{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T18:21:12Z","timestamp":1780510872951,"version":"3.54.1"},"reference-count":107,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Disease-a-Month"],"published-print":{"date-parts":[[2023,4]]},"DOI":"10.1016\/j.disamonth.2023.101548","type":"journal-article","created":{"date-parts":[[2023,3,15]],"date-time":"2023-03-15T10:10:40Z","timestamp":1678875040000},"page":"101548","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":10,"title":["Sudden cardiac death in childhood hypertrophic cardiomyopathy"],"prefix":"10.1016","volume":"69","author":[{"given":"Keval","family":"Thakkar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adnan Rafiq","family":"Karajgi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anusha Manoj","family":"Kallamvalappil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chaithanya","family":"Avanthika","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharan","family":"Jhaveri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arundhati","family":"Shandilya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Anusheel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rayan","family":"Al-masri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"8","key":"10.1016\/j.disamonth.2023.101548_bib0001","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1161\/01.CIR.102.8.858","article-title":"Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population","volume":"102","author":"Maron","year":"2000","journal-title":"Circulation"},{"key":"10.1016\/j.disamonth.2023.101548_bib0002","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2021.708679","article-title":"Childhood hypertrophic cardiomyopathy: a disease of the cardiac sarcomere","volume":"9","author":"Norrish","year":"2021","journal-title":"Front Pediatr"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0003","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1007\/s00424-019-02259-2","article-title":"Three perspectives on the molecular basis of hypercontractility caused by hypertrophic cardiomyopathy mutations","volume":"471","author":"Spudich","year":"2019","journal-title":"Pflugers Arch"},{"key":"10.1016\/j.disamonth.2023.101548_bib0004","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.ijcard.2019.06.073","article-title":"Prevalence and clinical significance of red flags in patients with hypertrophic cardiomyopathy","volume":"299","author":"Limongelli","year":"2020","journal-title":"Int J Cardiol"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0005","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1016\/j.cardfail.2012.01.017","article-title":"Pediatric cardiomyopathy: importance of genetic and metabolic evaluation","volume":"18","author":"Kindel","year":"2012","journal-title":"J Card Fail"},{"issue":"17","key":"10.1016\/j.disamonth.2023.101548_bib0006","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1056\/NEJMoa021715","article-title":"The incidence of pediatric cardiomyopathy in two regions of the United States","volume":"348","author":"Lipshultz","year":"2003","journal-title":"N Engl J Med"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0007","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1016\/j.jacep.2018.11.004","article-title":"Sudden death can be the first manifestation of hypertrophic cardiomyopathy: data from a United Kingdom pathology registry","volume":"5","author":"Finocchiaro","year":"2019","journal-title":"JACC Clin Electrophysiol"},{"issue":"9","key":"10.1016\/j.disamonth.2023.101548_bib0008","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1093\/eurheartj\/ehn122","article-title":"Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy","volume":"29","author":"Ostman-Smith","year":"2008","journal-title":"Eur Heart J"},{"issue":"24","key":"10.1016\/j.disamonth.2023.101548_bib0009","doi-asserted-by":"crossref","first-page":"2761","DOI":"10.1161\/CIR.0b013e318223e230","article-title":"2011 ACCF\/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American college of cardiology foundation\/American heart association task force on practice guidelines","volume":"124","author":"Gersh","year":"2011","journal-title":"Circulation"},{"issue":"7","key":"10.1016\/j.disamonth.2023.101548_bib0010","doi-asserted-by":"crossref","first-page":"2212","DOI":"10.1016\/S0735-1097(00)01003-2","article-title":"Sudden death in hypertrophic cardiomyopathy: identification of high risk patients","volume":"36","author":"Elliott","year":"2000","journal-title":"J Am Coll Cardiol"},{"issue":"39","key":"10.1016\/j.disamonth.2023.101548_bib0011","doi-asserted-by":"crossref","first-page":"2733","DOI":"10.1093\/eurheartj\/ehu284","article-title":"2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)","volume":"35","author":"Elliott","year":"2014","journal-title":"Eur Heart J"},{"issue":"25","key":"10.1016\/j.disamonth.2023.101548_bib0012","first-page":"e533","article-title":"2020 AHA\/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: executive summary: a report of the american college of cardiology\/American heart association joint committee on clinical practice guidelines","volume":"142","author":"Ommen","year":"2020","journal-title":"Circulation"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0013","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1161\/CIRCULATIONAHA.106.621185","article-title":"Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children","volume":"115","author":"Colan","year":"2007","journal-title":"Circulation"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0014","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1136\/hrt.2006.094730","article-title":"Outcomes after implantable cardioverter-defibrillator treatment in children with hypertrophic