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The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.
Summary Myocardial revascularization is now an accepted therapeutic modality for severe coronary arterial obstructive disease produced by Kawasaki disease which is amenable to usual medical treatment. The mortality rate of myocardial infarction is surprisingly high in this setting, and surgery may be able to prevent many of these deaths. This article focuses on current issues in surgical treatment of ischemic heart disease in children secondary to Kawasaki disease. Coronary arterial obstructive disease is, apparently, a leading problem followed by valvar and myocardial involvements. Introduction of bypass grafting using the internal thoracic artery in children was a major recent advance, because this graft has an excellent capability to adapt to the increments of increased flow and growth of the patient. The saphenous vein is less satisfactory when used as a graft in terms of its long-term patency, particularly in young children less than 8 years old. Although longer follow-up is certainly required, the current results of surgery have been excellent in improving myocardial perfusion, in ameliorating symptoms, and improving the quality of life.
Objectives: We evaluated the efficacy of percutaneous transluminal coronary angioplasty for anastomotic stenosis after coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease. Subjects and Methods: From July 1997 to April 2000, four boys and one girl underwent percutaneous transluminal coronary angioplasty for 6 anastomotic lesions following coronary arterial bypass grafting using the left or right internal thoracic artery. Progressive severe stenosis of the grafts in the follow-up angiograms after grafting, and evidence of ischemia, were regarded as indications for percutaneous transluminal coronary angioplasty. Age at coronary angioplasty ranged from 4.2 to 16.7 years, with a median of 6.9 years, while the interval from operation ranged from 0.3 to 3.0 years, with a median of 1.1 years. The diameter of the balloon catheter employed varied from 1.5 to 2.5 mm, and the pressure of inflation ranged from 8 to 16 atmospheres. Results: The degree of stenosis decreased from 63 to 99%, with a median of 88%, to 0 to 40%, with a median of 17% immediately after angioplasty. A follow-up angiogram either 3 months or 1 year later revealed no restenosis in any patient. Conclusion: Percutaneous transluminal coronary angioplasty is a feasible and useful procedure for treating anastomotic stenosis following coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease.
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