The Recent Surgical Result of Total Anomalous Pulmonary Venous Return

장소익,송진영,김수진,최은영,심우섭,이철,임홍국,이창하 대한심장학회 2010 Korean Circulation Journal Vol.40 No.1

Background and Objectives: Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute. Subjects and Methods: A total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction. Results: The study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery. Conclusion: Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.

The results of cardiopulmonary exercise test in healthy Korean children and adolescents: single center study

이준석,장소익,김성호,이상윤,백재숙,심우섭 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.6

Purpose: The cardiopulmonary exercise test (CPET) is an important clinical tool for evaluating exercise capacity and is frequently used to evaluate chronic conditions including congenital heart disease. However,data on the normal CPET values for Korean children and adolescents are lacking. The aim of this study was to provide reference data for CPET variables in children and adolescents. Methods: From August 2006 to April 2009, 76 healthy children and adolescents underwent the CPET performed using the modified Bruce protocol. Here, we performed a medical record review to obtain data regarding patient’ demographics, medical history, and clinical status. Results: The peak oxygen uptake (VO2Peak) and metabolic equivalent (METMax) were higher in boys than girls. The respiratory minute volume (VE)/CO2 production (VCO2) slope did not significantly differ between boys and girls. The cardiopulmonary exercise test data did not significantly differ between the boys and girls in younger age group (age, 10 to 14 years). However, in older age group (age, 15 to 19 years), the boys had higher VO2Peak and METMax values and lower VE/VCO2 values than the girls. Conclusion: This study provides reference data for CPET variables in case of children and adolescents and will make it easier to use the CPET for clinical decision-making.

혈소판 감소증읠 동반한 미숙아 패혈증의 임상 양상

심재훈,장소익,심은정,조도준,김덕하,민기식,유기양 대한소아청소년과학회 2004 Clinical and Experimental Pediatrics (CEP) Vol.47 No.10

Purpose:This study was performed to characterize sepsis with thrombocytopenia in premature infants to determine if thrombocytopenia is a prognostic factor in sepsis in premature infants. Methods:We retrospectively analyzed the medical records of sepsis in premature infants admitted to the neonatal intensive care unit(N=41) at the Hallym University Sacred Heart Hospital from January 1999 to December 2002. The incidence, risk factors, symptoms, hematologic and bacteriologic findings were analyzed during episodes of sepsis. Results:Of the 41 cases, 29(72%) were associated with thrombocytopenia. The ratio of male to female was 1.2:1. The vast majority(98%) were late-onset sepsis. The risk factors of the thrombocytopenic group were low birth weight and low gestational age. Major symptoms were poor activity (72%), apnea/tachypnea(52%), but were not significantly different between two groups. In the thrombocytopenic group(N=29), low total WBC count and high CRP level were discovered(P=0.03, P<0.01). The mean platelet count was 70.17(×103/mm3) at diagnosis of sepsis, and a mean platelet nadir was 43.10(×103/mm3). The severe thrombocytopenia(below 50×103/mm3) in the thrombocytopenic group was discovered in 69% and the duration of thrombocytopenia was about eight days. The majority of pathogens were gram-negative bacteria and candida. The thrombocytopenic group showed a prolonged length of stay and a high mortality rate. According to comparisons between the survived and expired groups, low birth weight, low gestational age, neutropenia and thrombocytopenia were significantly correlated with mortality(P<0.05). Conclusion:The sepsis with thrombocytopenia in premature infants showed late-onset sepsis and high morbidity and mortality, although differences were not significant statistically. Especially, low birth weight and low gestational aged infants should be cautiously treated and monitored. 목 적:미숙아에서 패혈증이 발병한 경우 혈소판 감소증이 초기에 동반되는 경우가 많은데, 이는 신생아 패혈증의 조기 진단에 비특이적인 지표로 이용되었고 예후에 관여하는 인자로 고려되기도 하였다. 이에 저자들은 혈소판 감소증이 동반된 미숙아 패혈증의 임상 양상을 분석하여 혈소판 감소증을 동반하지 않은 미숙아 패혈증과의 차이점 및 특징을 유추해보고자 하며 더불어 병발 초기에 동반된 혈소판 감소증이 미숙아 패혈증의 예후 지표가 되는지 알아보기 위해 본 연구를 실시하였다. 방 법:1999년 1월부터 2002년 12월까지 4년 동안 한림대학교 성심병원의 신생아 집중 치료실에 입원하였던 재태 연령 37주 미만의 미숙아에서 입원기간 중에 패혈증이 발병되었던 환아 41명을 대상으로 하였고 병력지 고찰을 통하여 혈소판 감소증을 동반한 미숙아 패혈증(혈소판 감소군)의 발생 빈도, 발생 시기별 분포, 위험 인자, 임상 증상, 혈액 검사 소견, 세균학적 소견 등을 후향적으로 분석하였으며 각각에 대해 혈소판 비감소군과 비교 분석하였다. 결 과: 1)대상 환아 41명 중 혈소판 감소군이 29명(71%), 혈소판 비감소군이 12명(29%)이었고 각각의 남녀 비는 1.2:1, 1.4:1 이었으며 혈소판 감소군의 대부분(98%)은 지발형 패혈증이었다. 2) 혈소판 감소군에서 패혈증의 위험인자는 재태 연령이 어리고 출생 체중이 낮은 것이었다. 3)패혈증 발병시 혈소판 감소군에서 가장 흔한 증상은 처짐이었고(72%) 무호흡 또는 빈호흡 등의 호흡기 증상이 약 52%에서 관찰되었으나 혈소판 비감소군과 비교하여 유의한 차이를 보이지 않았다. 4)혈액검사소견 중 혈소판 감소군은 비감소군에 비해 총백혈구수가 낮았으며 CRP는 의미있게 높게 측정되었다(P=0.03, P< 0.01). 5)혈소판 감소군에서 패혈증 발병시 평균 혈소판 수치는 70.17(×103/mm3)이었고 혈소판 수치의 최저치는 평균 43.10(×103/mm3)이었으며 혈소판 감소 기간은 약 8일이었다. 혈소판이 50(×103/mm3) 미만으로 감소하는 심한 경우가 69%에서 관찰되었다. 6)원인균으로는 비혈소판 감소군에 비해 혈소판 감소군에서 그람음성균과 Candida가 많이 검출되었다. 7) 혈소판 감소군의 재원 기간은 평균 46.8일이었고 비감소군은 31.7일로 혈소판 감소군에서 재원 기간이 더 길었으며 사망률은 각각 34%, 17%로 혈소판 감소군이 비감소군에 비해 2배정도 사망률이 높았다. 8)전체 환아를 생존군과 사망군으로 나누어 비교 분석해보면 사망군은 생존군에 비해 출생 체중이 유의하게 적었으며(P= 0.02) 재태 연령이 유의하게 낮았다(P=0.01). 검사 소견으로는 생존군에 비해 사망군에서 호중구 감소증과(P=0.01) 혈소판 감소증이(P<0.01) 유의 있게 많이 관찰되었다. 그러나 다중 회귀 분석으로 분석한 결과 이러한 검사 소견은 재태 연령과 출생 체중을 고려하였을 때 유의한 결과를 보이지 않았다(P>0.05). 결 론:미숙아에서 패혈증이 발병시 혈소판 감소군은 연구 대상이 적어 통계적 유의성은 없으나 혈소판 비감소군과 비교하여 재원 기간이 길고 사망률이 비교적 높게 나타나는 등 예후가 좋지 않으며, 특히 재태 연령과 출생 체중이 적은 미숙아에서 혈소판 감소증과 중성구 감소증을 동반한 패혈증이 발생한 경우 사망률이 높기 때문에 이러한 환아에서 적극적인 치료와 세심한 주의를 요한다고 생각되며 이에 대한 좀 더 많은 자료의 수집과 연구가 필요하리라 사료된다.

