• Title/Summary/Keyword: Congenital heart diseases

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Open heart surgery in the first two years of life (2 세 이하 유아기의 개심술)

  • Park, Lee-Tae;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.16 no.4
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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.

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Clinical Experience of Open Heart Surgery; 1000 Cases (심장수술 1,000례의 임상적 고찰)

  • 조광현
    • Journal of Chest Surgery
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    • v.26 no.4
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    • pp.282-293
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    • 1993
  • From Sep. 1985 to Dec. 1992, total 1000 cases of open heart surgery [OHS] were performed in the department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College Of Medicine, Inje University.Among the total 1,000 cases of OHS, there were 823 cases with congenital heart diseases [CHD] and 177 cases with acquired heart diseases [AHD]. The age distribution was 9 days [4.0 kg] to 49 years in CHD and 11 to 64 years in AHD, In the 823 cases of CHD, there were 763 acyanotic cases and 60 cyanotic cases. The CHD cases consisted of 520 VSD [63.2 %], 177 ASD [21.5 %], 60 TOF[7.3 %], 27 PS [3.3 %], 17 ECD [2.1%], 7 Valsalva sinus rupture [0.9 %], 4 TGA [0.5 %], 3 Ebstein`s anomaly [0.4%], 3 DORV[0.4%], and others. The corrective operations were applied for congenital heart disease with the result of 2. 8 % hospital mortality. In the 177 AHD, 168 cases were valvular heart diseases, 7 cases were cardiac tumors and one LA thrombus and one annuloaortic ectasia. In the 168 valvular heart diseases, there were 115 single valve replacement cases [16 AVR, 99 MVR], 20 cases of double valve replacement[AVR & MVR], 15 cases of MVR with TVA, and 10 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 199`. In MVR, 66 of St. Jude Medical valves, 78 ofCarpentier-Edward valves, and 5 of Ionescu-Shiley valves were used. In AVR, 38 of St. Jude Medical valves and 12 of Carpentier-Edward valves were used.The hospital operation mortality rate of congenital acyanotic, cyanotic, and acquired heart diseases were 1.6%, 18.3 % and 3.4% respectively. The overall mortality rate was 2.9 % [29/1000].

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Open Heart Surgery 110 Cases in One Year(1987) (연간 (1987년) 개심술 110례에 대한 검토)

  • 조광현
    • Journal of Chest Surgery
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    • v.21 no.2
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    • pp.351-365
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    • 1988
  • During one year[1987], 110 cases of open heart surgeries were performed in the department of Thoracic and Cardiovascular Surgery, Pusan Paik Hospital, Inje Medical College. And the results were summarized as follows. 1. Among the 110 cases, there were 77 cases of congenital heart diseases and 33 cases of acquired heart diseases. Age range of the congenital patients was 8 months to 30 years with the mean age of 8 years, and acquired patients was 16 to 56 years with the mean age was 32 years. 2. The heart lung machine used for cardiopulmonary bypass was Sarns 7000, 5-head roller pump, and the number and type of oxygenators were 50 of membrane type and 60 of bubble type. For all cases GIK[glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 3. Among the 77 congenital anomalies, there were 67 cases of acyanotic patients[ASD: 12, VSD: 50, PS: 1, AP window: 1, Gerbode defect: 1, ECD: 2] and 10 cases of cyanotic patients[TOF: 10], and to all of which the appropriate radical operations were applied. 4. Among the 33 acquired diseases, there were one case of left atrial myxoma, one of annuloaortic ectasia, 20 of mitral valve diseases[MS: 2, MSr: 8, MR: 2, MRs: 8], 9 of double valve diseases[MRs+AR: 1, MRs+ARs: 2, MRs+TR: 1, MSr+TR: 3, MSr+ASr: 1, MSr+ARs: 1], 2 of triple valve diseases[MSr+AR+TR: 1, MSr+ASr+TR: 1]. The left atrial myxoma was removed well with right atriotomy and atrial septal approach. And to the annuloaortic ectasia, Bentall operation was applied with good result. Mitral valve replacement[MVR] was applied to 20 cases of mitral valve diseases, double valve replacement[MVR+AVR] was applied to 6 cases of double valve diseases, MVR & tricuspid annuloplasty[TVA] was applied to 3 cases of mitral 5. The number of replaced valve were 39 in 31 cases. In MVR, 5 of mechanical valves[St. Jude Medical Valve] and 26 of tissue valves[Carpentier-Edward valve] were used. In AVR, 3 of mechanical valves and 5 of tissue valves were used. 6. Postoperative complications were occurred in 23 cases, and among them 21 cases were recovered with intensive cares, but 2 cases were expired[mortality: 1.8%].

