Left main coronary artery atresia diagnosed by multidetector computed tomography

Saito, Tatsuhito; Motohashi, Masatoshi; Matsushima, Shouji; Saito, Naotaka; Kubota, Takehiro; Tasai, Kimitaka; Asajima, Hiroshi

doi:10.1016/j.ijcard.2008.03.068

Cited by 11 publications

(6 citation statements)

References 9 publications

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“…Patients with this condition have absent left coronary ostium, or absent left main coronary artery trunk which on coronary CT can sometimes be seen as a string-like structure suggesting a rudimentary form of the left main coronary artery 3. The left coronary circulation is supplied by the RCA through collaterals.…”

Section: Discussionmentioning

confidence: 99%

Unusual congenital coronary artery anomaly in a young adult presenting as sudden cardiac arrest

Cortes¹,

Roldan

Clegg

2018

BMJ Case Reports

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A previously asymptomatic young female with no previous medical or cardiac history collapsed during indoor exercise. A portable automatic external defibrillator showed a shockable rhythm. She received multiple electrical shocks with return to normal sinus rhythm without ischaemic ECG changes. Her troponin level was mildly elevated. A transthoracic echocardiogram revealed moderately reduced left ventricular ejection fraction with global hypokinesis. During emergent coronary angiography, the left main coronary artery could not be found. The right coronary artery was normal with robust collaterals to the entire left coronary circulation extending to the left main coronary artery, but did not fill the ostium. Coronary CT angiogram confirmed nearly complete absence of the left main coronary artery ostium. A diagnosis of left main coronary artery atresia was made. Patient underwent successful two vessel coronary artery bypass grafting. She continues to do well 1 year postoperatively.

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Section: Discussionmentioning

confidence: 99%

Unusual congenital coronary artery anomaly in a young adult presenting as sudden cardiac arrest

Cortes¹,

Roldan

Clegg

2018

BMJ Case Reports

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“…In older children and adults, syncope and chest pain are often the initial symptoms, but sometimes SCD could be the first symptom. In adult onset cases, a history of syncope in childhood has been reported [1][2][3]7,8]. The symptoms depend on the formation of feeding collateral vessels from the right coronary artery to the left coronary artery.…”

Section: Discussionmentioning

confidence: 99%

“…Poor development results in heart failure at a young age and sufficient development results in syncope, chest pain, and SCD during exercise, with relatively increasing metabolic demands of the heart. In recent years, MDCT has played an important role in the diagnosis of LMCAA in older children and adults [8]. Surgical treatment is often selected for the prevention of SCD, except for difficult patients of surgery who have strong hypoplasia of the left coronary arteries.…”

Section: Discussionmentioning

confidence: 99%

Congenital left main coronary artery atresia presenting as syncope and generalized seizure during exercise in a 13-year-old boy

Fujita

Sato

Nagata

et al. 2017

Journal of Cardiology Cases

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Congenital left main coronary artery atresia (LMCAA) is an extremely rare coronary artery anomaly in which the proximal left main trunk ends blindly and blood flows from the right coronary artery to the left coronary artery through collateral vessels [1][2][3]. Depending on the development of collateral vessels from the right coronary artery to the left coronary artery and on metabolic demands of the heart, clinical symptoms can vary widely from infants to adults. Here, we report the case of a 13-year-old boy with LMCAA who experienced syncope and seizure during strenuous exercise. Case reportA 13-year-old boy was transferred to our hospital emergency room. He had suddenly fallen while running in a school gymnasium in mid-August and experienced generalized tonicclonic seizure. At school, an AED was attached immediately, but AED analysis indicated no need for shock, and shock was not delivered. However, since his pulse was weak, his teachers started CPR. When ambulance crews arrived, his pulse had returned to normal and he was showing spontaneous breathing, but he continued to experience loss of consciousness. The patient's height was 156 cm and weight was 42 kg. Upon arrival at our hospital, his consciousness level was 300 on the Japan Coma Scale, and electrocardiogram monitoring showed normal sinus rhythm without ST-T wave abnormalities. Oxygen saturation was maintained at 92% on 10 L/min of oxygen. Heart rate was 120 bpm and blood pressure was 116/94 mmHg. He had a decerebrate rigid posture with shivering, with all limbs moving intermittently. His

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“…Despite a well‐developed collateral circulation, these patients usually develop myocardial ischemia, indicating inadequate myocardial perfusion. Although it is quite difficult to diagnose this disease with conventional instruments, a 64‐slice multidetector CT scan can be a useful tool in diagnosing the disease by detecting the “string‐like structure” suggestive of the rudimentary form of the LMCA 3 . As there is high risk of sudden death, CABG surgery is recommended for either symptomatic or asymptomatic patients with congenital atresia of LMCA, however long‐term results of CABG in pediatric patients are still questionable despite the cases of excellent repair 1,2 …”

Section: Discussionmentioning

confidence: 99%

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

Furuyama

Kinugawa

Nakajima

et al. 2010

Pediatrics International

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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

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Left main coronary artery atresia diagnosed by multidetector computed tomography

Cited by 11 publications

References 9 publications

Unusual congenital coronary artery anomaly in a young adult presenting as sudden cardiac arrest

Unusual congenital coronary artery anomaly in a young adult presenting as sudden cardiac arrest

Congenital left main coronary artery atresia presenting as syncope and generalized seizure during exercise in a 13-year-old boy

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

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