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1/14. adult type anomalous origin of the left coronary artery from the main pulmonary artery: report of one case.

    Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare disease. Most of the cases suffer from myocardial infarction, congestive heart failure or even death during the early infantile period, and thus are called "infantile type". We present one five-year-old girl with ALCAPA syndrome, adult type, who was initially suspected to be a case of atypical Kawasaki disease (KD) complicated with coronary arterial aneurysms. Due to persistent dilatation of the coronary artery after medical treatment for one year and a turbulent flow noticed between the left coronary artery (LCA) and the main pulmonary artery (MPA), cardiac catheterization was arranged. The diagnosis was established from the coronary cineangiography in spite of a direct connection between the aorta and LCA revealed from the echocardiogram. Thereafter open heart surgery was undertaken. diagnosis of ALCAPA syndrome can be made by echocardiogram, but this is not fully satisfactory. Selective coronary cineangiography remains the definitive diagnosis. Although the patients of adult type are more asymptomatic, surgical correction should be undertaken as soon as the diagnosis is established since a substantial risk is still present.
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2/14. Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study.

    BACKGROUND: The purpose of this study was to assess the sequential follow-up results of catheter intervention in Kawasaki disease by use of quantitative coronary angiography (QCA) and intravascular ultrasound imaging. methods AND RESULTS: Catheter intervention was performed on 23 stenotic lesions in 22 patients (aged 2 to 24 years). Percutaneous balloon angioplasty (PBA) was performed in 4 patients, stent implantation in 7, percutaneous transluminal coronary rotational ablation (PTCRA) in 10, and a combination of PTCRA with stent implantation in 2. A total of 21 lesions (91%) were successfully dilated by catheter intervention without major or minor complications. One patient immediately underwent coronary artery bypass grafting (CABG) surgery because stent implantation failed to resolve his lesion. At 4 to 6 months after catheter intervention, 2 restenotic lesions (9%) were detected by QCA in 2 patients who had undergone PBA, and these patients subsequently underwent CABG surgery. In 6 months to 3 years after catheter intervention, no patients showed evidence of ischemic findings. At 3 to 4 years after catheter intervention, QCA and intravascular ultrasound studies were performed on 15 lesions in 14 patients. Two restenotic lesions (13%) were detected by QCA in 2 patients. One of the 2 had stent implantation and underwent CABG surgery, and the other had undergone PTCRA and underwent re-PTCRA. Thirteen patients demonstrated no ischemic findings at 3 to 8 years after catheter intervention. CONCLUSION: Catheter intervention for Kawasaki disease can be accomplished and can be effective in the short term, but the long-term efficacy should be verified by further study.
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3/14. acetylcholine-induced coronary spasm with a history of Kawasaki disease: case report.

    A 21-year-old woman without any known coronary risk factors was found at coronary catheterization to have normal coronary angiograms, but demonstrated acethylcholine (ACh)-induced coronary spasm. She had a history of Kawasaki disease (KD) at 19 months of age and, although coronary angiography was not performed at that time, no coronary aneurysms were detected by echocardiography. To the best of our knowledge, this is the first case report of ACh-induced coronary spasm associated with normal coronary angiograms in a young person with a history of KD. The findings suggest that subclinical, persistent coronary endothelial dysfunction may exist in this patient; furthermore, the dysfunction appears diffuse and might be unrelated to coronary aneurysm formation. The long-term significance of coronary endothelial dysfunction in patients with KD, as suspected by coronary spasm, remains unknown but may be an important risk factor for future atherosclerosis.
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4/14. Successful catheter interventional therapy for acute coronary syndrome secondary to kawasaki disease in young adults.

    acute coronary syndrome occurred in 2 young adults who had a history of Kawasaki disease (KD), but few other coronary risk factors. The first patient was a 27-year-old male with acute myocardial infarction without stenosis detected by coronary arteriography 4 years earlier. Emergency coronary arteriography showed occlusion of the right coronary artery. Aspiration-thrombectomy and rescue balloon angioplasty were successfully performed. The second patient was a 32-year-old male with unstable angina. Right coronary arteriography showed total occlusion with severe calcification. Left coronary arteriography showed 99% stenosis at the proximal site of the circumflex artery, and a directional coronary atherectomy was performed. Histological examination of a specimen from this site revealed a lipid core, macrophages, and smooth muscle cells. Restenosis was not observed on follow-up coronary arteriography after 5-6 months in either case. The coronary stenosis in each case was probably caused by accelerated atherosclerosis at the site without aneurysm as it seemed to be 'normal' on arteriography. Conventional catheter intervention was effective treatment. The sequelae of KD should be recognized as independent coronary risk factors.
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5/14. Percutaneous transluminal coronary angioplasty in a patient with Kawasaki disease. A case report of an unsuccessful angioplasty.

    A 13-year-old boy with severe coronary stenosis due to Kawasaki disease underwent percutaneous transluminal coronary angioplasty (PTCA). The guide wire and the balloon catheter easily passed through the stenosis in the left anterior descending artery. However, effective dilatation could not be achieved even when the balloon size was increased to 2.5 mm in diameter. We discontinued further inflation of the balloon because serious resistance was encountered on withdrawal of the balloon catheter. In patients with Kawasaki disease, the value of PTCA as a treatment for coronary stenosis is questionable.
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6/14. Noninvasive assessment of myocardium at risk by Tl-201 SPECT imaging in a child with Kawasaki disease. A case report.

