D. Roe, Mitochondrial fatty acid oxidation disorders The Metabolic and Molecular Bases of Inherited Disease, pp.2297-326, 2001.

F. Feillet, G. Steinmann, C. Vianey-saban, C. De-chillou, N. Sadoul et al., Adult presentation of MCAD deficiency revealed by coma and severe arrythmias., Intensive Care Medicine, vol.29, issue.9, pp.1594-1601, 2003.
DOI : 10.1007/s00134-003-1871-3

A. Esc, guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death), J Am Coll Cardiol, vol.48, issue.5, pp.247-346, 2006.

C. Murray and A. Lopez, Alternative projections of mortality and disability by cause 1990???2020: Global Burden of Disease Study, The Lancet, vol.349, issue.9064, pp.1498-504, 1997.
DOI : 10.1016/S0140-6736(96)07492-2

C. Murray and A. Lopez, Global mortality, disability, and the contribution of risk factors: Global Burden of Disease Study, The Lancet, vol.349, issue.9063, pp.1436-1478, 1997.
DOI : 10.1016/S0140-6736(96)07495-8

C. Murray and A. Lopez, Mortality by cause for eight regions of the world: Global Burden of Disease Study, The Lancet, vol.349, issue.9061, pp.1269-76, 1997.
DOI : 10.1016/S0140-6736(96)07493-4

K. Okrainec, D. Banerjee, and M. Eisenberg, Coronary artery disease in the developing world, American Heart Journal, vol.148, issue.1, pp.7-15, 2004.
DOI : 10.1016/j.ahj.2003.11.027

S. Chugh, J. Jui, K. Gunson, E. Stecker, B. John et al., Current burden of sudden cardiac death: Multiple source surveillance versus retrospective death certificate-based review in a large U.S. community, Journal of the American College of Cardiology, vol.44, issue.6, 2004.
DOI : 10.1016/j.jacc.2004.06.029

H. Huikuri, A. Castellanos, R. Myerburg, S. Chugh, K. Kelly et al., Sudden death due to cardiac arrhythmias Sudden cardiac death with apparently normal heart, N Engl J Med Circulation, vol.345102, issue.116, pp.1473-82649, 2000.

D. Arking, S. Chugh, A. Chakravarti, and P. Spooner, Genomics in Sudden Cardiac Death, Circulation Research, vol.94, issue.6, pp.712-735, 2004.
DOI : 10.1161/01.RES.0000123861.16082.95

R. Myerburg, Scientific Gaps in the Prediction and Prevention of Sudden Cardiac Death, Journal of Cardiovascular Electrophysiology, vol.13, issue.7, pp.709-732, 2002.
DOI : 10.1046/j.1540-8167.2002.00709.x

Z. Zheng, J. Croft, W. Giles, and G. Mensah, Sudden Cardiac Death in the United States, 1989 to 1998, Circulation, vol.104, issue.18, pp.2158-63, 1989.
DOI : 10.1161/hc4301.098254

M. Daemen and L. Houben, Out-of-hospital cardiac arrest in the 1990's: a population-based study in the Maastricht area on incidence, characteristics and survival, J Am Coll Cardiol, vol.30, issue.6, pp.1500-1505, 1997.

A. Wisten, H. Forsberg, P. Krantz, T. Messner, S. Chugh et al., Sudden cardiac death in 15-35- year olds in Sweden during 1992-99 Cardiac pathologic findings reveal a high rate of sudden cardiac death of undetermined etiology in younger women Causes of sudden death in young female military recruits, Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome, pp.1457-9529, 2002.

G. Lombardi, J. Gallagher, and P. Gennis, Outcome of out-of-hospital cardiac arrest in New York City. The Pre-Hospital Arrest Survival Evaluation (PHASE) Study. Jama, pp.678-83, 1994.

B. Winkel, A. Holst, J. Theilade, I. Kristensen, J. Thomsen et al., Nationwide study of sudden cardiac death in persons aged 1???35 years, European Heart Journal, vol.43, issue.8
DOI : 10.1016/j.jacc.2003.11.052

B. Morentin, M. Suarez-mier, and B. Aguilera, Sudden unexplained death among persons 1???35 years old, Forensic Science International, vol.135, issue.3, pp.213-220, 2003.
DOI : 10.1016/S0379-0738(03)00212-3

D. Corrado, C. Basso, and G. Thiene, Sudden cardiac death in young people with apparently normal heart, Cardiovascular Research, vol.50, issue.2, pp.399-408, 2001.
DOI : 10.1016/S0008-6363(01)00254-1

S. Nattel and L. Carlsson, Innovative approaches to anti-arrhythmic drug therapy, Nature Reviews Drug Discovery, vol.112, issue.Suppl. 4
DOI : 10.1046/j.1540-8167.2005.40621.x

N. Rev-drug-discov-krahn, A. Healey, J. Chauhan, V. Birnie, D. Simpson et al., Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER). Circulation, pp.1034-49278, 2006.