영아 돌연사 증후군과 연관하여 아기를 재우는 방법에 대한 인식 연구

이동준,장소익,심은정,조도준,김덕하,민기식,유기양 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.6

Use of an Amplatzer Vascular Plug to occlude a tubular type of patent ductus arteriosus

최은영,장소익,김수진 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.9

Patent ductus arteriosus (PDA) is a common congenital heart defect. All PDAs, regardless of size or degree of symptoms, require occlusion. Transcatheter PDA occlusion features fewer complications than trans-thoracic closure. It is also more cost-effective and has an excellent occlusion rate. Therefore, transcatheter PDA occlusion is accepted as the standard treatment option for PDA. However, tubular-type PDAs are difficult to close with ordinary detachable coils or the Amplatzer Duct Occluder; thus, these lesions remain a challenge for transcatheter closure. We attempted to occlude a tubular-type PDA by using an oversized Amplatzer Vascular Plug, which allowed intraluminal packing of the ductus. By using this treatment method, PDA occlusion was achieved safely with an excellent final outcome. We suggest that this approach may be a good option for transcatheter closure of a tubular-type PDA.

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute

김문선,윤자경,김성호,방지석,장소익,이상윤,최은영,박수진,권혜원 대한소아청소년과학회 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.6

Purpose: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. Methods: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1–4 years). Medical records were reviewed retrospectively. Results: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0 ± 1.9 mm and 8.4 ± 1.6 mm to 10.1 ± 3.6 mm and 12.3 ± 2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7 ± 15.6 mmHg to 10.4 ± 10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56 ± 0.21 to 0.46 ± 0.19. In the CoA group, the pressure gradient decreased from 50 ± 33 mmHg to 17 ± 8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%–70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29 ± 12 months. Conclusion: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

Short-Term Change of Exercise Capacity in Patients with Pulmonary Valve Replacement after Tetralogy of Fallot Repair

황태웅,김성욱,김문선,장소익,김성호,이상윤,최은영,박수진,권혜원,임효빈 대한심장학회 2017 Korean Circulation Journal Vol.47 No.2