cardiomyopathy","volume":"93","author":"Kaski","year":"2007","journal-title":"Heart"},{"issue":"1","key":"10.1016\/j.disamonth.2023.101548_bib0015","doi-asserted-by":"crossref","DOI":"10.1161\/CIR.0000000000000682","article-title":"Cardiomyopathy in children: classification and diagnosis: a scientific statement from the American heart association","volume":"140","author":"Lipshultz","year":"2019","journal-title":"Circulation"},{"key":"10.1016\/j.disamonth.2023.101548_bib0016","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1161\/CIRCRESAHA.112.270207","article-title":"Human molecular genetic and functional studies identify TRIM63, encoding Muscle RING Finger Protein 1, as a novel gene for human hypertrophic cardiomyopathy","volume":"111","author":"Chen","year":"2012","journal-title":"Circ Res"},{"key":"10.1016\/j.disamonth.2023.101548_bib0017","first-page":"36","article-title":"The genetics of hypertrophic cardiomyopathy","volume":"2018","author":"Akhtar","year":"2018","journal-title":"Glob Cardiol Sci Pract"},{"key":"10.1016\/j.disamonth.2023.101548_bib0018","doi-asserted-by":"crossref","first-page":"1978","DOI":"10.1016\/j.jacc.2019.01.061","article-title":"Moving beyond the sarcomere to explain heterogeneity in hypertrophic cardiomyopathy: JACC review topic of the week","volume":"73","author":"Maron","year":"2019","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0019","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCGEN.119.002460","article-title":"Evaluating the clinical validity of hypertrophic cardiomyopathy genes","volume":"12","author":"Ingles","year":"2019","journal-title":"Circ Genom Precis Med"},{"key":"10.1016\/j.disamonth.2023.101548_bib0020","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2021.632293","article-title":"hypertrophic cardiomyopathy in children: pathophysiology, diagnosis, and treatment of non-sarcomeric causes","volume":"9","author":"Monda","year":"2021","journal-title":"Front Pediatr"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0021","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1038\/nrcardio.2011.159","article-title":"Mechanisms of disease: hypertrophic cardiomyopathy","volume":"9","author":"Frey","year":"2011","journal-title":"Nat Rev Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0022","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.3389\/fphys.2018.01391","article-title":"Altered Ca2+ and Na+ homeostasis in human hypertrophic cardiomyopathy: implications for arrhythmogenesis","volume":"9","author":"Coppini","year":"2018","journal-title":"Front Physiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0023","doi-asserted-by":"crossref","first-page":"1738","DOI":"10.1161\/CIRCULATIONAHA.115.020086","article-title":"Genotype-dependent and -independent calcium signaling dysregulation in human hypertrophic cardiomyopathy","volume":"134","author":"Helms","year":"2016","journal-title":"Circulation"},{"key":"10.1016\/j.disamonth.2023.101548_bib0024","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1172\/JCI44929","article-title":"MEK-ERK pathway modulation ameliorates disease phenotypes in a mouse model of Noonan syndrome associated with the Raf1(L613V) mutation","volume":"121","author":"Wu","year":"2011","journal-title":"J Clin Investig"},{"key":"10.1016\/j.disamonth.2023.101548_bib0025","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/S1095-6433(03)00007-2","article-title":"Glycolysis and pyruvate oxidation in cardiac hypertrophy\u2013why so unbalanced?","volume":"135","author":"Leong","year":"2003","journal-title":"Comp Biochem Physiol Part A Mol Integr. Physiol."},{"key":"10.1016\/j.disamonth.2023.101548_bib0026","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.120.017025","article-title":"Defining the cardiac fibroblast secretome in a fibrotic microenvironment","volume":"9","author":"Ceccato","year":"2020","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.disamonth.2023.101548_bib0027","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.1161\/CIRCULATIONAHA.118.034609","article-title":"Inhibiting fibronectin attenuates fibrosis and improves cardiac function in a model of heart failure","volume":"138","author":"Valiente-Alandi","year":"2018","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0028","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1161\/01.CIR.86.2.467","article-title":"Ventricular fibrillation in hypertrophic cardiomyopathy is associated with increased fractionation of paced right ventricular electrograms","volume":"86","author":"Saumarez","year":"1992","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0029","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1148\/rg.2016150137","article-title":"Hypertrophic cardiomyopathy from A to Z: genetics, pathophysiology, imaging, and management","volume":"36","author":"Baxi","year":"2016","journal-title":"RadioGraphics"},{"issue":"9","key":"10.1016\/j.disamonth.2023.101548_bib0030","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1001\/jamacardio.2019.2861","article-title":"Development of a novel risk prediction model for sudden cardiac death in childhood hypertrophic cardiomyopathy (HCM Risk-Kids)","volume":"4","author":"Norrish","year":"2019","journal-title":"JAMA Cardiol"},{"issue":"8","key":"10.1016\/j.disamonth.2023.101548_bib0031","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1161\/CIRCULATIONAHA.117.032966","article-title":"Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy","volume":"138","author":"Axelsson Raja","year":"2018","journal-title":"Circulation"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0032","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1161\/CIRCULATIONAHA.120.047235","article-title":"A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy","volume":"142","author":"Miron","year":"2020","journal-title":"Circulation"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0033","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1001\/jamacardio.2018.0789","article-title":"Long-term