• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.680-687
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• 2010

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects, hemodynamic changes as part of the natural history, surgical repair and related scarring, and residual hemodynamic abnormalities. Acquired factors such as aging, hypertension, diabetes, obesity, and others may also contribute to arrhythmogenesis in CHD. The first step in evaluating arrhythmias in CHD is to understand the complex anatomy and to find predisposing factors and hemodynamic abnormalities. A practical stepwise approach can lead to diagnosis and prompt appropriate interventions. Electrophysiological assessment and management should be done with integrated care of the underlying heart defects and hemodynamic abnormalities. Catheter ablation and arrhythmia surgery have been increasingly applied, showing increasing success rates with technological advancement despite complicated arrhythmia circuits in complex anatomy and the difficulty of access. Correction of residual hemodynamic abnormalities may be critical in the treatment of arrhythmia in patients with CHD.

• Journal of Chest Surgery
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• 제54권1호
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• pp.72-74
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• 2021

Congenital atresia of the left main coronary artery (LMCA) is an extremely rare coronary anomaly that necessitates surgical correction. Patients with LMCA atresia may have various clinical symptoms, which are determined by the degree of collateral vessel development from the right coronary system, the metabolic demands of the heart, and concomitant mitral insufficiency caused by myocardial ischemia. Unlike in adults, there are limited surgical options for coronary artery disease in children. Herein, we report a case of LMCA atresia with mitral regurgitation in a 19-month-old child that was successfully corrected by coronary artery bypass grafting and mitral valve repair.

• Clinical and Experimental Pediatrics
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• 제52권4호
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• pp.481-487
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• 2009

목 적 : 본 연구는 단일 기관에서 혈역학적으로 의미 있는 선천성 심기형을 가진 극소 저체중 출생아의 임상경과와 치료성적 및 예후 인자를 알아보고자 하였다. 방 법 : 1994년 11월부터 2007년 12월까지 13년간 삼성서울병원 신생아 집중치료실에 입원하였던 출생 체중 1,500 g 미만의 극소 저체중 출생아 1,098명을 대상으로 의무기록을 후향적으로 조사하였다. 선천성 심기형을 가진 대상아 33명의 임상경과, 합병증, 예후를 조사하였고 이들을 대조군 1,065명과 비교하였다. 결 과 : 총 극소 저체중 출생아 1,098명 중 33명(3%)에서 선천성 심기형이 있었으며, 이들 중 7명(21%)에서 선천기형 및 염색체 이상을 동반하였다. 심기형 종류로는 심실 중격 결손이 21명(64%)으로 가장 많았다. 자궁 내 성장 지연은 심기형군이 대조군에 비해 높았으나(34% vs. 20%), 재태기간, 출생체중, 신생아 호흡 곤란 증후군, 기관지폐 형성 이상, 괴사성 장염, 뇌실내 출혈, 그리고 낭종성 뇌실주위 백질 연화증의 빈도는 심기형군과 대조군 사이에 차이가 없었다. 심장수술은 단순 심기형에서 4명(19%), 복합 심기형에서 9명(75%)에게 시행되었고, 9명(69%)이 다단계 수술을, 10명(77%)은 조기 중재시술을 받았다. 심기형군의 사망률은 대조군에 비해 증가되어 있었고(27% vs. 16%), 특히 염색체 이상 및 다발성 기형을 동반한 경우에만 증가되었고(86% vs. 11%), 그 외 단순, 복합 심기형 여부(19% vs. 42%)와는 상관관계가 없었다. 결 론 : 극소 저체중 출생아에서 발생한 선천성 심기형의 예후는 심기형 자체의 복합성 보다는 동반된 선천 기형 및 염색체 이상이 결정한다.

• Clinical and Experimental Pediatrics
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• 제51권2호
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• pp.219-221
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• 2008

저자들은 발열과 식욕저하를 주소로 패혈증 의증으로 전원 된 27일된 신생아에서 구토와 혈변 등은 없었으나, 입원 당시 복부팽만과 단순복부촬영에서 보인 소장 확장소견으로 개복술을 실시한 결과 선천성 밴드에 의해 내탈장된 소장이 압박되어 유발된 장폐색증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Neonatal Medicine
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• 제15권2호
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• pp.183-189
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• 2008

신생아의 선천성 거대 간내 혈관내피종을 복부 단층 촬영과 $^{99m}Tc$-RBC 간스캔을 통해 진단하고, 대증요법과 함께 IFN$\alpha$를 11개월간 단독으로 피하 투여하여 크기 감소를 유도한 후 남은 병변의 완전한 수술적 제거와 조직학적 확진을 시행하였음을 보고하는 바이다.

• Journal of Chest Surgery
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• 제29권2호
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• pp.136-146
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• 1996

Studies on normal human embryos and on malformed human hearts have been two main sources of the information on the developmental cardiology, Recent advances in the biological technology has opened a new era and descriptive embryology is being shifted into dynamic developmental biology. In this review, we discuss the current understanding on the cardiac embryology relevant to clinical practices of pediatric cardiology. Classical cardiac embryology starts with understanding on five segments of a straight heart tube : the sinus venosus, the primitive atria, the embryonic left ventricle, the embryonic right ventricle and the truncus arteriosus. Key steps in the normal morphogenetic process are the complex spiral septation of ventriculoarterial junction and two jumping connections : between the embryonic right atrium and embryonic right ventricle, and between the embryonic left ventricle and the aorta. Only after these two steps are successfully completed, the third fetal stage tak s place, when myocardial growth and remodeling take place There are two outstanding progresses on the cardiac embryology during recent five-year period. One is immunohistochemical mapping of the conduction system in the developing heart and the other is the understanding on the neural crest cell migration followed by molecular detection of the microdeletion of chromosome 22. A balanced progress of classical morphological studies, modern biological technics and advanced clinical medicine is an urgent task for doctors and scientists dealing with children with sick hearts.

