• Clinical and Experimental Pediatrics
• /
• v.58 no.3
• /
• pp.84-88
• /
• 2015

Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.

• Journal of Chest Surgery
• /
• v.37 no.1
• /
• pp.56-63
• /
• 2004

Background: Patch angioplasty is an alternative surgical procedure to coronary artery bypass grafting (CABG) for left main coronary ostial stenosis. The purpose of this study is to evaluate the outcome of patch angioplasty by analyzing the short-term and long-term results. Material and Method: Twenty nine patients who had undergone patch angioplasty due to left main coronary ostial stenosis between July 1991 and May 2003 were enrolled in the study. The mean age of the patients was 53.1 $\pm$12.5 years. There were 8 males and 21 females, and there were 12 female patients who had no risk factor for atherosclerosis. Twenty six (89.7%) patients showed isolated coronary ostial stenosis without any distal coronary lesion. Result: Anterior approach was used in 28 patients and superior approach was used in one patient. Transsection of the main pulmonary artery was used in one patient. Concomitant CABG was performed in 4 patients because of left anterior descending artery lesions in 3 patients and unstable postoperative hemodynamic status in one patient. Hospital mortality had occurred in one patient (3.4%) and late mortality also in one patient, therefore the overall 5 year survival rate was 91.2$\pm$6.1%. Seventeen coronary angiographies were done in 13 patients (44.8%) postoperatively. Two distal patch stenoses, 1 proximal patch stenosis, and 1 new right coronary ostial lesion were identified and 3 percutaneous interventions and 1 CABG were performed during the follow-up period. The overall 5 year freedom from reintervention rate was 82.4 $\pm$ 8.5%. Aortic regurgitation less than grade 1 had developed postoperatively in 4 patients and one patient showed progression of preexisting aortic regurgitation from grade II to III. Conclusion: Patch angioplasty in left main coronary ostial lesion showed acceptable short-term and long-term results in this study. However, restenosis at the patch anastomosis site and aortic regurgitation should be carefully investigated during the follow-up period.

• Clinical and Experimental Pediatrics
• /
• v.46 no.2
• /
• pp.162-166
• /
• 2003

Purpose : We evaluated the effects of intravenous immunoglobulin(IVIG) on level of laboratory parameters examined serially according to the existence of coronary artery lesions in children with Kawasaki disease. Methods : Children with Kawasaki disease(n=63), treated with IVIG at a dose of 2.0 g/kg, were classified as a group with coronary artery lesions(CALs+ group, n=9) or a group without coronary artery lesions(CALs- group, n=54). Levels of various laboratory parameters were determined three times during admission; before, 24 hrs after and 7 days after IVIG administration. Results : There were no significant differences in laboratory parameters performing at, before and 7 days after IVIG administration. However WBC and neutrophil counts, and CRP were significantly higher, and the level of albumin was significantly lower at 24 hrs after IVIG administration. Conclusion : Approximately 15% of patients with Kawasaki disease showed CALs in the acute stage. Kawasaki disease patients with CALs were associated with persistent elevated levels of inflammatory parameters including WBC count, neutrophil count and CRP examined 24 hours after IVIG administration.

• Nuclear Medicine and Molecular Imaging
• /
• v.42 no.1
• /
• pp.77-78
• /
• 2008

A 71-year-old woman was assigned to our department for Tc-99m myocardial perfusion SPECT(MPS) and coronary CT angiography. She admitted for substernal pain, via the ER, 2 days ago. The heart was scanned after intravenous injection of 925 MBq of $^{99m}Tc$-sestamibi adenosine-induced stress SPECT using dual head gamma camera (Hawkeye, GE healthcare. USA). The MPS shows decreased tracer uptake in the apical & mid area of anterior & lateral wall and mid & basal inferior wall. Coronary CT angiograph was obtained using Discovery VCT (GE healthcare). 3D angiography portrayed significant stenosis of ramus intermedius(RI) and posterolateral branch of right coronary artery(PLB) with fibrocalcified plaque. Two images were fused using Cardiac IQ fusion softwear package (Advantage workstation 4.4, GE healthcare) The fusion images explain the perfusion defect of anterior, lateral and inferior wall is due to stenosis of the RI and PLB. And 3 days later, coronary angiography was done and revealed the marked stenosis of RI and PLB. Then balloon angioplasty and stent was instituted in RI. Cardiac SPECT/CT fusion imaging provides additional information about hemodynamic relevance and facilitates lesion interpretation by allowing exact allocation of perfusion defects to its subtending coronary artery.

