• Title/Summary/Keyword: Congenital heart defect

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Minimal Skin Incision with Full Sternotomy for Congenital Heart Surgery (최소 피부 절개술을 이용한 선천성 심장 질환 수술)

  • Park, Choung-Kyu;Park, Pyo-Won;Jun, Tae-Gook;Park, Kay-Hyun;Chae, Hurn
    • Journal of Chest Surgery
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    • v.32 no.4
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    • pp.368-372
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    • 1999
  • Background: Although there have been few reports about minimal skin incision for the repair of congenital heart lesions, minimizing an unsightly scar is a particularly important factor in growing children. We have adopted a technique that permits standard full sternotomy, conventional open chest cardiopulmonary bypass, aortic cross-clamping, left atrial vent, and antegrade cardioplegia with minimal surgical scar. Material and Method: With minimal skin incision and full sternotomy, 40 patients with congenital heart disease underwent open heart surgery from April 1997 through September 1997. Defects repaired included 30 ventricular septal defects, 4 atrial septal defects, and 1 sinus Valsalva aneurysm in 35 children(M:F=17: 18), and 3 Atrial septal defects, 1 ventricular septal defect, and 1 partial atrioventricular septal defect in 5 adults(M:F=1:4). Midline skin incision was performed from the second intercostal space to 1 or 2 cm above the xiphoid process. For full sternotomy, we used the ordinary sternal saw in sternal body, and a special saw in manubrium under the skin flap. During sternal retraction, surgical field was obtained by using two retractors in a crossed direction. Result: The proportion of the skin incision length to the sternal length was 63.1${\pm}$3.9%(5.2∼11cm, mean 7.3cm) in children, and 55.0${\pm}$3.5%(10∼13.5cm, mean 12cm) in adults. In every case, the aortic and venous cannulations could be done through the sternal incision without additional femoral cannulation. There was no hospital death, wound infection, skin necrosis, hematoma formation, or bleeding complication. Conclusion: We conclude that minimal skin incision with full sternotomy can be a safe and effective alternative method for the repair of congenital heart diseases in children and adults.

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Cor triatriatum with left superior vena cava[Report of a case] (좌측 상공정맥을 동반한 삼심방증 1례 보고)

  • 박병순
    • Journal of Chest Surgery
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    • v.18 no.2
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    • pp.293-298
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    • 1985
  • Cor triatriatum is a rare congenital malformation of the heart in which a septum stretches in a transverse plane through the left atrium, thus creates two left atrial subchambers. The upper one connects with the pulmonary veins, and the lower connects with the left ventricles. Due to the rarity of, and difficulty in diagnosing car triatriatum, data on the surgery of the disease are of necessity and very limited. A case of cor triatriatum combined with atrial septal defect and persistent left superior vena cava was experienced in November, 1984 in Chonnam University Medical School. There was a transverse septum in the left atrium below atrial septal defect, all pulmonary veins were drained into the upper chamber of the left atrium which connected with the right atrium via atrial septal defect and the lower chamber via an oval opening[8mm] in the abnormal septum and the lower chamber was connected with the left atrial appendage, and the left ventricle via mitral valve. There was persistent left superior vena cava drained to left atrium and coronary sinus. The abnormal transverse septum within the left atrium was completely excised and the atrial septal defect was repaired with Woven Dacron patch. The post-operative course was not eventful and the patient was discharged to home with good result on the 15th postoperative day, and has been in good condition upto now.

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Clinical characterization of a Korean case with 3p25 deletion

  • Lee, Hye Jin;Kim, Ja Hye;Cho, Ja Hyang;Lee, Beom Hee;Choi, Jin-Ho;Yoo, Han-Wook
    • Journal of Genetic Medicine
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    • v.11 no.1
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    • pp.36-39
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    • 2014
  • Chromosome 3 (3p) deletion syndrome is a rare genomic disorder caused by a deletion at the terminal end of the short arm of chromosome 3. The primary characteristics of the syndrome are delayed development, dysmorphic features, and several other congenital anomalies. Here, we describe the case of a 2-year-old Korean girl with typical features of 3p deletion syndrome, including dysmorphic facial features, low birth weight, developmental delay, growth and cognitive retardation, and congenital heart disease. This case represents the first report of 3p deletion syndrome in Korea. Although phenotypes can be variable among patients, a clinically recognizable pattern has been described for this genetic defect, and our report helps to identify other cases with 3p deletion syndrome from a clinical and genetic perspective.