• Neonatal Medicine
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• 제18권2호
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• pp.395-398
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• 2011

굴기능부전증후군은 매우 드물지만 신생아에서도 발생할 수 있으며 소아 환자의 경우 대부분 선천심장병이 있거나 심장수술 후에 발생한다. 그러나 이와 같은 심질환의 선행 요인이 없는 신생아에서도 발생할 수 있기 때문에 이에 대한 인지가 필요하다. 굴기능부전증후군은 대부분 특발성으로 발생하며 증상도 다양하고 24시간 심전도 검사상에서도 다양한 형태로 관찰된다. 본 증례에서는 초극소저체중출생아에서 선천심장병 없이 발생한 굴기능부전증후군에 대해 보고하였다. 환아는 심전도상 굴느린맥과 함께 심방세동이 동반된 느린맥과 빠른맥이 반복되어 관찰되었으며 무호흡, 산소포화도의 저하, 청색증의 징후를 나타냈다. 증상이 있는 굴기능부전증후군의 경우 대부분 심장박동조율기의 삽입이 필요하나 본 환아는 당시 체중 750 g으로 심장박동조율기의 삽입에 실패하여 약물 치료를 시작하였다. Isoproterenol과 atropine으로 치료하면서 환아의 체중이 1,500 g을 넘어서자 증상과 심전도상의 호전을 보였다. 향후 본 환아에서처럼 심장박동조율기의 삽입이 어려운 소아 환자의 경우에 대한 치료 체계에 대해 많은 연구가 필요하다.

• Journal of Chest Surgery
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• 제55권2호
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• pp.158-167
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• 2022

Background: This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD). Methods: CHD patients requiring postoperative ECMO support between May 2011 and May 2021 were retrospectively reviewed. Patients were divided into non-survivors and survivors to hospital discharge. Survival outcomes and associations of various factors with in-hospital death were analyzed. Results: Fifty patients required postoperative ECMO support. Patients' median age and weight at the time of ECMO insertion were 1.85 months (interquartile range [IQR], 0.23-14.5 months) and 3.84 kg (IQR, 3.08-7.88 kg), respectively. Twenty-nine patients (58%) were male. The median duration of ECMO support was 6 days (IQR, 3-12 days). Twenty-nine patients (58%) died on ECMO support or after ECMO weaning, and 21 (42%) survived to hospital discharge. Postoperative complications included renal failure (n=33, 66%), bleeding (n=11, 22%), and sepsis (n=15, 30%). Prolonged ECMO support (p=0.017), renal failure (p=0.005), continuous renal replacement therapy (CRRT) application (p=0.001), sepsis (p=0.012), bleeding (p=0.032), and high serum lactate (p=0.002) and total bilirubin (p=0.017) levels during ECMO support were associated with higher mortality risk in a univariate analysis. A multivariable analysis identified CRRT application (p=0.013) and a high serum total bilirubin level (p=0.001) as independent risk factors for death. Conclusion: Postcardiotomy ECMO should be considered as an important therapeutic modality for patients unresponsive to conventional management. ECMO implementation strategies and management in appropriate patients without severe complications, particularly renal failure and/or liver failure, are crucial for achieving positive outcomes.

• Clinical and Experimental Pediatrics
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• 제59권6호
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• pp.262-270
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• 2016

Purpose: Pulmonary arterial hypertension (PAH) leads to right ventricular failure (RVF) as well as an increase in pulmonary vascular resistance. Our purpose was to study the effect of sildenafil on right ventricular remodeling in a rat model of monocrotaline (MCT)-induced RVF. Methods: The rats were distributed randomly into 3 groups. The control (C) group, the monocrotaline (M) group (MCT 60 mg/kg) and the sildenafil (S) group (MCT 60 mg/kg+ sildenafil 30 mg/kg/day for 28 days). Masson Trichrome staining was used for heart tissues. Western blot analysis and immunohistochemical staining were performed. Results: The mean right ventricular pressure (RVP) was significantly lower in the S group at weeks 1, 2, and 4. The number of intra-acinar arteries and the medial wall thickness of the pulmonary arterioles significantly lessened in the S group at week 4. The collagen content also decreased in heart tissues in the S group at week 4. Protein expression levels of B-cell lymphoma-2 (Bcl-2)-associated X, caspase-3, Bcl-2, interleukin (IL)-6, matrix metalloproteinase (MMP)-2, endothelial nitric oxide synthase (eNOS), endothelin (ET)-1 and ET receptor A (ERA) in lung tissues greatly decreased in the S group at week 4 according to immunohistochemical staining. According to Western blotting, protein expression levels of troponin I, brain natriuretic peptide, caspase-3, Bcl-2, tumor necrosis factor-${\alpha}$, IL-6, MMP-2, eNOS, ET-1, and ERA in heart tissues greatly diminished in the S group at week 4. Conclusion: Sildenafil alleviated right ventricular hypertrophy and mean RVP. These data suggest that sildenafil improves right ventricular function.

• Clinical and Experimental Pediatrics
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• 제50권3호
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• pp.298-301
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• 2007

선천성 심장병의 수술후 발생되는 유미흉은 드문 합병증이다. 이는 수분, 전해질 및 영양의 불균형을 초래할 뿐만 아니라 감염에 취약함을 보이기도 한다. 지금까지의 보존요법에 대해 비교적 치료성적이 좋으나 치료에 반응하지 않는 경우에는 수술적 중재가 불가피하다. 저자들은 보존요법과 octreotide 주입을 병행하여 수술적 중재를 피하고 효과적으로 치료한 수술후 발생된 유미흉 2례를 보고하는 바이다.