C. Allegue, R. Gil, P. Sanchez-diz, M. Torres, I. Quintela et al., A new approach to long QT syndrome mutation detection by Sequenom MassARRAY?? system, ELECTROPHORESIS, vol.2, issue.10, pp.311648-55
DOI : 10.1002/elps.201000022

M. Brion, I. Quintela, B. Sobrino, M. Torres, C. Allegue et al., New technologies in the genetic approach to sudden cardiac death in the young, Forensic Science International, vol.203, issue.1-3, pp.1-315
DOI : 10.1016/j.forsciint.2010.07.015

M. Ackerman, D. Tester, and D. Driscoll, Molecular Autopsy of Sudden Unexplained Death in the Young, The American Journal of Forensic Medicine and Pathology, vol.22, issue.2, pp.105-116, 2001.
DOI : 10.1097/00000433-200106000-00001

M. Ackerman, State of Postmortem Genetic Testing Known as the Cardiac Channel Molecular Autopsy in the Forensic Evaluation of Unexplained Sudden Cardiac Death in the Young, Pacing and Clinical Electrophysiology, vol.129, issue.2, pp.86-95, 2009.
DOI : 10.1016/j.jacc.2006.10.010

F. Marcus and S. Chugh, Unexplained sudden cardiac death: an opportunity to identify hereditary cardiac arrhythmias, European Heart Journal, vol.7, issue.8
DOI : 10.1016/j.hrthm.2010.05.026

URL : https://academic.oup.com/eurheartj/article-pdf/32/8/931/7111953/ehq505.pdf

S. Chugh, Genomics revolution: Forging a path to the arrhythmia patient, Heart Rhythm, vol.7, issue.8
DOI : 10.1016/j.hrthm.2010.05.026

K. Hong, J. Brugada, A. Oliva, A. Berruezo-sanchez, D. Potenza et al., Value of Electrocardiographic Parameters and Ajmaline Test in the Diagnosis of Brugada Syndrome Caused by SCN5A Mutations, Circulation, vol.110, issue.19, pp.3023-3030, 2004.
DOI : 10.1161/01.CIR.0000144299.17008.07

S. Priori, C. Napolitano, M. Gasparini, C. Pappone, D. Bella et al., Natural history of Brugada syndrome: insights for risk stratification and management. Circulation, pp.1342-1349, 2002.

W. Stremmel, Uptake of fatty acids by jejunal mucosal cells is mediated by a fatty acid binding membrane protein., Journal of Clinical Investigation, vol.82, issue.6, pp.2001-2011, 1988.
DOI : 10.1172/JCI113820

J. Luiken, A. Bonen, and J. Glatz, Cellular fatty acid uptake is acutely regulated by membrane-associated fatty acid-binding proteins. Prostaglandins Leukot Essent Fatty Acids, pp.2-373, 2002.
DOI : 10.1054/plef.2002.0401

S. Neubauer, P. De-lonlay, F. Djouadi, J. Bonnefont, J. Saudubray et al., The Failing Heart ??? An Engine Out of Fuel, New England Journal of Medicine, vol.356, issue.11, pp.1140-51, 2007.
DOI : 10.1056/NEJMra063052

J. Mcgarry and N. Brown, The Mitochondrial Carnitine Palmitoyltransferase System - From Concept to Molecular Analysis, European Journal of Biochemistry, vol.281, issue.1, pp.1-14, 1997.
DOI : 10.1042/bj2810561

D. Wallace, Mitochondrial Diseases in Man and Mouse, Science, vol.283, issue.5407, pp.1482-1490, 1999.
DOI : 10.1126/science.283.5407.1482

M. Brivet, A. Boutron, A. Slama, C. Costa, L. Thuillier et al., Defects in activation and transport of fatty acids, Journal of Inherited Metabolic Disease, vol.22, issue.4, pp.428-469, 1999.
DOI : 10.1023/A:1005552106301

