature system is utmost important to facilitate effective communication among pediatric cardiologists, cardiothoracic surgeons and radiologists. The Van Praagh segmental approach to the complex congenital heart disease (CHD) was developed in the 1960s and has been used widely as the language for describing complex anatomy of CHD over the decades. It utilizes a systematic and sequential method to describe the cardiac segments and connections which in turn allows accurate, comprehensive and unambiguous description of CHD. It can also be applied to multiple imaging modalities such as echocardiogram, cardiac CT and MRI. The Van Praagh notation demonstrates a group of three letters, with each letter representative for a key embryologic region of cardiac anatomy: the atria, ventricles and great vessels. By using a 3-steps approach, we can evaluate complex CHD precisely and have no difficulties in communicating with other medial colleague. This pictorial essay revisits the logical steps of segmental approach, followed by a pictorial illustration of its application.

Aorto-Right Ventricular Tunnel: An Uncommon Problem with a Common Solution

  • Mitropoulos, Fotios;Kanakis, Meletios A.;Chatzis, Andrew;Kiaffas, Maria;Azariades, Prodromos;Tzifa, Aphrodite
    • Journal of Chest Surgery
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    • v.49 no.4
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    • pp.295-297
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    • 2016
  • Aorto-ventricular tunnel is a rare congenital malformation, and aorto-right ventricular tunnel (ARVT) is an even less common entity. Here, we report the case of a 3-month-old female who underwent successful surgical closure of ARVT. The origin of the right coronary artery was proximal to the ostium of the tunnel.