Clinical Analysis of Surgical Results for Discrete Subaortic Stenosis

Background: Discrete subaortic stenosis is known to recur frequently even after surgical resection. We retrospectively reviewed the preoperative and postoperative changes in pressure gradient through left ventricular outflow tract, and the recurrence rate. Material and Method: Between September 1984 and December 2004, 34 patients underwent surgical treatment. Mean age of patients was 17.1±15.2 years and 19 patients (55.9%) were male. 16 patients (47.1%) had previous operations and associated diseases were aortic regurgitation (11), coarctation of aorta (3), and others. Result: Immediate postoperative peak pressure gradient was significantly lower than preoperative peak pressure gradient (21.8 mmHg vs 75.8 mmHg, p＜0.01). Peak pressure gradient measured after 50.3 months of follow up was 20.2 mmHg, which was also significantly lower than that of preoperative value but not significantly different from that of immediate postoperative value. There was no surgical mortality but one patient developed cerebral infarction. Mean follow up duration was 69.8±54.6 months. During this period, 5 patients (14.7%) had reoperation, 3 (8.8%) of whom were due to recurred subaortic stenosis. We found no risk factors for recurrence and survival for free from reoperation was 76.4%. Conclusion: Excision of subaortic membrane combined with or without myectomy in discrete subaortic stenosis showed sufficient relief of left ventricular outflow tract obstruction with low mortality and morbidity, but careful long term follow up is necessary for recurrence, since it is not predictable.ope

Morphologic Changes of the Pulmonary Arteries after Stent Implantation on Branch Pulmonary Artery Stenosis- Impact of Pulmonary Insufficiency -

Purpose: Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. Methods: The subjects in our study were 23 children who had undergone stent implantation from Jan. 1995 to Jul. 2002 in Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. Results: The CSAI of RPA without stenosis changed from mean 238±17 ㎟/BSA to mean 249±20 ㎟/BSA(P=0.47), but didn't regress. The CSAI of the PLA with stenosis was increased effectively by stent implantation from the mean 102±12 ㎟/BSA to mean 125±11 ㎟/BSA(P<0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. Conclusion: In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stage of stent implantation. So, we suspect that the pulmonary regurgitation remaining after post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPAope

Clinical Experiences for Primary Cardiac Tumors

BACKGROUND: Primary cardiac tumors are known to be rare. We studied the surgical results for primary cardiac tumors. MATERIAL AND METHOD: Between August 1980 and December 2003, we classified 86 patients who had operation for primary cardiac tumors in our center into 3 groups; myxoma, nonmyxoma benign tumors, and malignant tumors. The mean age was 44.3+/-20.8 years and 59 patients (66.3%) were female. In postoperative pathologic diagnosis, there were 81 cases (94.2%) of benign tumors in which myxoma was the most common tumor (70 cases, 78.7%); 5 fibroma (5.6%), 3 rhabdomyoma (3.4%), and 5 malignant tumors (5.8%). RESULT: 86.4% of benign tumor was myxoma and the mean age was 50.4+/-15.4 (range 7~80) years. Tumor was more common in females (49 cases) and most common preoperative symptom was dyspnea (62.9%). 57 cases were located at left atrial septum and only one case, which was located at right ventricular septum, was resected incompletely. There were no hospital deaths and one patient had mitral valve replacement on the first operative day due to newly developed postoperative mitral regurgitation. The mean follow up period was 109.3+/-71.8 months and there was no evidence of recurrence in this period. 11 cases (12.8%) were nonmyxoma benign tumors; 5 fibromas, 3 rhabdomyomas, etc. There were two hospital deaths and the causes of death were fungal endocarditis and hypoxia. There were no reoperations in nonmyxoma benign tumors. Malignant tumors were in 5 cases (5.8%); undifferentiated sarcoma in 2, rhabdomyosarcoma in 1, etc. Although there were no hospital mortalities, 3 patients who were followed up died from complications of tumors. CONCLUSION: Myxomas showed very excellent prognosis after complete resection and nonmyxoma benign tumors showed relatively good results for relief of symptoms. Surgery helped to relieve symptoms for malignant tumors, but the prognosis was poor.ope