G. Wilhelm, Sudden death in a young woman from medium chain acyl-coenzyme A dehydrogenase (MCAD) deficiency, The Journal of Emergency Medicine, vol.30, issue.3, pp.291-295, 2006.
DOI : 10.1016/j.jemermed.2005.05.030

N. Gregersen, B. Andresen, M. Corydon, T. Corydon, R. Olsen et al., Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship, Human Mutation, vol.31, issue.3, pp.169-89, 2001.
DOI : 10.1212/WNL.52.2.366

N. Blau, G. , J. L. Saudubray, J. Martin, D. De-lonlay et al., Physician's guide to the treatment and follow-up of metabolic diseases: Springer; 2006. 53 Recognition and management of fatty acid oxidation defects: a series of 107 patients, J Inherit Metab Dis, vol.22, issue.4, pp.488-502, 1999.
DOI : 10.1007/3-540-28962-3

B. Wilcken, M. Haas, P. Joy, V. Wiley, M. Chaplin et al., Outcome of neonatal screening for medium-chain acyl-CoA dehydrogenase deficiency in

. Australia, a cohort study. Lancet, pp.37-42, 2007.

A. Hutchesson, S. Bundey, M. Preece, S. Hall, and A. Green, A comparison of disease and gene frequencies of inborn errors of metabolism among different ethnic groups in the West Midlands, UK., Journal of Medical Genetics, vol.35, issue.5, pp.366-70, 1998.
DOI : 10.1136/jmg.35.5.366

M. Nagao, Frequency of 985A-to-G mutation in medium-chain acyl-CoA dehydrogenase gene among patients with sudden infant death syndrome, Reye syndrome, severe motor and intellectual disabilities and healthy newborns in Japan, Pediatrics International, vol.10, issue.4
DOI : 10.1016/S0387-7604(88)80084-6

N. Manning, S. Olpin, R. Pollitt, and J. Webley, A comparison of10-3H]palmitic and10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts, J Inherit Metab Dis, vol.9913, issue.1, pp.58-68, 1990.

M. Pourfarzam, A. Morris, M. Appleton, A. Craft, and K. Bartlett, Neonatal screening for medium-chain acyl-CoA dehydrogenase deficiency. Lancet, pp.1063-1067, 2001.
DOI : 10.1016/s0140-6736(01)06199-2

H. Seddon, G. Gray, R. Pollitt, A. Iitia, and A. Green, Population screening for the common G985 mutation causing medium-chain acyl-CoA dehydrogenase deficiency with Eu-labeled oligonucleotides and the DELFIA system, Clin Chem, 1997.

S. Giroux, A. Dube-linteau, G. Cardinal, Y. Labelle, N. Laflamme et al., Assessment of the prevalence of the 985A>G MCAD mutation in the French-Canadian population using allele-specific PCR, Clinical Genetics, vol.338, issue.8766, pp.569-75, 2007.
DOI : 10.3310/hta8120

S. Opdal and T. Rognum, The Sudden Infant Death Syndrome Gene: Does It Exist?, PEDIATRICS, vol.114, issue.4, pp.506-518, 2004.
DOI : 10.1542/peds.2004-0683

Z. Yang, P. Lantz, and J. Ibdah, Post-mortem analysis for two prevalent betaoxidation mutations in sudden infant death. Pediatr Int, pp.883-890, 2007.

I. Yokota, P. Coates, D. Hale, R. P. Tanaka, and K. , The molecular basis of medium chain acyl-CoA dehydrogenase deficiency: survey and evolution of

A. Pandor, A. Eastham, J. Beverley, C. Chilcott, J. Paisley et al., Methodology guide: conducting an appraisal of a proposed screening programme Clinical effectiveness and cost-effectiveness of neonatal screening for inborn errors of metabolism using tandem mass spectrometry: a systematic review, Health Technol Assess, vol.69, 2004.

C. Wilson, M. Champion, J. Collins, P. Clayton, and J. Leonard, Outcome of medium chain acyl-CoA dehydrogenase deficiency after diagnosis Arch Dis Child 71. HAS. Evaluation de l'extension du dépistage néonatal à une ou plusieurs erreurs innées du métabolisme par spectrométrie de masse en tandem. 1er volet: déficit en MCAD. 2011 [cited; Available from: http://www.hassante.fr/portail/jcms/c_1069254/evaluation-de-lextension-du-depistage-neonatal-a- une-ou-plusieurs-erreurs-innees-du-metabolisme-par-spectrometrie-de-masse-en- tandem-1er-volet-deficit-en-mcad 72, pp.459-62, 1999.