Background and Objectives: The aim of this study was to investigate the effect of pulmonary valve replacement (PVR) on exercise capacity and determine cardiopulmonary exercise (CPEX) parameters associated with improvement in right ventricle (RV) function. Subjects and Methods: We retrospectively analyzed CPEX and magnetic resonance imaging parameters in a total of 245 patients who underwent PVR from January 1998 to October 2015. In addition, we analyzed the characteristics of the patients who showed improved exercise capacity after PVR. Results: Twenty-eight patients met the inclusion criteria for the study. CPEX parameters after PVR showed no significant changes in all patients. However, baseline predicted peak oxygen uptake (VO2peak) (%) value was significantly lower in patients with significant improvement in exercise capacity after PVR, as compared to patients who showed decreased exercise capacity after PVR (60.83±10.28 vs. 75.81±13.83) (p=0.003). In addition, patients with improved exercise capacity showed a positive correlation between the change of right ventricular ejection fraction (RVEF) (%) and the change of anaerobic threshold (r=0.733, p=0.007); whereas, patients with decreased exercise capacity showed a negative correlation between the change of RVEF (%) and the change of predicted VO2peak (%) (r=-0.575, p=0.020). Conclusion: The importance of predicted VO2peak (%) in evaluating exercise capacity differentiated from other CPEX variables. The change of anaerobic threshold and predicted VO2peak (%) might be a useful predictor of the change in RV function after PVR.

선천성심장병 환아에서의 Respiratory syncytial virus 감염례 관찰

심우섭,이재영,송진영,김수진,김성혜,장소익,최은영 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.3

Purpose:Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. Methods:On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. Results:During the winter season, RSV caused 20–50% of LRI admissions in children. In patients with completely repaired simple left to right (L–R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L–R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. Conclusion:To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied. 목적:RSV는 영아기에 세기관지염이나 폐렴을 일으키는 중요한 요인으로 알려져 있다. 건강한 영아에서는 RSV 감염으로 위중한 병을 일으키는 경우는 드물지만 선천성심장병과 관련된 심부전이나 청색증, 폐동맥고혈압의 상태에 있거나 조산 미숙 등과 관련된 만성폐질환 상태에 있는 영아의 경우 위중한 병을 일으킬 수 있다. 본 연구에서는 선천성심장병 소아와 비선천성심장병 소아에서 RSV 감염례를 조사하여 RSV 감염이 선천성심장병 환아에 미치는 영향에 대하여 조사하고자 하였다. 방법:2003년 7월부터 2006년 6월까지 세종병원에 세기관지염이나 폐렴으로 입원한 343례의 환아의 비강 분비물에서 RSV 항원검사를 시행하여 RSV 감염에 의한 하기도염의 연령별 계절별 발생 양상을 조사하였다. RSV 감염된 하기도염 환아들에서 선천성심장병 환아의 여러 경우와 비심장병 환아의 입원 기간, 중환자 치료, 인공환기 치료 등 임상 경과를 비교하였다. 결과:RSV에 의한 하기도염은 어린 영아기에 많이 발생하고 있으며 겨울철에 유행하는 양상을 보이고 있다. RSV에 의한 하기도염 환아 중 선천성심장병 환아에서 비심장병 환아에 비하여 중증 감염이 많았고 장기입원을 보였다(P<0.01). 비교적 간단한 단순단락 병변인 ASD, PDA, VSD 등의 선천성심장병에서 심장수술로 교정되고 충분히 회복된 환자에서는 RSV에 의한 하기도염에서 비교적 짧은 입원기간과 가벼운 경과를 보여 비심장병 환아와 큰 차이가 없었다. 선천성심장병에 대한 완전교정 수술이나 단계적 고식수술 후에도 감염, 폐동맥고혈압, 판막역류, 혈관협착 등의 원인으로 심폐계에 상당한 문제가 남아있는 경우 RSV 감염시 비심장병 환아에 비하여 장기 입원을 보이고(P<0.01) 중증 경과를 보였다. 교정수술 받지 않은 상태의 선천성심장병을 가진 환아에서는 대부분 6개월 이내의 어린 나이에 RSV에 의한 하기도염이 발생하고 있으며 비심장병 환아에 비하여 장기 입원을 보이고(P<0.01) 중증 경과를 보였다. 선천성심장병 심장수술 후 1개월 이내의 회복기에 병원 감염을 포함하여 상당수의 RSV 감염이 발생하고 있음을 확인하였으며 이들은 심장수술 직후의 면역기능 저하와 관련하여 중증 감염으로 발전할 가능성이 있을 것으로 생각된다. 결론:미교정 상태의 선천성심장병 환아와 교정 후에도 계속 심폐계에 문제가 남아있는 선천성심장병 환아에서 RSV 감염에 의해 중증 경과와 장기 입원이 초래될 수 있다. 선천성심장병 환아에서 이러한 RSV 감염의 피해를 줄이기 위해서는 RSV 감염으로 심각한 위험이 초래 될 수 있는 고위험 세부군의 파악과 이들에 대한 예방 항체 치료 등 적절한 예방 노력이 필요하다.