Patient Reports A rare cause of cardiac syncope mimicking epilepsy: Left main coronary artery stenosis Yakup Ergul, Kemal Nisli and Umrah Aydogan Department of Pediatric Cardiology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey Key words children, epilepsy, left main coronary artery stenosis, syncope. Although isolated congenital coronary anomalies are seen rarely in children (1% of all child patients undergo coronary angiogra- phy), this disorder has great importance because of its potential effects. 1 Isolated stenosis or atresia of the left coronary artery ostium is extremely rare. 2 Clinically, this can present in late childhood with myocardial infarction, ventricular tachycardia, chest pain, syncope or sudden death, while growth retardation and cardiac failure can be seen at infancy. 3 We report a patient diagnosed with isolated stenosis of the left main coronary artery (LMCA) ostium that presented with recurrent effort-related syncope and generalized tonic–clonic convulsions and was treated with antiepileptic agents as she was thought to suffer from epilepsy. No previous pediatric case has been reported where a misdiagnosis of epilepsy was made in a patient with coronary anomaly. This case emphasizes the role of accounting for coro- nary artery anomalies in the differential diagnosis of children who have a history of effort-related syncope and seizure. Case report An 8-year-old girl presented with recurrent syncope and seizures. Her family reported that initially the generalized tonic–clonic convulsions lasted 10 min and started while playing around in the morning four years earlier and was later accompanied by urinary incontinence. It was noted that oral secretions and cyanosis occurred during the seizure and vomiting occurred after waking. Valproic acid was initiated, considering the diagnosis as epilepsy. Due to the recurrent nature of the seizures, several studies includ- ing electroencephalogram (EEG), biochemistry, and cranial mag- netic resonance imaging were ordered, and all remained negative. Because the patient’s complaints continued one more EEG was performed and although no pathological results were seen clon- azepam was added to the valproic acid that had been taken for one year. While taking history about her latest seizure, the family reported that all the seizures were related to effort. The patient was then referred to our center. No metabolic, cardiac or neuro- logical disorders were noted in the family history. No other abnormality was detected during physical examinations includ- ing detailed cardiovascular and neurological examinations at which neuromotor and growth percentiles were normal. The complete blood count, serum biochemistry, serum lipid profiles and cardiac enzymes were normal in laboratory findings. No cardiomegaly was detected on chest X-ray. The 12-lead elec- trocardiogram (Fig. 1) and 24-hour Holter electrocardiogram were normal. Cranial magnetic resonance imaging and sleep/ awake EEG were applied and no pathology was detected. Two dimensional, M mode, colored and tissue Doppler echocardio- graphy results were normal. Because the complaints were related to effort an exercise test was applied. On the effort electrocardio- gram, T-wave negativity was detected on DII and DIII derivations. Cardiac computed tomography (CT) and cardiac magnetic reso- nance imaging were performed after detecting ischemic signs at the left ventricle anterior and lateral walls on stress myocardial perfusion scintigraphy done with dipyridamole (Fig. 2). On the coronary CT angiography severe stenosis was detected inside the lumen along, approximately a 3 mm segment at ostium level at the proximal part of the LMCA (Fig. 3). Thinning and dyskinesia were detected at the left ventricle distal wall with cardiac magnetic resonance imaging. Cardiac catheterization and coronary angiog- raphy were obtained. The proximal part of the LMCA could not be visualized and it was evident that the circulation in the left coro- nary branches were carried out by the right coronary artery retro- gradely by means of the collaterals (Fig. 4). The antiepileptic treatment was tapered and discontinued and the patient was referred for coronary vascular surgery. The patient underwent coronary ostial plasty. After placing the patient on cardiopulmo- nary bypass and aortic cross clamping, the main pulmonary trunk was retracted laterally. The LMCA was approached anteriorly through a curved aortotomy. Reconstruction was performed using a fresh pericardial patch. No syncope occurred in subsequent follow-up coronary surgery and after six months myocardial per- fusion scan revealed no significant ischemia. Discussion Epilepsy is one of the frequently seen paroxysmal disorders of childhood; the incidence is 0.4–0.7% in developed countries and 1–1.9% in developing ones. 4 It is known that the sudden death Correspondence: Yakup Ergul, MD, Basaksehir Konutlari 5. Etap 1. kisim, D 9 blok, Daire 15 Basaksehir, Istanbul, Turkey. Email: yakupergul77@hotmail.com Received 13 January 2010; revised 14 July 2010; accepted 3 August 2010. doi: 10.1111/j.1442-200X.2010.03254.x Pediatrics International (2011) 53, 581–605 © 2011 The Authors Pediatrics International © 2011 Japan Pediatric Society