D. Martin, P. De, L. Villain, E. Jouvet, P. Rabier et al., Muscle Nerve, pp.153-62, 2006.
URL : https://hal.archives-ouvertes.fr/hal-01159569

S. Datorre, M. Creer, S. Pogwizd, and P. Corr, Amphipathic lipid metabolites and their relation to arrhythmogenesis in the ischemic heart*1, Journal of Molecular and Cellular Cardiology, vol.23, 1991.
DOI : 10.1016/0022-2828(91)90019-I

M. Arita, T. Sato, H. Ishida, and H. Nakazawa, Cellular electrophysiological basis of proarrhythmic and antiarrhythmic effects of ischemia-related lipid metabolites

J. Huang, H. Xian, and M. Bacaner, Long-chain fatty acids activate calcium channels in ventricular myocytes., Proceedings of the National Academy of Sciences, vol.89, issue.14, pp.6452-6458, 1992.
DOI : 10.1073/pnas.89.14.6452

G. Schmilinsky-fluri, V. Valiunas, M. Willi, and R. Weingart, Modulation of Cardiac Gap Junctions: The Mode of Action of Arachidonic Acid, Journal of Molecular and Cellular Cardiology, vol.29, issue.6, 1997.
DOI : 10.1006/jmcc.1997.0409

R. Sharma, A. Perszyk, D. Marangi, C. Monteiro, and S. Raja, Lethal Neonatal Carnitine Palmitoyltransferase II Deficiency: An Unusual Presentation of a Rare Disorder, American Journal of Perinatology, vol.20, issue.1, pp.25-32, 2003.
DOI : 10.1055/s-2003-37952

M. Deschauer, T. Wieser, and S. Zierz, Muscle Carnitine Palmitoyltransferase II Deficiency, Archives of Neurology, vol.62, issue.1, pp.37-41, 2005.
DOI : 10.1001/archneur.62.1.37

URL : http://archneur.jamanetwork.com/data/journals/neur/12041/nnr40006.pdf

D. Millington, N. Kodo, D. Norwood, and C. Roe, Tandem mass spectrometry: A new method for acylcarnitine profiling with potential for neonatal screening for inborn errors of metabolism, Journal of Inherited Metabolic Disease, vol.28, issue.3, pp.321-325, 1990.
DOI : 10.1007/BF01799385

C. Roe and D. Roe, Recent Developments in the Investigation of Inherited Metabolic Disorders Using Cultured Human Cells, Molecular Genetics and Metabolism, vol.68, issue.2, 1999.
DOI : 10.1006/mgme.1999.2911

M. Bennett, Analysis of carnitine esters by radio-high performance liquid chromatography in cultured skin fibroblasts from patients with mitochondrial fatty acid oxidation disorders, Pediatr Res, vol.44, issue.2, pp.210-214, 1998.

P. Corr, R. Gross, and B. Sobel, Arrhythmogenic amphiphilic lipids and the myocardial cell membrane, Journal of Molecular and Cellular Cardiology, vol.14, issue.11, pp.619-645, 1982.
DOI : 10.1016/0022-2828(82)90159-6

M. Oliver, Sudden cardiac death: the lost fatty acid hypothesis, QJM, vol.99, issue.10, 2006.
DOI : 10.1093/qjmed/hcl084

M. Bodman, D. Smith, W. Nyhan, and R. Naviaux, Medium-Chain Acyl Coenzyme A Dehydrogenase Deficiency, Archives of Neurology, vol.58, issue.5, 2001.
DOI : 10.1001/archneur.58.5.811

URL : http://archneur.jamanetwork.com/data/journals/neur/6665/nob00048.pdf

S. Korman, A. Gutman, R. Brooks, T. Sinnathamby, N. Gregersen et al., Homozygosity for a severe novel medium-chain acyl-CoA dehydrogenase (MCAD) mutation IVS3-1G>C that leads to introduction of a premature termination codon by complete missplicing of the MCAD mRNA and is associated with phenotypic diversity ranging from sudden neonatal death to asymptomatic status, Molecular Genetics and Metabolism, vol.82, issue.2
DOI : 10.1016/j.ymgme.2004.03.002

S. Sanatani, N. Mahkseed, H. Vallance, and R. Brugada, The Brugada ECG Pattern in a Neonate, Journal of Cardiovascular Electrophysiology, vol.67, issue.4, pp.342-346, 2005.
DOI : 10.1161/01.RES.67.4.1040

S. Hendrickson, S. Louis, J. Lowe, and J. , Abdel-aleem S. Free fatty acid metabolism during myocardial ischemia and reperfusion, Mol Cell Biochem, 1997.