Congenital Left Atrial Appendage Aneurysm - A case report- (선천성 좌심방이류 - 1례 보고 -)

  • 김근직;이응배;전상훈;장봉현;이종태;김규태
    • Journal of Chest Surgery
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    • v.32 no.9
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    • pp.827-830
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    • 1999
  • Congenital left atrial appendage aneurysm is a very rare congenital cardiac abnormality. That is postulated to arise from a developmental weakness in the atrial wall in utero. Clinically, patients are often asymptomatic and are diagnosed incidentally, but supraventricular arrhythmias and systemic thromboembolism have also been reported in some cases. Surgical resection at the time of diagnosis is recommended because of the propensity for thromboembolic complications. A 13-month-old female, who was suspected preoperatively as having partial absence of pericardium with left atrial herniation through the defect, underwent surgical resection of the left atrial appendage aneurysm. Exposure through a median sternotomy showed an intact pericardium. The postoperative course was uneventful.

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Intensive management of acute right heart failure (급성 우심 부전의 집중 치료)

  • Kim, Gi Beom;Noh, Chung Il
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1041-1048
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    • 2007
  • Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF) can be evaluated in terms of decreased RV contractility, RV volume overload, and/or RV pressure overload. The management for RVF starts from clear understanding of the pathophysiology of RVF. In addition to correction of the underlying disease, management of RVF per se is very important. Meticulous control of volume status, inotropic agents, vasopressors, and pulmonary selective vasodilators are the main tools in the management of RVF. The relative importance of each tool depends on the individual clinical status. Medical assist device and surgery can be considered selectively in case of refractory RVF to optimal medical treatment.

Scimitar Syndrome with Atrial Septal Defect (Scimitar 증후군 수술치험)

  • Cho, Bum-Koo;Kim, H.;Kang, M.S.
    • Journal of Chest Surgery
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    • v.21 no.6
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    • pp.1099-1102
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    • 1988
  • A vertical skin incision with median sternotomy for open heart surgery usually leaves an unsightly hypertrophic scar, and it makes cosmetic and psychic problems especially in young female patients. Since November 1986, we have used a bilateral submammary skin incision with dissection of a fasciocutaneous flap in 5 young female patients with uncomplicated acyanotic congenital heart disease. The exposure of the operative field was excellent. And we had good results without any significant complication associated with this type of incision.

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Airway Improvement with Medium-Term Compression Duration after Right Pulmonary Artery Anterior Translocation

  • Jang, Woo Sung;Kim, Jae Bum;Kim, Jae Hyun;Choi, Hee Jeong
    • Journal of Chest Surgery
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    • v.51 no.1
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    • pp.57-60
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    • 2018
  • Left main bronchus compression occasionally occurs in patients with cardiac disease. A 19-month-old female patient weighing 6.7 kg was admitted for recurrent pneumonia and desaturation. S he had an atrial septal defect (AS D) with a right aortic arch. Her left main bronchus had been compressed between the enlarged right pulmonary artery (RPA) and the descending thoracic aorta for 14 months. We conducted ASD closure and RPA anterior translocation via sternotomy. The left main bronchus compression was relieved despite the medium-term duration of compression.

Transvenous occlusion of patent ductus arteriosus using an embolization coil in a Maltese dog

  • Lee, Seung-Gon;Moon, Hyeong-Sun;Choi, Ran;Hyun, Changbaig
    • Korean Journal of Veterinary Research
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    • v.47 no.4
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    • pp.461-467
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    • 2007
  • A 6-year-old female Maltese dog (body weight 2.0 kg) was referred to the Veterinary Teaching Hospital, Kangwon National University with primary complaints including exercise intolerance and heart murmur. Based on clinical and diagnostic findings including grade V/VI left basal continuous murmur, bounding femoral pulsation, left ventricular enlargement pattern in electrocardiogram, cardiomegaly with aortic bulging on the thoracic radiography, and shunt flow between aorta and pulmonary artery on the echocardiography, the dog was diagnosed as the left-to-right patent ductus arteriosus. The patent ductus arteriosus was successfully treated by lodging a single embolization coil with transjugular approach.

The Norwood Operation in Infants with Complex Congenital Heart Disease (복잡 선천성 심기형 환자에서의 Norwood 술식)

  • 박정준;김용진
    • Journal of Chest Surgery
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    • v.30 no.3
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    • pp.263-269
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    • 1997
  • From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

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Corrected transposition of the great arteries: surgical treatment of associated anomalies (교정형 대혈관전위증동반된 심혈관기형의 수술요)

  • Kim, Gi-Bong;No, Jun-Ryang;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.371-380
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    • 1984
  • Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.

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