• Clinical and Experimental Pediatrics
• /
• v.55 no.1
• /
• pp.18-23
• /
• 2012

Purpose: Transforming growth factor beta receptor 2 ($TGFBR2$) is a tumor suppressor gene that plays a role in the differentiation of striated cells and remodeling of coronary arteries. Single nucleotide polymorphisms (SNPs) of this gene are associated with Marfan syndrome and sudden death in patients with coronary artery disease. Cardiovascular remodeling and T cell activation of $TGFBR2$ gene suggest that the $TGFBR2$ gene SNPs are related to the pathogenesis of Kawasaki disease (KD) and coronary artery lesion (CAL). Methods: The subjects were 105 patients with KD and 500 healthy adults as controls. Mean age of KD group was 32 months age and 26.6% of those had CAL. We selected $TGFBR2$ gene SNPs from serum and performed direct sequencing. Results: The sequences of the eleven SNPs in the $TGFBR2$ gene were compared between the KD group and controls. Three SNPs (rs1495592, rs6550004, rs795430) were associated with development of KD ($P$=0.019, $P$=0.026, $P$=0.016, respectively). One SNP (rs1495592) was associated with CAL in KD group ($P$=0.022). Conclusion: Eleven SNPs in $TGFBR2$ gene were identified at that time the genome wide association. But, with the change of the data base, only six SNPs remained associated with the $TGFBR2$ gene. One of the six SNPs (rs6550004) was associated with development of KD. One SNP associated with CAL (rs1495592) was disassociated from the $TGFBR2$ gene. The other five SNPs were not functionally identified, but these SNPs are notable because the data base is changing. Further studies involving larger group of patients with KD are needed.

• The Korean Journal of Nuclear Medicine Technology
• /
• v.14 no.2
• /
• pp.33-37
• /
• 2010

Purpose: In the early stage of using PET/CT, it was used to damper revision but recently shows that CT with MDCT is commonly used and works well for an anatomical diagnosis. This hospital makes the accuracy and convenience more higher in the diagnosis and evaluate of coronary heart disease through concurrently running myocardial perfusion SPECT examination, myocardial PET examination with FDG, and CT coronary artery CT angiography(coronary CTA) used PET/CT with 64-slice. This report shows protocol and image based on results from about 400 coronary heart disease examinations since having 64 channels PET/CT in July 2007. Materials and Methods: An Equipment for this examination is 64-slice CT and Discovery VCT (DVCT) that is consisted of PET with BGO ($Bi_4Ge_3O_{12}$) scintillation crystal by GE health care. First myocardial perfusion SPECT with pharmacologic stress test to reduce waiting time of a patient and get a quick diagnosis and evaluation, and right after it, myocardial FDG PET examination and coronary CTA run without a break. One-stop evaluation protocol of ischemic heart disease is as follows. 1)Myocardial perfusion SPECT with pharmacologic stress: A patient is injected with $^{99m}Tc$-MIBI 10 mCi and does not have any fatty food for myocardial PET examination and drink natural water with ursodeoxcholic acid 100 mg and we get SPECT image in an hour. 2)Myocardial FDG PET: To reduce blood fatty content and to increase uptake of FDG, we used creative oral glucose load using insulin and Acipimox to according to blood acid content. A patient is injected with $^{18}F$-FDG 5 mCi for reduction of his radiation exposure and we get a gated image an hour later and get delay image when we need. 3) Coronary CTA: The most important point is to control heart rate and to get cooperation of patient's breath. In order to reduce a heart rate of him or her below 65 beats, let him or her take beta blocker 50 mg ~ 200 mg after a consultation with a doctor about it and have breath-practices then have the examination. Right before the examination, we spray isosorbide dinitrate 3 to 5 times to lower tension of bessel wall and to extension a blood wall of a patient. It makes to get better the shape of an anatomy. At filming, a patient is injected CT contrast with high pressure and have enough practices before the examination in order to have no problem. For reduction of his radiation exposure, we have to do ECG-triggered X-ray tube modulation exposure. Results: We evaluate coronary artery stenosis through coronary CTA and study correlation (culprit vessel check) of a decline between stenosis and perfusion from the myocardial perfusion SPECT with pharmacologic stress, coronary CTA, and can check viability of infarction or hibernating myocardium by FDG PET. Conclusion: The examination makes us to set up a direction of remedy (drug treatment, PCI, CABG) because we can estimate of effect from remedy, lesion site and severity. In addition, we have an advantage that it takes just 3 hours and one-stop in that all of process of examinations run in succession and at the same time. Therefore it shows that the method is useful in one stop evaluation of ischemic heart disease.

