• Title/Summary/Keyword: Congenital Heart Disease

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Vasopressin in Young Patients with Congenital Heart Defects for Postoperative Vasodilatory Shock (선천성 심장병 수술 후 발생한 혈관확장성 쇼크에 대한 바소프레신의 치료)

  • 황여주;안영찬;전양빈;이재웅;박철현;박국양;한미영;이창하
    • Journal of Chest Surgery
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    • v.37 no.6
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    • pp.504-510
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    • 2004
  • Background: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardio-pulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. Material and Method: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2∼41 days) and median body weight was 2,870 grams (ranges; 900∼3,530 grams). Preoperative diag-noses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. Result: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002∼0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26∼140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7$\pm$7.4 mmHg, 53.7$\pm$11.4 mmHg, and 56.3$\pm$13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3$\pm$7.2, 21.0$\pm$8.4, and 21.2$\pm$8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin admin- istration. Conclusion: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.

Screening Test for Heart Diseases in the First Grade Elementary School Children in Busan (부산 지역 초등학교 1학년 학생들의 심장질환의 집단검진에 관한 연구)

  • Oh, Jae Min;Park, Hee Ju
    • Clinical and Experimental Pediatrics
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    • v.46 no.5
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    • pp.490-494
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    • 2003
  • Purpose : We'd like to determine the incidence of congenital heart disease and arrhythmia in elementary school children in Busan, and to provide adequate prevention and treatment. Methods : A total of 23,802(male 12,909, female 10,893) 1st grade elementary school children living in Busan were studied. All children were 7-8 years old. We obtained their medical history by questionnaire and checked elecrocardiography(ECG). Subsequent screening tests including a 2nd ECG, chest X-ray, phonocardiogram and CBC for the students who had abnormal findings at the first screening test. The third screening test was done for students who had cardiac murmurs or abnormal ECG findings in the second screening test by echocardiogram, treadmill test and 24-hour Holter monitoring. Results : Among 23,802 children participants, 605(2.54%) had abnormal ECG findings at the first screening test. Q wave abnormality(0.58%) was observed most frequently, and complete right bundle branch block(RBBB)(0.26%), sinus tachycardia(0.24%), right axis deviation(0.22%) and ventricular premature contraction(VPC)(0.21%) followed in order. Four hundred and twenty four children participated in the second ECG screening test. Two hundred and two children(47.6%) had an abnormality such as sinus tachycardia(18.8%), VPC(17.8%), or complete RBBB(17.3%). After completing the third examination including echocardiogram, we couldn't find the students with ventricular tachycardia (VT) or SVT at the third arrhythmia screening test. Conclusion : A high incidence of arrhythmia was found in the 1st grade elementary school children in Busan despite their healthy appearances, although fatal heart diseases were not noted. Early diagnosis, adequate preventative measures and treatment will prevent and decrease the frequency of emergent situations like syncope and sudden death.

Follow-Up of Residual Shunt after Repair of Ventricular Septal Defect (심실중격결손 봉합 후 잔류 단락의 추적관찰)

  • 정태은;이장훈;김도형;백종현;이동협;이정철;한승세;이영환
    • Journal of Chest Surgery
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    • v.35 no.8
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    • pp.580-583
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    • 2002
  • Background: Obtaining precise hemodynamic and morphological information in the early postoperative period after surgical correction of congenital heart disease is important in determining the need for future medical or surgical intervention. We investigated the residual shunting after surgical repair of simple ventricular septal defect in order to know the incidence of residual shunting in the postoperative period and the natural history of small residual shunts located in the peripatch area. Material and Method: Forty three consecutive patients under one year of age who underwent patch repair of a simple ventricular septal defect were evaluated for incidence of residual shunts by echocardiography. Result: Eleven patients had echocardiographic residual shunt in the peripatch area at immediate postoperative period, however, there were no patients who needed reoperation due to deteriorated hemodynamic effect of residual shunt. The incidence of residual shunts was not significantly different with type of ventricular septal defect and material used for closure. During follow up period, two patients were lost and remaining nine patients no longer showed evidence of residual shunt. The mean time of last evidence of shunt was $4.2{\pm}3.6$ months after operation. Conclusion: Residual peripatch shunt flow was frequently noted in the immediate postoperative period following surgical repair of ventricular septal defect, however, most of them were disappeared within six months.

