선천성 심장 질환과 심장 외 기형

유용균(Yong Gyun Yoo),조재성(Jae Sung Cho),박용원(Yong Won Park),한성식(Sung Shik Han),이연혜(Yeon Hye Lee),윤혜정(Hye Jeong Yoon) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.3

N/A Purpose: The diagnostic usefulness of fetal echocardiography in congenital heart disease is well known. One of the indications for fetal echocardiography is presence of extracardiac anomalies and such coexistent congenital heart disease may have important implications for obstetric and neonatal management. Materials and methods: 496 women with singleton pegnancies with amgenital anomalies that underwent penatal care and follow-up in Severance Hospital for 5 years from 1991 to 1995 were evaluated for the incidence of congenital heart disease. Results: The incidence of congenital heart disease in fetuses with the associated extracardiac anomalies were as follows; central nervous system 6.9%(11/160), gastro-intestinal system 10.7%(8/75), genito-urinary sysem 4.7%(4/85), others 5.6%(4/72). Thirty-one of eighty-ane fetuses with congenital heart disease were found to have the associated extracardidac anomalies and ten of them chenoscenal abnormalities. Conclusion: We recommend that fetuses with congenital extracardiac anomalies sbould be checked for the presence of congenital heart disease and chmmosomal abnormalities, if needed.

선천성 심장병 환아에서 음향학적 요소의 변동

오정은,최윤미,김선준,주찬웅 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.2

Purpose:To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. Methods:Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter¸ and shimmer) were analyzed using a multi-dimensional voice program. Results:The average fundamental frequency showed a significant decrease in patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot, except in atrial septal defect and pulmonary stenosis. The length of the analyzed sample (duration) did not show a significant difference when compared with the control group. There was a significant increase in jitter percent in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. There was an increase in shimmer in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. The noise-to-harmonic ratio increased in ventricular septal defect, patent ductus arteriosus, and atrial septal defect but there was no significant difference in pulmonary stenosis and tetralogy of Fallot. While analyzing acoustic variables, the voice change was significantly higher, especially in patent ductus arteriosus followed by ventricular septal defect and atrial septal defect. Most of these acoustic variables were deviant in left-to-right shunt lesions in congenital heart disease, especially in patent ductus artriosus. Conclusion:The results of the voice change analysis of preoperative children with congenital heart disease revealed that the acoustic variables differed by each congenital heart disease. Moreover, the acoustic variables were prominently deviant in congenital heart disease with left-to-right shunts. 목적:애성 및 음성 변동은 심장병 환자에서 관찰될 수 있는데 이러한 음성 변동 정도를 음향학적 지표의 평가로 분석하여, 소아에서 선천성 심장병 종류에 따른 음성 변동을 음향학적 요소로 분석하고자 하였다. 방법:수술 전 선천성 심장병 환아 94명을 연구 대상으로 하였고 심실 중격 결손, 동맥관 개존, 심방 중격 결손, 폐동맥 협착, 활로 사징이 포함되었으며, 음성 변동을 조사하기 위한 음향 지표 분석은 통증 자극을 통하여 얻어진 음성 자료를 multi-dimensional voice program system을 이용하여 평가하였다. 연구에 사용된 음향 지표로 average fundamental frequency, length of analyzed sample, jitter percent, shimmer, noise to harmonic ratio를 분석하였다. 결과:음향 지표인 average fundamental frequency은 동맥관 개존, 심실 중격 결손, 활로 사징 경우에서 유의한 감소를 나타냈으며, length of analyzed sample은 연구 대상 선천성 심장병 환아에서 대조군에 비해 의미있는 차이를 나타내지 않았다. Jitter percent, shimmer, noise to harmonic ratio는 심실 중격 결손, 동맥관 개존, 심방 중격 결손에서 대조군에 비해 증가되었다. 이러한 음성 변동은 연구 대상 선천성 심장병 중 동맥관 개존에서 음도변이, 강도변이, 음성의 소음 정도가 가장 뚜렷하게 나타났으며, 동맥관 개존, 심실 중격 결손, 심방 중격 결손 순으로 그 음성 변동의 특징을 나타냈다. 결론:수술 전 선천성 심장병 환아에서 음성을 음향학적 지표로 비교 분석한 결과 음향학적 지표들이 심장 질환의 유형별 차이를 보이는데 좌우 단락 심질환군에서 현저한 비정상적 음향학적 지표를 나타냈다.

