• Journal of Chest Surgery
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• 제54권5호
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• pp.393-395
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• 2021

The anomalous connection of umbilical vessels to the heart is rare and has not yet been reported in the international scientific literature. Herein, we report the case of a newborn who was diagnosed with an anomalous connection of the umbilical vessels to the left ventricle. These anomalous vessels were functionally open for 2 weeks, and cellulitis was present in the area of the blood vessels connected to the skin. We performed division of these abnormal vessels and removal of the skin lesion.

• Journal of Chest Surgery
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• 제56권4호
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• pp.290-293
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• 2023

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart disease that often needs a critical decision on whether to open the right ventricular outflow tract (RVOT). Significant morbidity and considerable mortality might preclude the safe use of percutaneous or surgical right ventricular decompression in patients with muscular PAIVS. We report the case of a 21-day-old neonate weighing less than 3 kg who underwent hybrid RVOT stent insertion as initial palliation for muscular PAIVS and subsequent anatomical correction at 5 months of age, with 6 years of follow-up.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권6호
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• pp.594-600
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• 2019

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

• Journal of Chest Surgery
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• 제31권12호
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• pp.1226-1229
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• 1998

환아는 생후 50일된 체중 4.5kg의 남아로 선천성 심장질환 및 울혈성 심부전증으로 본원에 내원하여 시행한 심초음파상 막주위형 심실중격결손으로 진단되어 수술하였다. 수술후 중환자실에서 치료하던 중 방실접합부 이소성 빈맥이 발생하면서 혈역학적으로 불안정한 상태가 되었고 요량이 감소하여 강심제 및 이뇨제등의 약물 치료를 하였으나 호전되지 않아서 경도의 저체온(34℃)치료를 병행하였다. 술후 2일 동안 4차례의 저체온 치료로 방실접합부 이소성 빈맥은 정상 동율동으로 전환 되었고 혈역학적으로도 안정되었으며 요량도 증가 되어 수술후 8일에 정상 퇴원하였다.

• 대한영상의학회지
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• 제81권3호
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• pp.754-754
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• 2020

• Journal of Chest Surgery
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• 제36권3호
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• pp.136-141
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• 2003

성인 선천성 심기형에 동반된 삼첨판막 폐쇄부전에 대한 판막륜 성형술의 중기 성적 및 잔존폐쇄부전의 위험인자를 분석하였다. 대상 및 방법： 1989년 8월부터 2001년 6월까지 총 73명의 성인환자가 선천성 심기형에 동반된 삼첨판막 폐쇄부전으로 판막륜 성형술을 받았다. 환자의 성비는 51：22로 여자가 많았고 연령은 16∼73세(평균 43세)였다. 진단은 심방중격결손(55), 심실중격결손(6), 부분 폐정맥 환류이상(4), 기타(8)의 순 이였다. 수술 전, 후 판막 폐쇄부전의 평균 혈류 속도는 각각 3.25 m/sec, 2.56 m/sec이었고, 판막 성형술의 방법은 De Vega 형(43), Kay 형(18), Ring Annuloplasty 형(12)의 순 이었다. 외래 추적기간은 2,347 patient-month (평균: 32.6개월)이었고, 이 기간 중 총 134예의 심초음파가 시행되었다. 초음파상 III/IV 이상의 잔존 판막 폐쇄 부전이 있는 경우 판막 성명술 실패로 간주하였다. 결과: 7명의 환자(9.6%)에서 판막 성명술 실패의 소견을 보였고, 이중 1명에서 삼천판막 치환술이 시행되었다. 잔존 폐쇄부전의 위험인자로는 심방 중격 결손 이외의 진단, 폐쇄부전이 우심실 압력 과부하에 의한 경우, 수술 후 높은 판막 폐쇄부전 혈류 속도 등으로 해석되었고. 판막 성형술의 방법은 잔존 폐쇄부전과 무관하였다. 결론: 선천성 심기형에 동반된 삼첨판막의 폐쇄부전이 우심실 압력 과부하에 의한 경우 판막 성형술 시 주의를 요하며, 수술 후 우심실 압부하가 소실되지 않는 경우 잔존 폐쇄부전의 위험이 높다.