Identification and Assessment of Paradoxical Ventricular Wall Motion Using ECG Gated Blood Pool Scan - Comparison of Cine Loop, Phase Analysis and Paradox Image - (ECG Gated Blood Pool Scan을 이용한 심실벽 역행성 운동의 평가 - Cine Loop, Phase Analysis, Paradox Image의 비교 -)

  • Lee, Jae-Tae;Kim, Gwang-Weon;Jeong, Byeong-Cheon;Lee, Kyu-Bo;Whang, Kee-Suk;Chae, Sung-Chul;Jeon, Jae-Eun;Park, Wee-Hyun;Lee, Hyong-Woo;Chung, Jin-Hong
    • The Korean Journal of Nuclear Medicine
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    • v.24 no.2
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    • pp.244-253
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    • 1990
  • Sixty-four patients with paradoxical ventricular wall motion noticed both in angiocardiography or 2-dimensional echocardiography were assessed by ECG gated blood pool scan (GBPS). Endless cine loop image, phase and amplitude images and paradox image obtained by visual inspection of each cardiac beat or Fourier transformation of acquired raw data were investigated to determine the incremental value of GBPS with these processing methods for identification of paradoxical ventricular wall motion. The results were as follows: 1) Paradoxical wall motions were observed on interventricular septum in 34 cases, left ventricular free wall in 26 and right ventricular wall in 24. Underlying heart diseases were ischemic (23 cases) valvular(9), congenital heart disease (12), cardiomyopathy (5). pericardial effusion(5), post cardiac surgery(3), col pulmonale (2), endocarditis(1) and right ventricular tumor(1). 2) Left ventricular ejection fractions of patients with paradoxical left ventricular wall motion were significantly lower than those with paradoxical septal motion(p<0.005). 3) The sensitivity of each processing methods for detecting paradoxical wall motion was 76.9% by phase analysis, 74.6% by endless cine loop mapping and 68.4% by paradox image manipultion respectively. Paradoxial motions visualized only in phase, paradox or both images were appeared as hypokinesia or akinesia in cine loop image. 4) All events could be identified by at least one of above three processing methods, however only 34 cases (48.4%) showed the paradoxical molies in all of the three images. By these findings, we concluded that simultaneous inspection of all above three processing methods-endless cine loop, phase analysis and paradox image-is necessary for accurate identification and assessment of paradoxical ventricular wall motion when performing GBPS.

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Clinical Analysis of Patch Repair of Ventricular Septal Defect in Infant (영아기 심실중격결손 봉합술의 임상적 고찰)

  • Jung, Tae-Eun;Lee, Jang-Hoon;Lee, Dong-Hyup;Lee, Jung-Cheul;Han, Sung-Sae;Kim, Sae-Yeun;Ji, Dae-Lim
    • Journal of Yeungnam Medical Science
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    • v.19 no.2
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    • pp.99-106
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    • 2002
  • Background: Simple ventricular septal defect(VSD) is the most common congenital heart disease. Although closure of VSD is currently associated with a relatively low risk, experience with younger and smaller infants has been variably less satisfactory. We assessed the results of surgical closure of VSD in infant. Materials and Methods: Between 1996 and 2000, 45 non-restrictive VSD patients underwent patch repair and retrospective analysis was done. Patients were divided into two groups based on weight: group I infants weighed 5kg or less(n=16), and group II infants weighed more than 5kg(n=29). Both groups had similar variation in sex, VSD location, aortic cross clamp time and total bypass time. But combined diseases (ASD, PDA, MR) were more in group I. We closed VSD with patch and used simple continuous suture method in all patients. Results: There were no operative mortality, no reoperation for hemodynamically significant residual shunt and no surgically induced complete heart block. As a complication, pneumonia(group I: 2 cases, group II: 2 cases), transient seizure(group II: 2), wound infection(group I: 1, group II: 1), urinary tract infection(group I: 1) and chylopericardium(group I: 1) developed, and there was no significant difference between two groups(p>0.05). Conclusion: Early primary closure with simple continuous suture method was applicable in all patients with non-restrictive VSD without any serious complications.

