• Title/Summary/Keyword: 선천심장병

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One Stage Repair of Berry Syndrome in a Neonate (Berry 증후군의 신생아기 일차완전교정술)

  • 최창휴;곽재건;김진현;정요천;김동진;오세진;이정렬;김용진;노준량
    • Journal of Chest Surgery
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    • v.37 no.11
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    • pp.918-921
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    • 2004
  • Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

The changes in signal-averaged electrocardiogram after surgical correction of congenital heart disease (선천성 심장병의 개심술 후 신호 평준화 심전도의 변화)

  • Kim, Yeo Hyang;Choe, Hee Jung;Kim, Gun Jik;Cho, Joon Yong;Hyun, Myung Chul;Lee, Sang Bum
    • Clinical and Experimental Pediatrics
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    • v.52 no.12
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    • pp.1364-1369
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    • 2009
  • Purpose : To analyze abnormal ventricular activation in childhood congenital heart disease induced by postoperative changes in ventricular volume and pressure and ventricular scar formation using signal-averaged electrocardiography (SAECG). Methods : Fifty-two patients who had undergone open heart surgery (OHS) were enrolled. Patients were divided into the following 3 groups: right ventricular volume overload (atrial septal defect, group1), left ventricular volume overload (ventricular septal defect, group2), and right ventricular pressure overload (tetralogy of Fallot, group 3). The patients were monitored by standard 12-lead ECG and SAECG before and 2 months after the operation. QRS duration, QT and QTc intervals, filtered QRS (f-QRS), high frequency low amplitude potential (HFLA), and root mean square (RMS) voltage in the terminal 40 ms of SAECG were determined. Results : In the preoperative period, group1 showed significant increase in QRS (P=0.011) compared to those of the other 2 groups. In the postoperative period, group3 showed significant increase in the QTc interval (P=0.004) compared to those in the other 2 groups. SAECG parameters showed no significant differences among the groups in the pre- or postoperative period. Of the 52 patients, 12 (23%) in the preoperative period and 21 (40%) in the postoperative period had at least 1 SAECG abnormality. The prevalence of SAECG abnormalities was significantly higher in the postoperative group 2 and group 3 (preoperative: 20% versus postoperative: 28%, P<0.001, preoperative: 14% versus postoperative: 64%, P<0.001, respectively). Conclusion : Abnormal SAECG patterns may be attributed to postoperative scars, OHS itself, and/or ventricular overload.

One Stage Total Repair of the Aortic Arch Anomaly using the Regional Perfusion (대동맥궁 이상이 동반된 선천성 심장병에서 국소 순환을 이용한 일차 완전 교정)

  • Jang Woo-Sung;Lim Cheong;Lim Hong-Kook;Min Sun-Kyung;Kwak Jae-Kun;Chung Eui-Seuk;Kim Dong-Jin;Kim Woong-Han
    • Journal of Chest Surgery
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    • v.39 no.6 s.263
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    • pp.434-439
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    • 2006
  • Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.

The Effects of Right Anterolateral Thoracotomy in Cardiac Surgery (개심술시 우전측방흉부절개술의 효과)

  • Gwak, Mong-Ju;O, Bong-Seok;Lee, Dong-Jun
    • Journal of Chest Surgery
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    • v.30 no.10
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    • pp.986-990
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    • 1997
  • Twenty-three patients underwent operations to repair a congenital heart disease through right anterolateral thoracotomy(RALT) between December 1989 and December 1996. Defects repaired 22 atrial septum(13 ostium secundum;3 lower sepal defect;4 posterior septal defect; 1 sinus venosus;1 ostium primum) and 1 ventricular septal defect. There was no operative mortality or late morbidity directly related to RALT. The RALT incision is a safe and effective me hod to a median sternotomy in selective patients(especicially female). The cosmetic results are very good during the follow up periods.