Cerebral glucose metabolism analysis by using 18FDG-PET and SPM in children with developmental delay and epilepsy

의학과/석사[한글] (연구배경 및 목적)간질은 간질뿐만 아니라 학습장애, 기억령장애, 공격성 성향 등 정신과적인 문제를 동반하는 경향이 많으며, 발달지연등과도 연관 있는 복합적인 질환이다. 간질병소의 조직적인 이상뿐만 아니라 국소적인 당대사의 저하를 18FDG-PET 에서 확인할 수 있으며 이런 당대사의 저하가 기능적 결함과 연관되어 있다는 보고들이 있다. 이에 소아에서 발달지연을 동반한 간질환자의 당대사를 확인하여 특정부위의 당대사의 저하가 발달지연을 동반한 간질환아에서 어떤 의미를 가지는지 평가하는 것이 이 연구의 목적이다. (연구대상 및 방법)연세대학교 의과대학 세브란스병원 소아과에서 치료 받고 있는 정상 발달을 보인 간질환아 10명, 발달지연 13명, 발달지연을 동반한 간질환아 10명을 대상으로 발달평가 및 18FDG-PET 를 시행하고 이 결과를 정상 성인 대조군 7명의 결과와 SPM을 이용하여 비교 분석하였다. (결과)1. 정상 발달을 보인 간질환자군 10명의 평균나이는 12.8세 남녀 비는 6:4였으며 , 발달지연군 13명 평균 9.7세 남녀비는 10:3, 발달지연을 동반한 간질환자군 10명의 평균나이 10.4세였으며 남녀 비는 7:3였다. 발달지연 군과 발달지연을 동반한 간질환아의 발달지수는 각각 53.5, 53.8 이었다. 2. 정상발달을 보인 간질환아군에서는 뇌간과 parahippocampal gyrus를 중심으로 당대사의 감소가 있었으며 발달지연만 있었던 환아는 cingulate gyrus와 변연계에 유의한 당대사의 감소가 있었고 발달지연과 간질을 동반한 환아에서는 정상발달을 보인 간질환아군과 유사한 형태를 보였으나 변연계를 포함한 전두엽의 당대사의 저하가 뚜렷하였다. (결론)이상의 연구 결과로 발달지연이 동반된 간질환아에서 정상발달을 보인 간질환아군과 구분이 되는 변연계 및 전두엽의 당대사의 저하가 있었다. 이는 간질이 지속될수록 대뇌피질 특히 전두엽의 기능적 손상이 대뇌피질 하부구조와의 연결망 손상과 연관되어 인지 및 발달지연에 중요한 역할을 하는 것으로 추정된다. [영문](Purpose) To evaluate brain regions with abnormal function in children with developmental delay and epilepsy by using 2-deoxy-2-［18F］fluoro-D-glucose (FDG) positron emission tomography(PET) and statistical parametric mapping(SPM). (Methods) Detailed psychodevelopmental assessment and FDG-PET scanning were performed on 10 children aged between 6.3 and 12.8 years ( mean age 10.4 years) old with developmental delay and epilepsy. We applied the objective technique of statistical parametric mapping (SPM) to define focal abnormalities of glucose metabolism, and compared with those of a group of normal adult subjects (n=7, mean age, 29 years) as well as age-matched children with developmental delay but without epilepsy (n = 13, mean age 9.7 years) and epilepsy without developmental delay (n = 10, mean age 12.8 years). (Results) SPM analysis in the group showing developmental delay with epilepsy, revealed extensive glucose hypometabolism in the anterior cingulate, limbic lobe and frontal areas when compared with that in normal adult control group. In children with development delay without epilepsy, hypometabolism were noted in areas of cingulate gyrus and limbic lobe. Comparing children with both developmental delay and epilepsy and those with epilepsy only, reduced glucose metabolism showed similar pattern but hypometabolism in the anterior cingulate, limbic lobe and frontal areas were prominent in the former group only. (Conclusions) Hypometabolism in the anterior cingulate, limbic lobe with frontal glucose hypometabolism in children with developmental delay and epilepsy may be related to decreased cognitive function and widespread dysfunction of cortical regions.ope