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Lung cancer, chronic obstructive pulmonary disease and air pollution (대기오염에 의한 폐암 및 만성폐색성호흡기질환 -개인 흡연력을 보정한 만성건강영향평가-)

  • Sung, Joo-Hon;Cho, Soo-Hun;Kang, Dae-Hee;Yoo, Keun-Young
    • Journal of Preventive Medicine and Public Health
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    • v.30 no.3 s.58
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    • pp.585-598
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    • 1997
  • Background : Although there are growing concerns about the adverse health effect of air pollution, not much evidence on health effect of current air pollution level had been accumulated yet in Korea. This study was designed to evaluate the chronic health effect of ai. pollution using Korean Medical Insurance Corporation (KMIC) data and air quality data. Medical insurance data in Korea have some drawback in accuracy, but they do have some strength especially in their national coverage, in having unified ID system and individual information which enables various data linkage and chronic health effect study. Method : This study utilized the data of Korean Environmental Surveillance System Study (Surveillance Study), which consist of asthma, acute bronchitis, chronic obstructive pulmonary diseases (COPD), cardiovascular diseases (congestive heart failure and ischemic heart disease), all cancers, accidents and congenital anomaly, i. e., mainly potential environmental diseases. We reconstructed a nested case-control study wit5h Surveillance Study data and air pollution data in Korea. Among 1,037,210 insured who completed? questionnaire and physical examination in 1992, disease free (for chronic respiratory disease and cancer) persons, between the age of 35-64 with smoking status information were selected to reconstruct cohort of 564,991 persons. The cohort was followed-up to 1995 (1992-5) and the subjects who had the diseases in Surveillance Study were selected. Finally, the patients, with address information and available air pollution data, left to be 'final subjects' Cases were defined to all lung cancer cases (424) and COPD admission cases (89), while control groups are determined to all other patients than two case groups among 'final subjects'. That is, cases are putative chronic environmental diseases, while controls are mainly acute environmental diseases. for exposure, Air quality data in 73 monitoring sites between 1991 - 1993 were analyzed to surrogate air pollution exposure level of located areas (58 areas). Five major air pollutants data, TSP, $O_3,\;SO_2$, CO, NOx was available and the area means were applied to the residents of the local area. 3-year arithmetic mean value, the counts of days violating both long-term and shot-term standards during the period were used as indices of exposure. Multiple logistic regression model was applied. All analyses were performed adjusting for current and past smoking history, age, gender. Results : Plain arithmetic means of pollutants level did not succeed in revealing any relation to the risk of lung cancer or COPD, while the cumulative counts of non-at-tainment days did. All pollutants indices failed to show significant positive findings with COPD excess. Lung cancer risks were significantly and consistently associated with the increase of $O_3$ and CO exceedance counts (to corrected error level -0.017) and less strongly and consistently with $SO_2$ and TSP. $SO_2$ and TSP showed weaker and less consistent relationship. $O_3$ and CO were estimated to increase the risks of lung cancer by 2.04 and 1.46 respectively, the maximal probable risks, derived from comparing more polluted area (95%) with cleaner area (5%). Conclusions : Although not decisive due to potential misclassication of exposure, these results wert drawn by relatively conservative interpretation, and could be used as an evidence of chronic health effect especially for lung cancer. $O_3$ might be a candidate for promoter of lung cancer, while CO should be considered as surrogated measure of motor vehicle emissions. The control selection in this study could have been less appropriate for COPD, and further evaluation with another setting might be necessary.

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A Trend for Atroventricular Valve Regurgitation after a Modified Fontan Operation (변형 폰탄 수술 시행 이후에 방실 판막 폐쇄부전의 변화 양상)

