Congenital atresia of left main coronary artery followed up for ages as a sequela of Kawasaki disease

Abstract

Key words congenital atresia, coronary calcification, Kawasaki disease, left main coronary artery.Congenital atresia of the left main coronary artery (LMCA) is a rare cardiac anomaly. The disease can cause sudden death as a consequence of myocardial ischemia, so surgical treatment is required. Kawasaki disease (KD) is an acute febrile pediatric vasculitis of unknown etiology. The serious complications of KD involve coronary arterial abnormalities, including coronary aneurysm, stenosis or total occlusion. Case reportA 16-year-old boy, who had been followed up as having occlusion of the LMCA due to KD, was admitted to our hospital for cardiac catheterization to evaluate his present coronary arterial status. He had suffered from KD at the age of 9 months old and was treated only by oral aspirin without intravenous high-dose gamma globulin. Echocardiography at that time disclosed a giant aneurysm (8 mm) in the LMCA and a small one (4 mm) in the right coronary artery (RCA). At age 2 years 8 months, he suffered from myocardial infarction. After that cardiac event, he received coronary angiogram in another hospital and was found to have occlusion of the LMCA and collateral vessels from the RCA to the left anterior descending artery (LAD) and to the left circumflex artery (LCX).He has been doing well and has had no cardiac complaints except for the myocardial infarction at age 2 years 8 months. He showed normal physical findings and clear heart sounds with regular rhythm. Blood pressure was 114/74. Electrocardiography exhibited an entirely normal pattern, and chest X-ray film showed neither cardiomegaly nor visible calcification. Echocardiography revealed that the LMCA seemed to originate from the left aortic Valsalva's sinus as shown in Figure 1. Myocardial scintigraphy stressed with adenosine triphosphate disclosed the presence of perfusion defect in the mid-anterior area, which was incompletely redistributed on the rest of the scan. Although we performed a 16-slice multidetector computed tomography (CT) scan, we could not assess the LMCA because of a blurred 3-D reconstruction image.In cardiac catheterization as shown in Figure 2, the LMCA was not identified at the left aortic Valsalva's sinus or any other sites in the aorta by ascending aortic root angiogram. The rudimentary orifice of the LMCA, which is expected to exist in cases with the occluded LMCA after KD, was not detected on repeated aortography. Moreover, there was no calcification in the putative LMCA region. The LAD and the LCX were retrogradely supplied via collateral arteries from the RCA. The diameters of LAD and LCX were, respectively, 1.4 mm and 2.1 mm, both of which were apparently small for his age, indicating hypoplasia of them. On the other hand, the diameter of the RCA was 5.1 mm, which was relatively large, suggesting compensatory dilation of RCA. No perfusion to the pulmonary artery was identified either after

Characteristics and long-term outcome for congenital left main coronary artery atresia

“…The orifice of the left coronary artery seems to be normal at its position on the two-dimensional echocardiogram; however, the left coronary artery is slightly small. 8 The diameter of the right coronary artery is not always dilated on the two-dimensional echocardiogram at the initial presentation. Therefore, it is likely that a diagnosis of atresia of the left coronary artery will be missed.…”

Occlusion of left main coronary artery presenting with ventricular fibrillation in teenagers: an unrecognised cause of sudden death?

“…4,7 A review of literature reveals numerous other case studies with delayed diagnosis of left main coronary artery atresia. [3][4][5][6] Amaral et al described two paediatric cases confirmed via coronary angiography or direct inspection in the operating room, despite normal coronaries on echocardiogram. Shah et al described a paediatric case with recurrent exertional syncopal episodes, an echocardiogram with prominent right coronary artery (otherwise normal), normal exercise testing and diagnosis confirmed by cardiac catheterisation.…”

“…3 Imaging modalities such as echocardiography and CT angiography can identify coronary artery anomalies; however, they may also provide false reassurance of normal coronary anatomy. [4][5][6] Early diagnosis and surgical intervention lead to improved outcomes. 4 We report two adolescent patients who presented with syncope and ventricular fibrillation secondary to occlusion of the left main coronary artery.…”

Cardiac surgical procedures for the coronary sequelae of Kawasaki disease

“…Occlusion of the RCA may induce not only inferior infarction of the left ventricle but also right ventricular infarction and fatal heart block, and hence a sole RCA occlusion is still an indication of coronary artery revascularization (82). Remarkably, occlusion of the LM was sometimes a congenital disorder but not a sequela of Kawasaki disease (75). …”