    Serial two-dimensional echocardiograms documented the formation of giant aneurysms, measuring from 0.55 to 3.1 cm in diameter, in the distribution of the left and right coronary arteries, in a 2 1/2-year-old boy with Kawasaki disease. His global left ventricular function, assessed by M-mode echocardiography, was normal, and no significant wall motion abnormalities could be detected on two-dimensinal evaluation. cardiac catheterization showed multiple aneurysms with no evidence of stenosis. Although he had no clinical symptoms or electrocardiographic evidence of ischemia, pharmacologic stress and delayed Tl-201 SPECT images revealed prominent stress-induced myocardial ischemia in the left ventricle. A resting gated blood pool study showed hypokinesia in corresponding regions of the left ventricle. This case demonstrates the usefulness of Tl-201 myocardial SPECT imaging and resting gated blood pool studies in the management of coronary artery disease in children with Kawasaki disease.
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7/14. Transcatheter polytetrafluoroethylene-covered stent implantation in a giant coronary artery aneurysm of a child with Kawasaki disease--a potential novel treatment.

    We report a case of an eight-year-old boy with Kawasaki disease (KD) who had a giant aneurysm with stenotic lesions located in the right coronary artery, and was treated by transcatheter implantation of a polytetrafluoroethylene(PTFE)-covered stent. Follow-up coronary angiography showed good coronary blood flow. To the best of our knowledge, this is the first child with KD who underwent covered-stent implantation in a coronary aneurysm. Although close follow-up is mandatory, because the long-term outcome is unclear, implantation of a covered stent in a giant aneurysm appears to be a promising treatment option.
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8/14. Five-year clinical course of aortic regurgitation without coronary aneurysm following Kawasaki disease.

    Kawasaki disease has recently been considered as a major cause of acquired valvular diseases in infants and children. But there have been few reports on the clinical course of the lesions caused by this disease, particularly aortic regurgitation. In February 1982, we examined a six-month-old male infant who had Sellers third degree aortic regurgitation (AR) and Sellers first degree mitral regurgitation (MR) without discernible dilatation, aneurysm formation or stenosis of the coronary arteries, and in January 1987 we performed a second cardiac catheterization in order to estimate the progress of these lesions. The results were: 1) His AR showed no apparent worsening; 2) His MR had completely recovered; 3) His volume and pressure studies were almost normal. The catheterization data were compatible with those of other clinical examinations. He no longer had cardiac enlargement as seen on a chest roentgenogram, and there were no signs of left ventricular hypertrophy on an electrocardiogram (ECG).
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9/14. Simultaneous surgical treatment of tetralogy of fallot and coronary artery aneurysm due to mucocutaneous lymph node syndrome in a 4-year-old child.

    The simultaneous correction of tetralogy of fallot and an aneurysm of the coronary artery due to mucocutaneous lymph node syndrome (MCLS) in a 4-year-old boy was successfully performed. Of the four aneurysms found on the coronary angiogram at the age of six months, the smaller three regressed spontaneously leaving slight dilatation and thickening of the arterial wall during the 4-year follow-up period. However, the largest one in the right coronary artery, which remained almost the same size, showed marked stagnation of blood in the aneurysm with the possibility of thrombosis and resultant myocardial infarction. At operation, there was a large aneurysm in the right coronary artery, 9 mm in width and 18 mm in length. The wall of the aneurysm was very thick and tightly adherent to the myocardium. The aneurysm was resected and an aorto-coronary bypass graft was fashioned from a saphenous vein taken from the patient's mother, since an autogenous vein was not available. A ventricular septal defect was closed and pulmonary stenosis was relieved without any difficulty. Postoperative cardiac catheterization and coronary angiography four weeks after the operation revealed satisfactory correction of the tetralogy of fallot and a patent aorto-coronary vein graft. Several problems regarding surgical treatment of coronary artery aneurysm due to MCLS are discussed.
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10/14. Coronary arterial lesions of Kawasaki disease: cardiac catheterization findings of 1100 cases.

    In our institute, 1100 patients with a history of Kawasaki disease have been catheterized for selective coronary arteriography. Their age at examination ranged from four months to 13 years. Coronary artery lesions (CAL) were found in 262 patients. As far as the type of the CAL was concerned, occlusion was noted in 20 (7.6%), segmental stenosis in 15 (5.7%), localized stenosis in 62 (23.7%), aneurysm in 93 (35.5%), and dilatation in 72 patients (27.5%). In terms of the total number of lesions, there were 23 occlusions, 19 segmental stenoses, 109 localized stenoses, 449 aneurysms and 307 dilatations. The 262 patients with CAL were analyzed according to the interval from the onset to the time of selective coronary arteriography. The incidence of both occlusion and segmental stenosis was lowest in the group who were catheterized shortly after the onset of disease, whereas the prevalence of aneurysm was highest in this group. But the prevalence of dilatation was highest in the group of patients who were catheterized late. A total of 12 patients had to undergo femoral arterial thrombectomy for arterial thrombosis following the catheterization, but no other major complication was experienced.
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