• 대한핵의학회:학술대회논문집
• /
• 2001.05a
• /
• pp.24-34
• /
• 2001

Restenosis remains a major limitation of percutaneous coronary interventions. Numerous studios including pharmacological approaches and new devices failed to reduce the restenosis rate except coronary stenting. Since the results of $BENESTENT^{1)}\;and\;STRESS^{2)}$ studies came out, coronary stenting has been the most popular interventional strategy in the various kinds of coronary stenotic lesions, although the efficacy of stenting was shown only in the discrete lesion of the large coronary artery. The widespread use of coronary stenting has improved the early and late outcomes after coronary intervention, but it has also led to a new and serious problem, e.g., in-stent restenosis. Intravascular radiation for prevention of restenosis is a new technology in the field of percutaneous coronary intervention. Recent animal experiments and human trials have demonstrated that local irradiation, in conjunction with coronary interventions, substantially diminished the rate of restenosis. This paper reviews basic radiation biology of intracoronary radiation and its role in the inhibition of restenosis. The current status of intracoronary radiation therapy using Re-188 liquid balloon is also discussed.

• Journal of Chest Surgery
• /
• v.25 no.9
• /
• pp.925-929
• /
• 1992

Supravalvular aortic stenosis is relatively uncommon form of congenital heart disease and the most important lesion of this anomaly is various narrowing of the aortic lumen just above the sinus of Valsalva. We experienced a case of hourglass type of supravalvular aortic stenosis involving lcm from length from lcm above the sinus of Valsalva. The patient was associated with mental retardation, peculiar facies and dental anomaly. The diagnosis was confirmed preoperatively by retrograde left heart catheterization and left ventriculography. An incision was made in the ascending aorta and into the right coronary and noncornary sinus. Care was taken to protect the right coronary artery. A Y-shaped patch of Dacron was made to enlarge the stenotic portion of aorta. Postoperative pressure gradient between the aorta and left ventricle markedly reduced 36 mmHg in comparison with preoperative pressure gradient 150mmHg. The boy was discharged without any event.

• Clinical and Experimental Pediatrics
• /
• v.59 no.7
• /
• pp.298-302
• /
• 2016

Purpose: Recent reports showed that plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) could be a useful biomarker of intravenous immunoglobulin (IVIG) unresponsiveness and coronary artery lesion (CAL) development in Kawasaki disease (KD). The levels of these peptides are critically influenced by age; hence, the normal range and upper limits for infants and children are different. We performed an age-adjusted analysis of plasma NT-proBNP level to validate its clinical use in the diagnosis of KD. Methods: The data of 131 patients with KD were retrospectively analyzed. The patients were divided into 2 groups-group I (high NT-proBNP group) and group II (normal NT-proBNP group)-comprising patients with NT-proBNP concentrations higher and lower than the 95th percentile of the reference value, respectively. We compared the laboratory data, responsiveness to IVIG, and the risk of CAL in both groups. Results: Group I showed significantly higher white blood cell count, absolute neutrophil count, C-reactive protein level, aspartate aminotransferase level, and troponin-I level than group II (P<0.05). The risk of CAL was also significantly higher in group I (odds ratio, 5.78; P=0.012). IVIG unresponsiveness in group I was three times that in group II (odds ratio, 3.35; P= 0.005). Conclusion: Age-adjusted analysis of plasma NT-proBNP level could be helpful in predicting IVIG unresponsiveness and risk of CAL development in patients with KD.

• Journal of the Korea Computer Graphics Society
• /
• v.23 no.1
• /
• pp.49-55
• /
• 2017

Computed tomographic angiography (CTA) is widely used in the diagnosis and treatment of coronary artery disease because it shows not only the whole anatomical structure of the cardiovascular three-dimensionally but also provides information on the lesion and type of plaque. However, due to the large size of the image, there is a limitation in manually extracting coronary arteries, and related researches are performed to automatically extract coronary arteries accurately. As the coronary artery originate from the ascending aorta, the ascending aorta and ostium should be detected to extract the coronary tree accurately. In this paper, we propose an automatic segmentation for the ostium as a starting structure of coronary artery in CTA. First, the region of the ascending aorta is initially detected by using Hough circle transform based on the relative position and size of the ascending aorta. Second, the volume of interest is defined to reduce the search range based on the initial area. Third, the refined ascending aorta is segmented by using a two-dimensional geodesic active contour. Finally, the two ostia are detected within the region of the refined ascending aorta. For the evaluation of our method, we measured the Euclidean distance between the result and the ground truths annotated manually by medical experts in 20 CTA images. The experimental results showed that the ostia were accurately detected.