신생아에 심한 심부전증을 일으킨 선천성 뇌동정맥 기형: 풍선 색전 요법을 시도한 1례

박인숙,김영휘,홍창의,조영국,서대철,박영일 울산대학교 의과대학 1993 울산의대학술지 Vol.2 No.2

Cerebral arteriovenous malformation is a rare cause of congestive heart failure in neonates and is known to carry a very poor prognosis even wish prompt recognition and aggressive treatment. We recently encountered a 2-day-old male neonate with huge cerebral arteriovenous malformation who presented with intractable heart failure. Despite aggressive medical treatment and partial embolization with detachable balloon, baby died of severe hearts failure. Since there is no report of percutaneous balloon embolization in a neonate with this lesion in Korean literature, we wish to present our experience in one such neonate. Also we believe this is the first report of color Doppler findings in cerebral arteriovenous malformation in Korean literature.

Pediatric heart transplantation: how to manage problems affecting long-term outcomes?

Kim, Young Hwue The Korean Pediatric Society 2021 Clinical and Experimental Pediatrics (CEP) Vol.64 No.2

Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

Detection rate and clinical impact of respiratory viruses in children with Kawasaki disease

Kim, Ja Hye,Yu, Jeong Jin,Lee, Jina,Kim, Mi-Na,Ko, Hong Ki,Choi, Hyung Soon,Kim, Young-Hwue,Ko, Jae-Kon The Korean Pediatric Society 2012 Clinical and Experimental Pediatrics (CEP) Vol.55 No.12

Purpose: The purpose of this prospective case-control study was to survey the detection rate of respiratory viruses in children with Kawasaki disease (KD) by using multiplex reverse transcriptase-polymerase chain reaction (RT-PCR), and to investigate the clinical implications of the prevalence of respiratory viruses during the acute phase of KD. Methods: RT-PCR assays were carried out to screen for the presence of respiratory syncytial virus A and B, adenovirus, rhinovirus, parainfluenza viruses 1 to 4, influenza virus A and B, metapneumovirus, bocavirus, coronavirus OC43/229E and NL63, and enterovirus in nasopharyngeal secretions of 55 KD patients and 78 control subjects. Results: Virus detection rates in KD patients and control subjects were 32.7% and 30.8%, respectively (P=0.811). However, there was no significant association between the presence of any of the 15 viruses and the incidence of KD. Comparisons between the 18 patients with positive RT-PCR results and the other 37 KD patients revealed no significant differences in terms of clinical findings (including the prevalence of incomplete presentation of the disease) and coronary artery diameter. Conclusion: A positive RT-PCR for currently epidemic respiratory viruses should not be used as an evidence against the diagnosis of KD. These viruses were not associated with the incomplete presentation of KD and coronary artery dilatation.

First reported case of fetal aortic valvuloplasty in Asia

( Sun-young Yoon ),( Hye-sung Won ),( Mi-young Lee ),( Min Kyong Cho ),( Euiseok Jung ),( Ki-soo Kim ),( Young-hwue Kim ) 대한산부인과학회 2017 Obstetrics & Gynecology Science Vol.60 No.1

Prenatal intervention of severe fetal aortic valve stenosis by ultrasound-guided percutaneous balloon valvuloplasty has been performed to prevent the progression to hypoplastic left heart syndrome, and achieve biventricular circulation in neonates. Here we report a case of fetal aortic valvuloplasty prenatally diagnosed with aortic stenosis at 24 weeks of gestation and showed worsening features on a follow-up echocardiography. Prenatal aortic valvuloplasty was performed at 29 weeks of gestation, and was a technical success. However, fetal bradycardia sustained, and an emergency cesarean delivery was performed. To the best of our knowledge, this is the first reported case of fetal aortic valvuloplasty which was performed in Asia.

Retrospective Multicenter Study of Respiratory Syncytial Virus Prophylaxis in Korean Children with Congenital Heart Diseases

Ah Young Kim,Se Yong Jung,최재영,Gi Beom Kim,Young-Hwue Kim,Woo Sup Shim,I-Seok Kang,Jo Won Jung 대한심장학회 2016 Korean Circulation Journal Vol.46 No.5

Background and Objectives: We conducted a review of current data on respiratory syncytial virus (RSV) prophylaxis with palivizumab, in Korean children with congenital heart diseases (CHD). In 2009, the Korean guideline for RSV prophylaxis had established up to five shots monthly per RSV season, only for children <1 year of age with hemodynamic significance CHD (HS-CHD). Subjects and Methods: During the RSV seasons in 2009-2015, we performed a retrospective review of data for 466 infants with CHD, examined at six centers in Korea Results: Infants received an average of 3.7±1.9 (range, 1-10) injections during the RSV season. Fifty-seven HS-CHD patients (12.2%) were hospitalized with breakthrough RSV bronchiolitis, with a recurrence in three patients, one year after the initial check-up. Among patients with simple CHD, only five (1.1%) patients received one additional dose postoperatively, as per the limitations set by the Korean guideline. Among the 30 deaths (6.4%), five (1.1%) were attributed to RSV infection; three to simple CHD, one to Tetralogy of Fallot, and one to hypertrophic cardiomyopathy (HCM). Of the three HCM patients that exceeded guidelines for RSV prophylaxis, two (66.6%) were hospitalized, and one died of RSV infection (33.3%). Conclusion: In accordance to the Korean guideline, minimal injections of palivizumab were administered to patients having HS-CHD <one year of age during the RSV season; the risk of RSV infection remains significant among children with simple CHD, cardiomyopathy, and children above the age of one year with HS-CHD.