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Clinical report of 131 cases of open heart surgery in 1985 (1985년도 연간개심술 131예 보고)

  • 김규태
    • Journal of Chest Surgery
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    • v.19 no.3
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    • pp.399-406
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    • 1986
  • 131 cases of open heart surgery were performed in the Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital in 1985. There were 116 congenital cardiac anomaly and 15 acquired heart diseases. Out of 116 congenital cardiac anomaly, 73 cases of acyanotic group and 43 cases of cyanotic group were noted. In 73 cases of acyanotic group, 17 ASD, 52 VSD and 4 other acyanotic anomaly were noticed. In 43 cases of cyanotic group, 4 Trilogy of Fallot, 34 TOF, 1 Pentalogy, 3 DORV and 1 DCRV were included. Of the 15 acquired valvular heart disease cases, individual incidence was in mitral valve 10, double valve 3, and simple aortic valve 2 cases. Total number of valve replaced was 16, and 13 for mitral, 2 for aortic, and 1 for tricuspid in position, including 1 cases of double valve replacement. Overall operative mortality for 131 cases of open heart surgery was 4.5%, and the operative mortality was 5.5% in congenital acyanotic group, 2.3% in congenital cyanotic group, 0% in TOF group and 6.7% in acquired group.

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The relationships among health related quality of life and uncertainty in adult patients with congenital heart diseases (성인 선천성 심장 질환자의 삶의 질과 불확실성간의 관계)

  • Jang, Youha;Shin, Nayeon
    • Journal of the Korea Convergence Society
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    • v.10 no.3
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    • pp.347-354
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    • 2019
  • Purpose: The purpose of this study was to determine the relationships among health related quality of life and uncertainty in adult patients with congenital heart diseases. Methods: The subjects were 136 adult congenital heart disease patients at the congenital heart center out-patient clinic of the A General Hospital in S city. Data were analyzed using descriptive statistics, t-test, ANOVA, Pearson's correlation coefficient and multiple regression. Results: There were significant negative correlations of quality of life with general motor function area and uncertainty. Among predictors, gross motor(${\beta}=-.38$) and daily activity (${\beta}=-.21$) had statistically significant influences on uncertainty(F=6.15, p=.018). Conclusion: Nursing interventions to promote gross motor and daily activity might be essential for adult patients with congenital heart disease in order to reduce uncertainty.

Open Heart Surgery:Clinical Analysis of 180 Cases (개심술 180례에 대한 임상적 고찰)

  • 나명훈
    • Journal of Chest Surgery
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    • v.27 no.6
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    • pp.460-471
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    • 1994
  • Between Feb. 1990 and Aug. 1993, 180 cases of the open heart surgery were performed under cardiopulmonary bypass in the Department of Thoracic & Cardiovascular surgery, Gil General Hospital. There were 83 cases with congenital heart diseases [CHD] and 97 cases with acquired heart diseases [AHD]. The CHD consisted of 78 acyanotic[mortality: 3.8 %] and 5 cyanotic cases with heart anomaly[mortality:l case]. The AHD were 97 cases, which contained 53 valvular, 27 ischemic heart diseases, 10 aortic diseases, 5 cases with myxoma, 1 case with post-infarct VSD, and 1 case with removal of infected pacing wire in right ventricle. In the 53 valvular heart diseases, there were 45 cases with valve replacement[MVR 27, AVR 9,MVR + AVR 9] and 8 cases with valvuloplasty. The number of the implanted prosthetic valves were 53. In MVR, 25 St. Jude, 6 Sorin, 3 Carpentier-Edward and 2 Intact medical valves were used. In aortic position, 13 St. Jude, 3 Sorin and 1 Intact medical valves were applied. The operative mortality was 5.6 % [3/53]. The annuloplasty applying artificial ring was performed in 17 patients[4 cases associated with MVR] and the number of the implanted ring was 19, which included 14 Duran ring[10 mitral, 4 tricuspid] and 5 Carpentier ring [3 mitral, 22 tricuspid]. In the 27 ischemic heart diseases, there were 9 cases with left main coronary artery lesions, 7 one vessel, 5 two vessels, and 6 three vessels. Average number of anastomosis was 2.8 per patient. The operative mortality was 14.3 % [4/27]. Among the 10 patients with aortic diseases, 7 cases were aortic dissection[type A: 5, type B: 2] and 3 cases were descending thoracic aortic aneurysm. The operative morality occurred in 3 cases. The overall mortality and the operative mortality of congenital and acquired heart disease was 7.8 %, 4.8% and 10.4%, respectively.

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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.

Open Heart Surgery for Congenital Heart Disease in Adult (성인 선천성 심장기형의 개심수술)

  • 구본원;허동명
    • Journal of Chest Surgery
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    • v.29 no.9
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    • pp.940-944
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    • 1996
  • Patients over 15 years of age who have undergone a surgical correction of congenital heart disease at Kyungpook University Hospital during the period of January 1990 through October 1994 have been reviewed . One hundred forty three, 22.4 % of 628 operations, which have repaired congenital heart diseases during this period were adult patients. There were 23 patients under 20 years of age, 58 between 20∼29 years, 34 between 30∼39 years, 18 between 40∼49 years, and 10 between 50∼59 years. The most common defects were atrial septal defects which accounted for 73 cases (51.1 %) and other common anomalies were ven- tricular septal defects (57 cases, 39.9 %), tetralogy of Falloffs(4 cases, 2.8%) in order of incidence. There were 10 non-fatal operative complications (6.9 %) but there was no operative mortality. This study shows the incidence of operable congenital heart diseases in adults and the fact that it could be corrected surgically with low mortality and morbidity.

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Congenital heart disease in the newborn requiring early intervention

  • Yun, Sin-Weon
    • Clinical and Experimental Pediatrics
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    • v.54 no.5
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    • pp.183-191
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    • 2011
  • Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.