• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권1호
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• pp.72-78
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• 2020

Infantile hepatic hemangioma, the most common vascular tumor of the liver in infancy, can occur with acute postnatal liver and congestive heart failure. Nevertheless, its course is often benign, and many children can be diagnosed and treated without surgical intervention. The distinction from malignant diseases is not always easy and it not clear whether invasive procedures for diagnosis and therapy should be performed. Here we report our experiences in our Center for Pediatric Liver Disease and postulate that large studies are needed to avoid unnecessary invasive procedures for these patients in the future.

• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.532-537
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• 2010

목 적 : 선천성 심장병의 개심술 직후에 발생하는 부정맥은 술 후 조기 사망의 주된 원인의 하나인데, 이는 심폐 우회술 및 심근보호법의 발달, 술 후 집중치료와 적절한 약물 치료로 감소시킬 수 있다. 이에 이번 연구에서는 선천성 심장병의 개심술 후 조기에 발생한 부정맥의 빈도와 종류, 그 위험 인자들을 조사하여 보았다. 방 법: 2002년 1월부터 2008년 12월까지 선천성 심장병으로 경북대학교병원에서 개심술을 시행받은 561명의 환자를 대상으로 하였다. 이들의 의무기록지를 후향적으로 조사하여 진단명, 수술방법, 수술 후 부정맥이 발생한 시기와 기간, 부정맥의 종류와 치료방법, 부정맥으로 동반된 합병증 및 사망률, 부정맥 발생 당시 혈액 전해질 수치 등을 분석하였다. 결 과 : 소아 개심술 후 조기 부정맥의 빈도는 7.3% (42/578 개심술)로 남아 23명, 여아 18명이었으며, 개심술을 시행 받은 나이는 평균 $8.7{\pm}19.8$개월, 몸무게는 평균 $5.8{\pm}4.0kg$이었다. 발생한 부정맥 종류는 방실 접합부 이소성 빈맥이 17례로 가장 많았고, 다음으로 가속 심실고유 율동이 13례였으며, 또한 대혈관 전위에 대한 동맥 전환술에서 가장 많은 빈도의 부정맥을 보였다(36.4%). 대부분의 부정맥은 치료에 반응이 좋았다. 개심술 후 조기 부정맥의 위험 인자로는 수술 당시 작은 몸무게, 어린 나이, 긴 인공 심폐기 시간과 긴 대동맥 차단 시간이었다($P$<0.05). 부정맥이 발생한 환자에서 그렇지 않은 환자에 비해 인공호흡기 사용시간과 집중치료실 입원기간이 의미있게 길었으나($P$<0.05), 사망률에서는 의미있는 차이가 없었다. 결 론 : 선천성 심장병의 개심술 후 발생하는 조기 부정맥은 주의깊은 감시와 적극적인 치료로 치명적인 결과를 줄일 수 있다. 최근 개심술의 나이가 낮아짐에 따라 술 후 조기 부정맥에 대한 더욱 세심한 관리가 필요할 것으로 생각된다.

• Journal of Chest Surgery
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• 제47권3호
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• pp.280-282
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• 2014

Atrial septal defect (ASD) is one of the most common congenital heart defects in adults. Surgical repair is the most common treatment approach, but device closure has recently become widely performed in accordance with the trend toward less invasive surgical approaches. Although surgery is recommended when ASD is accompanied by atrial fibrillation, this study reports a case in which a complete cure was achieved by closure of a device and totally thoracoscopic ablation.

• 대한영상의학회지
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• 제81권2호
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• pp.310-323
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• 2020

선천성 심장질환의 치료에 있어서 그 해부학적인 구조를 올바르게 이해하는 것은 아주 중요하다. 이러한 선천성 심장질환의 구조적인 특징을 이해하는데 있어서 삼차원 프린팅 모델을 이용하는 것은 아주 효과적이다. 기존에 얻어진 전산화단층촬영이나 자기공명영상 혹은 심장 초음파 검사의 자료만으로는 정확한 복잡심장기형의 특징을 이해하는 것이 어려운 경우가 있으며, 삼차원 프린팅 모델의 사용이 이러한 제한점 들을 극복하는데 도움을 줄 수 있다. 최근 들어서는 삼차원 프린팅 모델을 이용해 선천성 심장질환의 교육과 수술 전 시뮬레이션 그리고 치료의 방침을 결정하는데 많은 도움을 받고 있으며, 실제 구체적인 환아들의 예를 통해서 이를 살펴보고자 한다. 또한 향후 그 기술의 발전 방향에 대해 알아보고, 심장외과 의사의 관점에서 수술 수기의 발전이나 훈련 등 여러 방면의 이용에 대해서도 살펴보고자 한다.