Functional Assessment for Congenital Heart Disease

Yiu-Fai Cheung 대한심장학회 2014 Korean Circulation Journal Vol.44 No.2

Significant improvement in survival of children with congenital cardiac malformations has resulted in an increasing population of adolescent and adult patients with congenital heart disease. Of the long-term cardiac problems, ventricular dysfunction remains an important issue of concern. Despite corrective or palliative repair of congenital heart lesions, the right ventricle, which may be the subpulmonary or systemic ventricular chamber, and the functional single ventricle are particularly vulnerable to functional impairment. Regular assessment of cardiac function constitutes an important aspect in the long-term follow up of patients with congenital heart disease. Echocardiography remains the most useful imaging modality for longitudinal monitoring of cardiac function. Conventional echocardiographic assessment has focused primarily on quantification of changes in ventricular size and blood flow velocities during the cardiac cycles. Advances in echocardiographic technologies including tissue Doppler imaging and speckle tracking echocardiography have enabled direct interrogation of myocardial deformation. In this review, the issues of ventricular dysfunction in congenital heart disease, conventional echocardiographic and novel myocardial deformation imaging techniques, and clinical applications of these techniques in the functional assessment of congenital heart disease are discussed.

선천성 심장병 영아 어머니의 모유수유 행위 영향요인

안혜진,손현미 부산대학교 간호과학연구소 2020 글로벌 건강과 간호 Vol.10 No.2

Purpose: The purpose of this study was to investigate the factors influencing the breastfeeding behavior of mothers of infants with congenital heart disease, focusing on Health Belief Model. Methods: The participants were 130 mothers of infants with congenital heart disease recruited from the outpatient clinic at the pediatric heart center of a hospital. Data were collected using self-report questionnaires consisting of breastfeeding behavior, perceived threat of infant’s disease, perceived benefits and barriers of breastfeeding, breastfeeding self-efficacy, and cues to action. Results: Only 40.0% of the participants were breastfeeding, and the average breastfeeding duration was 1.31 months. The factors influencing breastfeeding behavior of mothers of infants with congenital heart disease were direct breastfeeding (OR=2.76), breastfeeding support (OR=1.57), and breastfeeding self-efficacy (OR=1.08). Conclusion: Compared with mothers of normal baby, the breastfeeding rate of infants with congenital heart disease is very low and their duration of breastfeeding is very short. Based on the results of this study, encouraging direct breastfeeding and increasing breastfeeding self-efficacy can be effective strategies to promote breastfeeding for mothers of infants with congenital heart disease.