L. Opie, Metabolism of free fatty acids, glucose and catecholamines in acute myocardial infarction, The American Journal of Cardiology, vol.36, issue.7, pp.938-53, 1975.
DOI : 10.1016/0002-9149(75)90086-7

J. Neely and H. Morgan, Relationship Between Carbohydrate and Lipid Metabolism and the Energy Balance of Heart Muscle, Annual Review of Physiology, vol.36, issue.1, pp.413-59, 1974.
DOI : 10.1146/annurev.ph.36.030174.002213

R. Ferrari, P. Pepi, F. Ferrari, F. Nesta, M. Benigno et al., Metabolic derangement in ischemic heart disease and its therapeutic control, The American Journal of Cardiology, vol.82, issue.5
DOI : 10.1016/S0002-9149(98)00531-1

A. Gambardella, Association of fasting plasma free fatty acid concentration and frequency of ventricular premature complexes in nonischemic non-insulin-dependent diabetic patients, Am J Cardiol, vol.80, issue.7, pp.932-939, 1997.

X. Jouven, M. Charles, M. Desnos, and P. Ducimetiere, Circulating Nonesterified Fatty Acid Level as a Predictive Risk Factor for Sudden Death in the Population, Circulation, vol.104, issue.7
DOI : 10.1161/hc3201.094151

M. Murnaghan, EFFECT OF FATTY ACIDS ON THE VENTRICULAR ARRHYTHMIA THRESHOLD IN THE ISOLATED HEART OF THE RABBIT, British Journal of Pharmacology, vol.5, issue.4, pp.909-924, 1981.
DOI : 10.1016/0022-2828(73)90066-7

A. Katz, P. Nash-adler, J. Watras, F. Messineo, H. Takenaka et al., Fatty acid effects on calcium influx and efflux in sarcoplasmic reticulum vesicles from rabbit skeletal muscle, Biochimica et Biophysica Acta (BBA) - Biomembranes, vol.687, issue.1, pp.17-26, 1982.
DOI : 10.1016/0005-2736(82)90165-1

M. Makiguchi, H. Kawaguchi, M. Tamura, and H. Yasuda, Effect of palmitic acid and fatty acid binding protein on ventricular fibrillation threshold in the perfused rat heart, Cardiovascular Drugs and Therapy, vol.23, issue.4
DOI : 10.1007/978-1-4684-7433-6_35

A. Katz, Trans-fatty acids and sudden cardiac death. Circulation, Feb, vol.12105, issue.6, pp.669-71, 2002.

R. Lemaitre, I. King, T. Raghunathan, R. Pearce, S. Weinmann et al., Cell Membrane Trans-Fatty Acids and the Risk of Primary Cardiac Arrest, Circulation, vol.105, issue.6
DOI : 10.1161/hc0602.103583

V. Kurien and M. Oliver, A METABOLIC CAUSE FOR ARRHYTHMIAS DURING ACUTE MYOCARDIAL HYPOXIA, The Lancet, vol.295, issue.7651, pp.813-818, 1970.
DOI : 10.1016/S0140-6736(70)92412-8

R. Kelly, O. Hara, D. Mitch, W. Smith, and T. , Identification of NaK-ATPase inhibitors in human plasma as nonesterified fatty acids and lysophospholipids, J Biol Chem, vol.261, issue.25, pp.11704-11715, 1986.

D. Kim and R. Duff, Regulation of K+ channels in cardiac myocytes by free fatty acids, Circulation Research, vol.67, issue.4, pp.1040-1046, 1990.
DOI : 10.1161/01.RES.67.4.1040

A. Rettinger, K. Gempel, S. Hofmann, K. Gerbitz, and M. Bauer, Tandem Mass Spectrometric Assay for the Determination of Carnitine Palmitoyltransferase II Activity in Muscle Tissue, Analytical Biochemistry, vol.302, issue.2, pp.246-51, 2002.
DOI : 10.1006/abio.2001.5554

F. Akar, O. Rourke, and B. , Mitochondria are sources of metabolic sink and arrhythmias, Pharmacology & Therapeutics, vol.131, issue.3, pp.287-94
DOI : 10.1016/j.pharmthera.2011.04.005