Prenatal diagnosis of atrial isomerism in the Korean population

( Mi Young Lee ),( Hye Sung Won ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Byong Sop Lee ),( Ellen Ai Rhan Kim ),( Young Hwue Kim ),( Jeong Jun Park ),( Tae Jin Yun ),( Ahm Kim ) 대한산부인과학회 2014 Obstetrics & Gynecology Science Vol.57 No.3

Objective To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. Methods A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. Results Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. Conclusion Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

우수논문상(모체태아의학)

원혜성 ( Mi Young Lee ),( Hye Sung Won ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Byong Sop Lee ),( Ellen Ai Rhan Kim ),( Young Hwue Kim ),( Jeong Jun Park ),( Tae Jin Yun ),( Ahm Kim ) 대한산부인과학회 2015 대한산부인과학회 학술대회 Vol.101 No.-

Objective: To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. Methods: A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. Results: Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSO), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outiet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. Conclusion: Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSO and complete heart block In LAI and a better survival rate in RAI. Although the postnatal outcomes lor RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

Beneficial and adverse effects of bosentan treatment in korean patients with pulmonary artery hypertension.

Sohn, Dae-Won,Kim, Hyung-Kwan,Kim, Myung-A,Song, Yeong-Wook,Noh, Chung-Il,Kim, Duk-Kyung,Kang, I-Seok,Kim, Hojoong,Lee, Sang-Do,Kim, Young-Hwue,Youn, Ho-Joong,Chung, Namsik,Choi, Jae-Young,Jun, Jae-Bu Korean Society of Circulation 2009 Korean Circulation Journal Vol.39 No.3

<P>The purpose of this study was to investigate 1) the beneficial effect of bosentan treatment (125 mg twice daily) on exercise capacity and echocardiographic variables and 2) the profiles and frequency of adverse events in Korean patients with World Health Organization (WHO) class III or IV pulmonary artery hypertension (PAH).</P>

소아에서 발현한 배뇨 후 실신 1례

이선연,류수정,김덕수,김영휘,고태성,김재문,Lee, Sun Youn,Ryu, Su Jeong,Kim, Deok Soo,Kim, Young Hwue,Ko, Tae Sung,Kim, Jae Moon 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.12

배뇨 후 실신은 신경 매개성 반사에 의한 실신으로 사태실신의 한 종류이며 건강한 남성에서 아침에 기립 배뇨시에 잘 일어난다. 실신의 원인은 다양하기 때문에 배뇨후 실신의 진단을 위해 정확한 병력과 심전도, 운동부하검사, 심초음파, 기립경사검사, 뇌파, 뇌 자기공명영상, 요류 동태 검사 등이 사용된다. 배뇨후 실신의 기전은 부교감 신경계의 반사 자극으로 서맥과 말초혈관 확장이 일어나고 Valsalva 효과에 의해 심실로 정맥환류가 감소하게 되어서 대뇌 혈류감소가 일어나 실신하게 된다. 실신시 뇌파는 대뇌 혈류감소에 의해 고진폭의 서파와 평탄화 소견이 나타나게 되고 심한 허혈시에는 허혈성 경련을 일으킬 수도 있다. 저자들은 9세 남아로 기립배뇨시 실신을 보인 후 저혈압을 보이고 특징적인 뇌파소견을 보인 환아를 경험하였기에 보고하는 바이다. Syncope in children and adolescents have a common occurrence according for up to 15% before adulthood. Micturition syncope, a kind of situational syncope, can be considered a form of reflex syncope. It can typically occur in healthy young men after rising from bed in the early morning who experience sudden loss of consciousness during or immediately after urination. The mechanism of micturition syncope is not completely understood, but it has been suggested that vasovagal reflex mediated bradycardia and peripheral vasodilation and decreased venous return due to Valsalva effect and standing position lead to the decrease in cerebral blood flow resulting in syncope. The causes of syncope are variable. So complete history taking, physical examination, electrocardiography, exercise stress test, echocardiography, head-up tilt table test, electroencephalography(EEG), brain magnetic resonance image and urodynamic study should be required for the diagnosis of micturition syncope. There were several reports about micturition syncope. However, literature of micturition syncope at the pediatric age has rarely been reported in Korea so far. Therefore, we report a case of a 9-year-old boy with micturition syncope with typical EEG findings of high amplitude delta wave and flattening during syncope.

Chemoport 제거 중 발생한 카테터 색전증

송기병,남소현,김대연,김성철,김영휘,김인구,Song, Ki-Byung,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Young-Hwue,Kim, In-Koo 대한소아외과학회 2006 소아외과 Vol.12 No.2

Chemoport is widely used in pediatric surgery field. But various complications can occur during the process of insertion or removal of chemoport. Surgeons must be familiar with the treatment of these complications. We had one catheter cuts off during chemoport removal, become a catheter embolism. Interventional radiologic removal was successful. Verifying the length of removed catheter and careful observation of the catheter tip during removal procedure is important to prevent the possibility of catheter embolus. Radiologic intervention was accessible to remove the retained catheter.