심장질환의 진단을 위해 의뢰된 환아들에 대한 자료 분석

이영환,최광해 영남대학교 의과대학 2000 Yeungnam University Journal of Medicine Vol.17 No.1

소아의 심장질환을 진단하는데 필수적인 진단도구로 자리를 잡아가고 있는 심초음파 검사는 신속한 확진과 그 방법의 비침습성으로 인하여, 소아 심장 클리닉에 심장질환 검사에 대한 의뢰가 증가되고 있는 실정이다. 그러나 심장질환이 의심되어 소아 심장 클리닉에 진단을 위하여 의뢰된 환아들에 대한 자료의 분석이 거의 없는 상태이므로, 심장질환의 진단을 위해 의뢰된 환아들의 특성에 대한 기초자료로 활용하고자 이들에 대한 자료를 비교·분석하였다. 대상 및 방법으로는 1998년 10월 1일부터 1999년 7월 10일까지 심장 질환이 의심되어 소아 심장 클리닉에 심장 검사를 위하여 의뢰되어 심초음파 검사를 시행한 428명을 대상으로 하였다. 이들 환아들의 자세한 병력, 흉부 방사선 검사, 심전도 검사의 결과와 필요한 경우에 추가 시행한 운동 부하 심전도, 24시간 심전도 및 심혈관조영술의 결과를 병력지를 이용한 후향적 조사를 하였다. 의뢰 당시 환아의 성별 및 연령별 분포는 남아가 261례(61.0%), 여아가 167례(39.0%)로 남녀 비는 1.6:1이였으며, 1세 미만이 268례로 의뢰된 전체환아 중 62.6%를 차지하였다. 의뢰된 이유로는 심잡음이 248례(57.9%)로 가장 많았으며 그 다음이 발열이 52례(12.1%), 심전도 이상이 43례(10.0%), 청색증이 33례(7.7%) 등의 순이었다. 의뢰 당시 의심된 심장질환 중 선천성 심장질환이 의심되어 의뢰된 경우는 302례(70.6%)였으며, 후천성 심장질환이 의심된 경우는 76례(17.8%), 부정맥이 의심되어 의뢰된 경우는 50례(11.6%)로 나타나, 선천성 심장질환이 의심되어 의뢰된 경우가 가장 많았다. 의뢰 당시 의심되는 심장 질환별 임상적 양상 비교에서 선천성 심장질환이 의심된 경우는 심잡음이 248례(57.9%)로 가장 많았으며, 후천성 심장 질환이 의심된 경우는 발열이 52례(12.1%), 부정맥으로 의뢰된 경우는 심전도 검사의 이상 소견이 43례(10.0%)로 가장 많았다. 최종 진단 결과 선천성 심장병이 212례로 49.5%이었으며, 후천성 심장병이 59례로 13.9%이었고, 부정맥이 13례로 3.0%를 차지하였고, 정상인 경우도 144례로 33.6%나 되었다. 결론적으로 신생아기에는 선천성 심장질환이 많았고, 유아기 이후에는 후천성 심장질환과 부정맥이 증가하는 추세를 보였다. 그러나 심초음파검사를 위해 의뢰된 환아 중 144례(33.6%)가 심초음파검사에서 정상 소견을 나타내었으므로, 심음 청진에 대한 확신보다는 심초음파검사에 대한 의존도가 지나치게 높은 것으로 나타났다. 따라서, 선천성 심장질환의 감별을 위한 올바른 심음 청진에 대한 훈련이 다시 한번 강조되어야 할 것으로 사료된다. Background : Echocardiography is rapidly establishing itself as the primary diagnostic technique for investigation of children with heart diseases, and referrals are increasing to the pediatric cardiology clinic for investigation. However, because there is a lack of analyzed data on the patients referred to the pediatric cardiology clinics, we have proceeded to compare and analyze their characteristics to provide basic data base. Methods : From Oct. 1, 1998 to Jul. 10, 1999, total 443 cases referred to the pediatric cardiology clinic of Yeungnam University Hospital were studied retrospectively by medical records, chest X-ray, EKG and echocardiography, etc. Results : The results were as follows. 1. The proportion of male was 61.0%(261 cases) and that of female was 39.0%(167 cases). The ratio of male to female was 1.6:1. The proportion infants less than 1 year-old was 62.6%(268 cases) of all patients. 2. Cardiac murmur was present in 248 cases(57.9%), which was the most common case of referral ed to the pediatric cardiology clinic. The impression at referral was more congenital heart disease(70.6%) than acquired heart disease(17.8%) and arrhythmia(11.6%). 3. The final diagnosis was as follows : congenital heart disease was present in 212 cases(49.5%), acquired heart disease, 59 cases(13.9%) ; arrhythmia, 13 cases(3.0%) ; normal heart, 144 cases(33.6%). Conclusion : Among the patients referred to pediatric cardiology clinic, 33.6%(144 cases) had normal hearts and why these patients were referred may be possibly due to more dependence on echocardiography than on auscultaion instruction. Therefore, clinical and auscultatory skill should be emphasized to minimize dependence on expensive echocardiography for evaluation of pediatric heart disease.