  • Lim, Hong-Gook;Lee, Chang-Ha;Seo, Hong-Joo;Kim, Woong-Han;Hwang, Seong-Wook;Lee, Cheul
    • Journal of Chest Surgery
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    • v.41 no.3
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    • pp.305-312
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    • 2008
  • Background: Anatomic and functional abnormalities of the systemic atrioventricular (AV) valve are common in single ventricle. pathologies and continue to be associated with poor early and late outcomes in surgically palliated single. ventricle patients. We aggressively performed valvuloplasty for atrioventricular valve regurgitation (AVVR) during the course toward a Fontan operation. Material and Method: Between January 1995 and December 2004, 209 patients underwent a Fontan operation in our institution. We retrospectively evaluated the prevalence of AVVR and the influence of AV valve repair on outcome, and we analyzed the progression of AVVR after the Fontan operation for 168 patients where echocardiographic follow up results for more than 6 months after the Fontan operation were available. During the course toward a Fontan operation, 25 patients underwent 30 procedures for AVVR. These procedures. were. carried out during placement of a bidirectional cavopulmonary shunt (BCPS) for nine patients, between the time of placement of a BCPS and the Fontan operation for four patients, and during the Fontan operation for 17 patients. Five patients underwent procedures for AVVR twice. Result: The late mortality rate after the Fontan operation was 4.2% (n=7), with a median follow-up duration of 52 months (range, $6{\sim}123$ months). Seven patients (4%) had unfavorable outcomes such as significant (moderate or severe) AVVR in six patients, and significant AV valve stenosis in one patient was determined at the last follow up after the Fontan operation. Among the seven patients, four patients underwent AV valve repair after the Fontan operation, and one patient underwent subsequent AV valve replacement. Progression to AVVR of equal to or greater than grade 2 was noted in 30 patients (18%) at the last follow up after the Fontan operation, including 12 patients that underwent previous AV valve procedures. Initial grading of AVVR, a previous AV valve operation, and specific AV valve morphology such as a common AV valve or mitral atresia were significant risk factors for the progression of AVVR after the Fontan operation. Conclusion: In our surgical series, a small percentage of patients showed unfavorable outcomes. related to AVVR during the course toward a Fontan operation. However, a closer follow-up is required to evaluate the progression of the AVVR after a Fontan operation, especially for patients showing poor AV valve function at the first presentation and specific AV valve morphology.

Pediatric Peritoneal Dialysis in Korea : Practical Solution to the Problems of Peritoneal Dialysis for Children (한국 소아복막투석의 현황 - 다기관 공동연구 결과보고 -)

  • Kim Pyung-Kil
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.95-103
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    • 1998
  • Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.

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Fatal plastic bronchitis with eosinophilic casts in a previously healthy child (건강하였던 소아에서 발생한 치명적인 호산구성 증식성 기관지염)

  • Cho, Young Kuk;Oh, Soo Min;Choi, Woo-Yeon;Song, Eun Song;Han, Dong-Kyun;Kim, Young-Ok;Ma, Jae Sook
    • Clinical and Experimental Pediatrics
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    • v.52 no.9
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    • pp.1048-1052
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    • 2009
  • Plastic bronchitis is a rare disease characterized by the recurrent formation of branching mucoid bronchial casts that are large and more cohesive than those that occur in ordinary mucus plugging. Casts may vary in size and can be spontaneously expectorated, but some require bronchoscopy for removal. Plastic bronchitis can therefore present as an acute life-threatening emergency if obstruction of the major airways occurs. Three of 22 reported patients with eosinophilic casts were fatal, with death due to central airway obstruction. Here, we report a child with no history of atopy, allergy, or congenital heart disease who was diagnosed with plastic bronchitis with eosinophilic casts. Although he was administered intravenous (iv) antibiotics; iv corticosteroids; and a vigorous pulmonary toilet regimen, including chest physiotherapy and routine bronchoscopic removal of casts, he had brain death secondary to hypoxic brain damage. Plastic bronchitis can be fatal when casts obstruct the major airways, as in the present case. Clinicians should intervene early if a patient exhibits signs and symptoms consistent with plastic bronchitis.

Outcome of the Modified Norwood Procedure: 7 Years of Experience from a Single Institution (변형된 노우드(Norwood) 수술의 결과: 단일병원의 지난 7년간의 경험)

  • Kim, Hyung-Tae;Sung, Si-Chan;Kim, Si-Ho;Bae, Mi-Ju;Lee, Hyoung-Doo;Park, Ji-Ae;Chang, Yun-Hee
    • Journal of Chest Surgery
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    • v.43 no.4
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    • pp.364-374
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    • 2010
  • Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, $11.7{\pm}13.2days$) and weight at operation ranged from 2.2 to 4.8 kg (mean, $3.17{\pm}0.52kg$). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean $26.0{\pm}22.8months$). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.