• Neonatal Medicine
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• 제25권4호
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• pp.137-143
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• 2018

Purpose: This study was performed to determine the clinical features of full-term infants with hypoxemia detected by pulse oximetry and to establish the diagnosis of critical congenital heart disease (CCHD). Methods: We retrospectively reviewed the medical records of neonates who had been admitted to the neonatal intensive care unit within 2 weeks of birth at Korea University Ansan Hospital between January 2013 and October 2017 (n=450). We classified these neonates based on the presence of hypoxemia at admission and investigated neonatal characteristics, initial symptoms, echocardiographic findings, and final diagnosis associated with hypoxemic diseases. Results: Of 450 term infants, 265 infants (58.9%) were identified hypoxemia by pulse oximetry at admission. The most common symptoms of them were cyanosis and tachypnea. Among them, 80.1% of infants (214/265) were diagnosed with respiratory tract disease and 8.3% of infants (22/265) had congenital heart disease. Thirteen infants (13/265, 4.9%) had CCHD and were treated with urgent surgery or transcatheter intervention within 28 days of birth. Majority of infants with respiratory tract disorder were transferred from hospital immediately after birth, but 46.1% of infants (6/13) with CCHD remained asymptomatic after birth and were admitted after 48 hours after birth. In addition, other hypoxemic illnesses were identified as neonatal infectious and neurological diseases. Conclusion: This study showed the importance of assessment in neonates with hypoxemia, including those diagnosed with CCHD. The possibility of CCHD should be considered in the differential diagnosis in neonates demonstrating hypoxemia after 48 hours of birth. A larger prospective study is needed to assess the effectiveness and outcomes of pulse oximetry for neonatal screening in Korea.

• Clinical and Experimental Pediatrics
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• 제48권6호
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• pp.660-664
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• 2005

저자들은 신생아 시기에 심부전 증세를 보인 간내 혈관내피종 환아에서 내과적 치료와 간동맥 색전술 후 호전을 보이지 않아 간좌엽 절제술을 시행하여 치료한 1례를 경험하였기에 보고하는 바이다.

• Advances in pediatric surgery
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• 제9권1호
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• pp.19-23
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• 2003

Necrotizing Enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. Twenty-five infants with NEC were treated at Asan Medical Center between January 2000 and December 2002, and 13 of them were term infants. In each case, the diagnosis of NEC was established by a clinical illness fulfilling the Bell's stage II or III NEC as modified by Walsh or by surgical findings. There were six males and seven females. The birth weight was from 1,960 to 3,700 g. The age at diagnosis was from 1 to 40 days. Four patients had congenital heart disease: one of who had hypothyroidism and cleft palate. Abdominal distension was present in all, and bloody stools in four. One patient had history of hypoglycemia, three had Rota viral infection. Eight patients had leucopoenia (<$5.0{\times}10^9/L$), seven had thrombocytopenia (<$100{\times}10^9/L$), and three severe thrombocytopenia (<$50{\times}10^9/L$). Laparotomy was required in 10 of the 13 patients. Indications for operation in the acute phase were failure to respond to aggressive medical therapy in five, and perforation in three patients. There were two late phase operations for intestinal stricture and fistula. There were no operative complications. Ten of thirteen patients survived (76.9%). Two patients died of septic complication. There was a delayed death due to heart failure. There was a significant difference in survival according to platelet count ($50{\times}10^9/L$) (p<0.05). Congenital heart disease and Rota viral infection are associated with NEC in term infants and thrombocytopenia and leucopoenia may be surgical indications.

• Journal of Chest Surgery
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• 제32권3호
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• pp.299-302
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• 1999

심한 울혈성 심부전으로 Dopamine을 1개월간 사용해온 10세의 확장성 심근증 환아에서 심장 이식을 시행하려 하였으나, 적절한 크기의 공여 심장이 없었고, 환아의 증상이 악화되어 부분 심실 절제술과 승모판 성형술을 시행하였다. 수술후 환아의 임상 증상은 현저히 호전되었고 수술전과 수술후 3개월, 6개월 1년째의 심장 초음파 검사 소견을 비교하여 추적 관찰한 결과, 좌심실의 심박출 계수는 수술전 17 %에서 수술후 각각 29%, 35%, 36%로 개선되었고 좌심실 확장기말 직경은 수술전 72 mm에서 수술후 각각 59 mm, 61 mm, 61 mm로 변화를 보였다. 부분 좌심실 절제술과 승모판 성형술의 동반 시행은 심장 이식의 기회가 상대적으로 적은 소아의 확장성 심근증에 있어 심장의 부하를 감소시켜 증상 완화를 시킬 수 있는 외과적 방법으로 심장 이식을 시행할 때까지 유지시켜주는 고식적 가치가 크며 아울러 장기적 추적 관찰이 필요하다고 사료 된다.