Survival of the Infants with Bronchopulmonary Dysplasia and Congenital Heart Disease

Min Sub Jeung,Young Hwa Kong,Se In Sung,Jin Young Song 대한신생아학회 2016 Neonatal medicine Vol.23 No.4

Purpose: Pulmonary hypertension is a known risk factor for mortality in preterm infants with bronchopulmonary dysplasia. However, mortality in patients with bronchopulmonary dysplasia and congenital heart disease has been poorly investigated. Therefore, we conducted an investigation into the mortality and risk factors in these patients. Methods: We reviewed the records of 45 preterm infants who were diagnosed with bronchopulmonary dysplasia and congenital heart disease from 2010 to 2013. Their survival was compared with that of a group of control individuals who did not have congenital heart disease. A variety of factors associated with survival were examined. Results: Although initial pulmonary hypertension was more frequent in the patient group, no significant differences were found between the patients and the control subjects with respect to cumulative mortality. The log-rank test indicated that many factors, including follow-up pulmonary hypertension, the use of pulmonary vasodilators, and aggravated oxygen demand, but not the congenital heart disease type, impacted upon survival in the patient group. Aggravated oxygen demand was the only factor that was determined to be associated with mortality in the multivariate analysis. Conclusion: There was no significant difference between the patient and the control groups with respect to cumulative survival. Of the three factors that affected survival within the patient group, aggravated oxygen demand was the only risk factor that was associated with mortality.

청색형 선천형 심장질환과 관련된 뇌농양에 대한 임상적 고찰

정영섭,김동호,정희원,한대희 대한신경외과학회 1984 Journal of Korean neurosurgical society Vol.13 No.1

Fourteen cases of brain abscess with cyanotic congenital heart disease operated during 10 years from 1974 to 1983 were reviewed. The abscess associated with cyanotic congenital heart disease constituted 21.8% (14/64) of total brain abscesses during this period. Brain abscess occurred in 1.8% (14/784) of the patients with cyanotic congenital heart disease. Of 14 cases, 12 had a tetralogy of Fallot and others were transposition of the grear vessels and pulmonary atresia. A peak in the age distribution was seen at 5 to 11 years of age and the preponderance of males was present(2:1=M:F). The major symptoms and signs were due to the increased ICP and 21.4 %(3/14) of the abscesses was nonfebrile. All of the abscesses were supratentorial and located in temporal, parietal, occipital, frontal area in order of frequency. In one case multiple abscess was present. Sterile cultures were obtained in 57.1% (8/14) of the abscesses. Anaerobic streptococcus, bacteroides, streptococcus, staphylococcus, G(-) rods were cultured. All of the abscesses were treated by surgical management with the overall mortality of 14.3% (2/14). Total excision of the abscess generally resulted in better outcome. Patients were operated upon in the stabilized stage of the disease clinically with the aid of serial CT scan evidence. The introduction of CT scan has facilitated early diagnosis, location, and investigation of the evolution of a abscess to obtain a better postoperative result. However, recent progress in neurosurgery and cardiac surgery has made it possible to extend the normal life of these patients. Therefore we should have to give our attention to the surgical management of the brain abscess associated with cyanotic congenital heart disease seriously.

State-of-the-Art CT Imaging Techniques for Congenital Heart Disease

구현우 대한영상의학회 2010 Korean Journal of Radiology Vol.11 No.1

CT is increasingly being used for evaluating the cardiovascular structures and airways in the patients with congenital heart disease. Multi-slice CT has traditionally been used for the evaluation of the extracardiac vascular and airway abnormalities because of its inherent high spatial resolution and excellent air-tissue contrast. Recent developments in CT technology primarily by reducing the cardiac motion and the radiation dose usage in congenital heart disease evaluation have helped expand the indications for CT usage. Tracheobronchomalacia associated with congenital heart disease can be evaluated with cine CT. Intravenous contrast injection should be tailored to unequivocally demonstrate cardiovascular abnormalities. Knowledge of the state-of-the-art CT imaging techniques that are used for evaluating congenital heart disease is helpful not only for planning and performing CT examinations, but also for interpreting and presenting the CT image findings that consequently guide the proper medical and surgical management.