• Journal of Chest Surgery
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• 제49권2호
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• pp.107-111
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• 2016

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.

• Clinical and Experimental Pediatrics
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• 제50권11호
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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.

• Journal of Chest Surgery
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• 제35권1호
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• pp.48-51
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• 2002

폰탄수술 중 초기의 우심방-폐동맥 문합방식으로 시행한 환자의 장기추적에서 심방성 부정맥, 심방확장, 폐정맥폐쇄, 심실기능저하 등의 합병증이 발생된 경우 이에 대한 치료법으로 폰탄연결의 재교정이 권장된다. 심방성 빈맥을 동반한 폰탄연결 기능부전 환자에서 그에 대한 치료로서 혈류역학적 효율성이 월등한 것으로 알려진 심장외도관을 이용한 완전 체정맥-폐동맥연결과 부정맥 통로에 대한 냉동절제술의 병행이 선호된다. 본원에서는 삼첨판폐쇄증으로 우심방-폐동맥연결폰탄수술을 받은 후 장기추적 과정 중 이소성 심방빈맥,우심방매혈전, 폐정맥협착, 심실기능저하 등의 합병증이 발생된 환자 1례에서 냉동절제를 병행한 심장외도관 폰탄으로의 전환술을 경험하였기에 보고하는 바이다. 본 증례의 환자와 부모는 여호와의 증인 신자였으며 처치과정 중 수혈은 시행하지 않았다.

• Journal of Chest Surgery
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• 제51권1호
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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.

• Clinical and Experimental Pediatrics
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• 제60권8호
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• pp.237-244
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• 2017

During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously considered resistant to interventional therapies can now be managed with high success rates. The hybrid approach has enabled the overcoming of limitations inherent to percutaneous access, expanding the application of endovascular therapies as adjunct to surgical interventions to improve patient outcomes and minimize invasiveness. Percutaneous pulmonary valve implantation has become a successful alternative therapy. However, most of the current recommendations about pediatric cardiac interventions (including class I recommendations) refer to off-label use of devices, because it is difficult to study the safety and efficacy of catheterization and transcatheter therapy in pediatric cardiac patients. This difficulty arises from the challenge of identifying a control population and the relatively small number of pediatric patients with congenital heart disease. Nevertheless, the pediatric interventional cardiology community has continued to develop less invasive solutions for congenital heart defects to minimize the need for open heart surgery and optimize overall outcomes. In this review, various interventional procedures in patients with congenital heart disease are explored.

• Clinical and Experimental Pediatrics
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• 제59권11호
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• pp.446-450
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• 2016

Purpose: Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. Methods: We retrospectively reviewed the clinical data of 137 patients who underwent PVR using a bioprosthetic valve between January 2000 and December 2003. Among these patients, 89 were included in our study and divided into groups I (${\leq}12$ months) and II (>12 months) according to duration of aspirin use. We analyzed echocardiographic data from 9 to 11 years after PVR. Pulmonary vale stenosis and regurgitation were classified as mild, moderate, or severe. Results: The 89 patients consisted of 53 males and 36 females. Their mean age was $14.3{\pm}8.9$ years (range, 2.6-48 years) and body weight was $37.6{\pm}14.7kg$ (range, 14-72 kg). The postoperative duration of aspirin use was $7.3{\pm}2.9$ months in group I and $32.8{\pm}28.4$ months in group II. However, no significant difference in sex ratio, age, body weight, type of bioprosthetic valve, and number of early redo-PVRs. In the comparison of echocardiographic data about 10 years later, no significant difference in pulmonary valve function was found. The overall freedom rate from redo-PVR at 10 years showed no significant difference (P=0.498). Conclusion: Our results indicated no benefit from long-term aspirin medication (>6 months) in patients who underwent PVR with a bioprosthetic valve.