Pediatrics International (2011) 53, 581605

Patient Reports

ped_3254

581..605

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 angiography), 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 tonicclonic 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 coronary 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 tonicclonic 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 including electroencephalogram (EEG), biochemistry, and cranial magnetic resonance imaging were ordered, and all remained negative. Because the patients complaints continued one more EEG was performed and although no pathological results were seen clonazepam 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 neuroCorrespondence: 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

logical disorders were noted in the family history. No other abnormality was detected during physical examinations including detailed cardiovascular and neurological examinations at which neuromotor and growth percentiles were normal. The complete blood count, serum biochemistry, serum lipid proles and cardiac enzymes were normal in laboratory ndings. No cardiomegaly was detected on chest X-ray. The 12-lead electrocardiogram (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 echocardiography results were normal. Because the complaints were related to effort an exercise test was applied. On the effort electrocardiogram, T-wave negativity was detected on DII and DIII derivations. Cardiac computed tomography (CT) and cardiac magnetic resonance 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 angiography were obtained. The proximal part of the LMCA could not be visualized and it was evident that the circulation in the left coronary branches were carried out by the right coronary artery retrogradely 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 cardiopulmonary 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 perfusion scan revealed no signicant ischemia.

Discussion
Epilepsy is one of the frequently seen paroxysmal disorders of childhood; the incidence is 0.40.7% in developed countries and 11.9% in developing ones.4 It is known that the sudden death

2011 The Authors Pediatrics International 2011 Japan Pediatric Society

582

Y Ergul et al.

Fig. 1 A sample of baseline electrocardiogram.

risk is higher in epileptic patients than in the normal population.5 Moreover, in epileptic patients, electrical stimulation of different parts of the brain can cause cardiac arrhythmias and this could be up to 48% more likely during seizures. The most commonly seen arrhythmias are tachyarrhythmias, bradyarrhythmias, asystole, atrioventricular block, and ventricular brillation.6,7 In fact, the physiological response to the seizure is tachycardia, hypertension, increase of sympathetic tonus, cardiac and cerebral blood ow. Particularly in childhood seizures, bradycardia, hypotension and respiratory anomalies can be seen. In epileptic patients, the existence of syncope, pallor and fatigue in the history should be a warning of cardiac disease.4,6 Sometimes EEG and cranial screening tests can be normal in epileptic patients. In that case, treatment can be started if a seizure can be proved.4 Coronary anomalies are rare in childhood. However depending on the locations and types, these could be very important since they can cause myocardial infarction, effort-related
Fig. 3 A severe stenosis is seen just after the left main coronary ostium on the coronary angiography established by computed tomography. LMCA, left main coronary artery.

Fig. 2 A sample of electrocardiogram (ECG) during the test of the stress myocardial perfusion scintigraphy with dipyridamole. The ECG shows marked ST segment depression on D I, II, III, aVF, and V4-6 derivations. 2011 The Authors Pediatrics International 2011 Japan Pediatric Society

syncope, myocardial dysfunction, congestive heart failure, and sudden death. It is very important for pediatricians to know and be reminded of these risks which are higher in childhood and adolescent periods.1 Coronary anomalies in the pediatric period are mostly related to Kawasaki disease, familial hypercholesterolemia, Williams syndrome and congenital heart diseases, such as supravalvular aortic stenosis, ventricular septal defect, and pulmonary stenosis, although they could also be isolated.3 The main clinical sign of the congenital or acquired coronary artery anomalies is the myocardial ischemia, depending on a decrease or impairment of coronary artery perfusion.1 Cerebral ischemia and seizures can be seen in patients depending on the extent of myocardial ischemia or rhythm disorders and can be confused with epileptic seizures. In our case, it became evident while taking the history carefully that all seizures were effortrelated and signs like fatigue and pallor were seen before the

Coronary stenosis mimicking epilepsy

583

graphy is the best method for diagnosis of coronary anomalies; coronary CT angiography can provide images of coronary morphology and cardiac functions non-invasively.8 However, treatment must be surgical with the aim of revascularization of myocardium soon after the diagnosis.3 In conclusion, in cases presenting with syncope and seizures related to effort, cardiac etiologies denitely must be taken into account and myocardial stress tests should be performed even if the physical examination, electrocardiogram, Holter electrocardiogram and echocardiography are normal and coronary artery imaging must be performed, primarily in a non-invasive manner, on unclear cases.