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The Life of Adolescent Patients with Complex Congenital Heart Disease (복잡 심기형을 가진 환자의 청소년기 삶)

  • Lee, Sun-Hee;Kim, So-Sun
    • Journal of Korean Academy of Nursing
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    • v.40 no.3
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    • pp.411-422
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    • 2010
  • Purpose: In the present study, an analysis of the life of adolescents with complex congenital heart disease (CHD) was done using grounded theory. Consideration was given to the socio-cultural context of Korea. Methods: After approval from the institutional review board of Y hospital, 12 patients ranging in age from 14 to 35 were recruited. Data were gathered using in-depth interviews. Theoretical sampling was performed until the concepts were saturated. Results: The results confirmed the life of adolescents with complex CHD as a 'journey to finding uniqueness of oneself as a person with CHD'. The life consisted of 3 stages. In the crisis stage, participants had a feeling of threat to self-existence, and made an effort to be the same as others. In the self-recognition stage, participants who had sufficient role-performance built self-esteem while those who did not fell into self-accusation. In the self-establishment stage, participants who reached sufficiency in independence and knowledge planned the future, whereas those who did not conformed to the realities of life. Conclusion: The results of present study provide help in understanding the experiences of adolescents with CHD and provide a basis for developing nursing intervention strategies for these patients.

Clinical Experience of Cleft Lip and/or Palate Repair in Complex Congenital Heart Disease (선천성 복잡심장병 환아의 구순 구개열의 치험례)

  • Koh, Kyung Suk;Lee, Sang Hyuk;Eom, Jin Sup
    • Archives of Plastic Surgery
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    • v.32 no.3
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    • pp.385-388
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    • 2005
  • In cleft lip and/or palate patients with the complex congenital heart diseases, surgical repair of the cleft lip and/or palate has been postponed after the open heart surgery because the heart problem of the patient might cause more complications associated with anesthesia and surgery. There has been little report about experiences in the surgical management of these patients and optimal time of surgical intervention. Authors are introducing the experiences of performing corrective surgery of cleft lip and/or palate in the patients with congenital heart diseases before and after the open heart surgery. We managed five patients from May 1992 to March 2004. Two patients were male and the rest were female. One of them had cleft lip alone and others had cleft lip and palate. Two of them underwent delayed cleft lip and/or palate surgery after open heart surgery, and the rest had immediate intervention for cleft lip and/or palate. There was no complication during the operation and postoperative period. There would be no need to delay the corrective surgery of the cleft lip and/or palate after the open heart surgery, if solid medical team approach was available with the pediatric cardiologist and the anesthesiologist.

Systemic-Pulmonary Shunts for Cyanotic Congenital Heart Disease (선천성 청색증 심장병에서의 체-폐동맥 단락술)

  • Bang, Jong-Gyeong;Han, Seung-Se;Kim, Gyu-Tae
    • Journal of Chest Surgery
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    • v.21 no.1
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    • pp.136-142
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    • 1988
  • Between February, 1983, and March, 1987, thirty-one systemic-pulmonary shunts were performed in 28 patients with cyanotic congenital heart disease. Age ranged from 8 months to 28 years [mean age, 5.4 years, Weight ranged from 7 kg to 48 kg [mean weight, 16kg]. There were 4 classic Blalock-Taussig shunts, 5 central polytetrafluoroethylene shunts, 1 aorta-right pulmonary artery shunt with graft, and 21 modified Blalock-Taussig shunts. One patient required another shunt immediately due to insufficient pulmonary blood flow with patent graft. There was no postoperative death. Conduit diameters included 4mm [2 cases], 5 mm [22 cases], and 6 mm [3 cases]. Long term follow up was available in 27 patients [96.4%] with mean period of 20 months [range, 4 months to 49 months]. The effectiveness of shunt was evaluated by cardiac catheterization with angiography [15 patients] or clinically. They showed improvement of systemic oxygen saturation values by 12% and decrease of hemoglobin by 2.3gm/dl [P<0.01]. There were 2 shunt occlusion in central shunts at 32 and 48 months respectively, and one narrowing of graft in modified Blalock-Taussig shunt at 12 months. The patency rate was 91.6% at 24 months for 5 mm grafts in modified Blalock-Taussig shunt.