Major Adverse Cardiovascular Events in Korean Congenital Heart Disease Patients: A Nationwide Age- and Sex-Matched Case-Control Study

이주성,정진만,최종운,서우근,신홍주 연세대학교의과대학 2022 Yonsei medical journal Vol.63 No.12

Purpose: Congenital heart disease (CHD) is a known risk factor for acquired cardiovascular and cerebrovascular diseases. However, available evidence on CHD is limited mostly to Western populations. This study aimed to evaluate the prevalence of vascular events and all-cause mortality in Korean patients with CHD and to further corroborate CHD as a predictor of vascular events and all-cause mortality. Materials and Methods: The claims data of the Korean National Health Insurance Service (NHIS) were retrospectively reviewed. Information regarding diagnostic codes, comorbidities, medical services, income level, and residential area was also collected. Outcomes of interest included stroke, myocardial infarction (MI), all-cause mortality, and major adverse cardiovascular events(MACE). Results: We included 232203 patients with CHD and 3024633 individuals without CHD as a control group through age- and sex-matched 1:10 random sampling. The prevalences of hypertension, congestive heart failure, ischemic heart disease, hyperlipidemia, and atrial fibrillation were significantly higher in the CHD group, which had a more than two-fold higher incidence of vascular events and all-cause mortality, than in the group without CHD. Multivariable models demonstrated that CHD was a significant risk factor for stroke, MI, all-cause mortality, and MACE. Conclusion: In conclusion, this nationwide study demonstrates that Korean patients with CHD have a high incidence of comorbidities, vascular events, and mortality. CHD has been established as an important predictor of cardiovascular events. Further studies are warranted to identify high-risk patients with CHD and related factors to prevent vascular events.

성인 선천성 심장병 환자의 자가 간호 영향요인 : 질병 관련 지식, 사회적 지지를 중심으로

이지혜(Jihye Lee),박효정(Hyojung Park) 한국산학기술학회 2021 한국산학기술학회논문지 Vol.22 No.8

본 연구는 성인 선천성 심장병 환자의 자가 간호 수준에 영향을 미치는 요인을 질병 관련 지식, 사회적 지지를 중심으로 분석하였다. 방법 : 본 연구는 서술적 상관관계 연구로, 서울시에 소재한 A 종합병원에서 외래 진료를 받고 있는 208명의 환자를 대상으로 자가 간호 수준(SCHFI V6.2), 질병 관련 지식(LKQCHD), 사회적 지지(MSPSS) 수준에 대한 설문조사를 시행하였으며, 수집된 자료의 상관관계분석 결과를 바탕으로 자가 간호 모델을 제시하였다. 결과 : 대상자의 평균 자가 간호 수준은(59.1±11.2), 질병 관련 지식(r=.187, p =.007), 사회적 지지(r=.322, p <.001)와 통계적으로 유의한 상관관계를 보였다. 각 자가 간호 하위 영역인 자가 간호 자신감은 매개 요인으로 작용하여 사회적 지지의 효과를 강화시키는 것으로 확인되었으나(자가 간호 유지: Estimate=.35, p <.05, 심부전 증상 대처: Estimate=.20, p <.05), 질병 관련 지식과 자가 간호 수준 간에는 통계적으로 유의한 영향력이 없었다. 결론 : 선천성 심장병 환자의 자가 간호 수준은 자가 간호 지식에 대한 강화 보다는 사회적 지지를 통한 자신감 형성이 주요 요인으로 작용함을 알 수 있었다. 따라서 효과적인 자가 간호 이행을 위해서는 사회적 지지 강화를 위한 중재가 필요하다. The purpose of this study was to analyze the factors influencing self-care ability in adult congenital heart disease (CHD) patients with a focus on disease-related knowledge and social support. Methods: This study was a descriptive correlation study. Subjects were 208 patients receiving outpatient treatment for CHD at a hospital located in Seoul. The study was carried out using the Self-Care of Heart Failure Index V.6.2(SCHFI V6.2), the Leuven Knowledge Questionnaire for Congenital Heart Disease (LKQCHD), and related knowledge, and the Multidimensional Scale of Perceived Social Support (MSPSS). A self-care model was proposed based on correlation analysis. Results: The average self-care ability (59.1±11.2) was not significantly related to disease-related knowledge (r=.187, p =.007), and social support (r=.322, p <.001). In addition, as a sub-domain, self-care confidence played the role of a mediator (self-care maintenance: estimate=.35, p <.05, heart failure management: estimate=.20, p <.05), whereas disease-related knowledge had no statistically significant effect on the sub-domain of self-care. Conclusion: The results of this study demonstrated a significant correlation of self-care levels with social support through confidence rather than disease-related knowledge in patients with CHD. Therefore, it will be necessary to implement nursing interventions that focus on strengthening social support with the aim of building self-care confidence.