References
1 Frommelt PC, Frommelt MA. Congenital coronary artery anomalies. Pediatr. Clin. North Am. 2004; 51: 127388. 2 Gebauer R, Cerny S, Vojtovic P, Tax P. Congenital atresia of the left coronary artery-myocardial revascularization in two children. Interact. Cardiovasc. Thorac. Surg. 2008; 7: 11745. 3 Musiani A, Cernigliaro C, Sansa M, Maselli D, De Gasperis C. Left main coronary artery atresia: Literature review and therapeutical considerations. Eur. J. Cardiothorac. Surg. 1997; 11: 50514. 4 Goetz CG, Pappert EJ, eds. Textbook of Clinical Neurology, 1st edn. WB Saunders, Philadelphia, PA, 1999. 5 Walczak TS, Leppik IE, Amelio MD et al. Incidence and risk factors in sudden unexpected death in epilepsy: A prospective cohort study. Neurology 2001; 56: 51925. 6 ORegan ME, Brown JK. Abnormalities in cardiac and respiratory function observed during seizures in childhood. Dev. Med. Child. Neurol. 2005; 47: 49. 7 Nei M, Ho RT, Sperling MR. ECG abnormalities during partial seizures in refractory epilepsy. Epilepsia 2000; 41: 5428. 8 Friedman AH, Fogel MA, Stephens P Jr et al. Identication, imaging, functional assessment and management of congenital coronary arterial abnormalities in children. Cardiol. Young 2007; 17: 5667.
ped_3255 583..607

Fig. 4 Right coronary angiogram obtained on the right anterior oblique position. Left coronary system is lled up totally by right coronary artery retrogradely up to the left main coronary ostium.

seizures. Musiani et al.3 showed among 15 pediatric patients suffering from LMCA atresia and stenosis that the main referral signs were myocardial infarction in younger children and syncope and tachyarrhythmias in older children. The angio-

A case of d-bifunctional protein deciency: Clinical, biochemical and molecular investigations

Paolo Ghirri,1 Marco Vuerich,1 Sacha Ferdinandusse,4 Hans R. Waterham,2 Andrea Guzzetta,3 Maria C. Bianchi,2 Antonio Boldrini1 and Ronald J.A. Wanders4 1 Neonatology Unit, 2Department of Neuroradiology, S. Chiara Hospital, AOUP, and 3Department of Neurodevelopmental Neuroscience, Stella Maris Scientic Institute, Pisa, Italy, and 4Lab Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands
Key words d-bifunctional protein, peroxisomal fatty acid oxidation disorders, very long-chain fatty acids. d-bifunctional protein (DBP), also called multifunctional protein 2, is a crucial component of the peroxisomal fatty acid betaoxidation system, and is required for the oxidation of multiple fatty acids and fatty acid derivatives. It is a 79 kDa protein, harboring two different enzymatic activities, a 2-enoyl-coenzyme
2011 The Authors Pediatrics International 2011 Japan Pediatric Society

Correspondence: Paolo Ghirri, PhD, MD, U.O. di Neonatologia, Ospedale S. Chiara, AOUP, via Roma 67, 56126 Pisa, Italy. Email: pghirri@med.unipi.it Received 19 May 2009; revised 16 June 2010; accepted 2 August 2010. doi: 10.1111/j.1442-200X.2010.03255.x

Copyright of Pediatrics International is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.