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Reverse Patent Ductus Arteriosus with an Aberrant Left Common Carotid Artery in a Shih Tzu Dog (시츄개에서 발생한 비정상적 좌경동맥 분지장애를 동반한 우-좌형 동맥관 개존증)

  • Han, Suk-Hee;Yoon, Byung-Il;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.27 no.5
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    • pp.573-578
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    • 2010
  • A 4-year-old intact male Shih Tzu dog (weighing 5 kg), was presented with signs of severe dyspnea, exercise intolerance and cyanosis. Diagnostic studies suggested a reverse patent ductus arteriosus (rPDA) with pneumonia. Despite emergency treatments with oxygen, diuretics, antibiotics and medical therapy for hyperkalemia, the dog died of ventricular fibrillation from hyperkalemia and dehydration. Necropsy revealed the rPDA with a marked descending aortic dilation and abnormally arisen left common carotid artery.

Nutrition Supply, Biochemical Nutrition Indexes and Patient Outcomes in New Born Babies with Open Heart Surgery according to Post Operative Fasting Period (선천성 심장병 신생아의 개심술 후 금식기간에 따른 영양공급량, 생화학적 영양지표 및 환아결과)

  • Jang, Ji-Young;Kim, Myoung-Hee
    • Child Health Nursing Research
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    • v.18 no.3
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    • pp.119-126
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    • 2012
  • Purpose: The purpose of this study was to examine and analyze the post operative fasting period of neonates in the intensive care unit (ICU) after receiving open heart surgery in order to provide optimal nutrition support for these neonates. The variables included biochemical nutrition indexes (albumin, total lymphocyte count, total cholesterol) and patient outcomes (duration of mechanical ventilation, ICU stay, hospital stay, infectious complication). Methods: The participants were 124 neonates in ICU after receiving open heart surgery, and the design of this study was to investigate their post operative fasting period retrospectively to analyze the biochemical nutrition indexes and patient outcomes according to post operative fasting period. Results: The results for 4 groups according to post operative fasting period showed that the group with less 48 hours of fasting had the best biochemical nutrition indexes and patient outcomes, followed by the 48-72 hour group, the 72-144 hour group, and the over 144 hour group. Conclusion: The results indicate that for new born babies receiving open heart surgery, the period of fasting after the operation should be minimized and tube feeding should be started as soon as possible.

Genetic Screening of the Canine Transcription Factor AP-2 Beta(TFAP2B) Gene in Dogs with Patent Ductus Arteriosus(PDA) (동맥관 개존증(PDA)에 이환된 개에서의 전사 인자 AP-2 beta(TFAP2B) 유전자 스크리닝)

  • Nam, So-Jeong;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.26 no.2
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    • pp.123-129
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    • 2009
  • Patent ductus arteriosus(PDA) is an abnormal shunt between the descending aorta and pulmonary artery through the incompletely closed ductus arteriosus and is the most common congenital heart defect in dogs. Recent human genetic studies found that a the gene mutation in transcription factor AP-2 beta(TFAP2B) was responsible for syndromic cases of PDA. Mutations in the TFAP2B gene are associated with certain congenital cardiac defects in humans that include PDA. In this study, we isolated the entire coding exons of canine TFAP2B gene for genetic screening in dogs with PDA. Analysis of the deduced amino acid sequence suggested that the canine TFAP2B are phylogenetically closer to the human TFAP2B(100% identity in amino acid sequence) than mouse and rat. In cTFAP2B gene screening, one single c.936+203G>A base change was found in affected Maltese dogs with PDA. However, further screening found the same base change in one unaffected control dog, suggesting this base change might be polymorphism. No other base changes were found in other dog breeds enrolled in this study. Because the base change was located in the intronic region and found in an unaffected control dog, TFAP2B might not be responsible for familial PDA in Malteses and sporadic cases of other dog breeds, although the gene promoter region should be investigated before reaching to this conclusion. A future study that may take this study further would be to collect more samples and to screen TFAP2B in various breeds of dogs with PDA and other various congenital heart defects.