outcomes of pediatric-onset hypertrophic cardiomyopathy and age-specific risk factors for lethal arrhythmic events","volume":"3","author":"Maurizi","year":"2018","journal-title":"JAMA Cardiol"},{"issue":"12","key":"10.1016\/j.disamonth.2023.101548_bib0034","doi-asserted-by":"crossref","first-page":"1651","DOI":"10.1016\/S0002-9149(97)00216-6","article-title":"Comparison of morphologic assessment of hypertrophic cardiomyopathy by magnetic resonance versus echocardiographic imaging","volume":"79","author":"Pons-Llad\u00f3","year":"1997","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0035","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/j.ijcard.2017.07.068","article-title":"Cardiac defects, morbidity and mortality in patients affected by RASopathies. CARNET study results","volume":"245","author":"Calcagni","year":"2017","journal-title":"Int J Cardiol"},{"issue":"4","key":"10.1016\/j.disamonth.2023.101548_bib0036","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1016\/j.amjcard.2007.03.093","article-title":"Prevalence and clinical significance of cardiovascular abnormalities in patients with the LEOPARD syndrome","volume":"100","author":"Limongelli","year":"2007","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0037","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1186\/s12883-015-0412-3","article-title":"Diagnosis and treatment of late-onset Pompe disease in the Middle East and North Africa region: consensus recommendations from an expert group","volume":"15","author":"Al Jasmi","year":"2015","journal-title":"BMC Neurol"},{"issue":"4","key":"10.1016\/j.disamonth.2023.101548_bib0038","first-page":"271","article-title":"Infantile-onset Pompe disease: diagnosis and management. Enfermedad de Pompe infantil: diagn\u00f3stico y tratamiento","volume":"117","author":"Bay","year":"2019","journal-title":"Arch Argent Pediatr"},{"issue":"12","key":"10.1016\/j.disamonth.2023.101548_bib0039","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1001\/jama.2009.371","article-title":"Clinical outcome and phenotypic expression in LAMP2 cardiomyopathy","volume":"301","author":"Maron","year":"2009","journal-title":"JAMA"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0040","doi-asserted-by":"crossref","first-page":"3507","DOI":"10.3390\/ijms19113507","article-title":"A nationwide survey on danon disease in Japan","volume":"19","author":"Sugie","year":"2018","journal-title":"Int J Mol Sci"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0041","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.amjcard.2011.09.024","article-title":"Sudden death associated with danon disease in women","volume":"109","author":"Miani","year":"2012","journal-title":"Am J Cardiol"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0042","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1007\/s10545-016-9932-2","article-title":"Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome","volume":"39","author":"Sentner","year":"2016","journal-title":"J Inherit Metab Dis"},{"issue":"4","key":"10.1016\/j.disamonth.2023.101548_bib0043","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1093\/tropej\/50.4.196","article-title":"Liver Glycogenoses: are they a possible cause of polyneuropathy? A cross-sectional study","volume":"50","author":"Kotb","year":"2004","journal-title":"J Trop Pediatr"},{"issue":"1\u20132","key":"10.1016\/j.disamonth.2023.101548_bib0044","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.ymgme.2015.06.004","article-title":"MELAS syndrome: clinical manifestations, pathogenesis, and treatment options","volume":"116","author":"El-Hattab","year":"2015","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.disamonth.2023.101548_bib0045","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.pediatrneurol.2017.12.005","article-title":"MERRF classification: implications for diagnosis and clinical trials","volume":"80","author":"Finsterer","year":"2018","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0046","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.ijcard.2018.10.051","article-title":"Clinical profile and outcome of cardiac involvement in MELAS syndrome","volume":"276","author":"Brambilla","year":"2019","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_sbref0047","article-title":"Genes frequently associated with sudden death in primary hypertrophic cardiomyopathy","volume":"90","author":"Herrera-Rodr\u00edguez","year":"2020"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0048","doi-asserted-by":"crossref","first-page":"1012","DOI":"10.1016\/j.hrthm.2021.01.019","article-title":"Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: twenty years with the implantable cardioverter-defibrillator","volume":"18","author":"Maron","year":"2021","journal-title":"Heart Rhythm"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0049","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1177\/2047487317702519","article-title":"Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: a systematic review and meta-analysis","volume":"24","author":"Norrish","year":"2017","journal-title":"Eur J Prev Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0050","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.hrtlng.2021.11.006","article-title":"Factors associated with the risk of cardiac death in children with hypertrophic cardiomyopathy: a systematic review and meta-analysis [published online ahead of print, 2021 Nov 24]","volume":"52","author":"Xia","year":"2021","journal-title":"Heart Lung"},{"key":"10.1016\/j.disamonth.2023.101548_bib0051","first-page":"11","article-title":"Risk stratification for sudden death in children with hypertrophic cardiomyopathy: an international multicenter study","author":"Balaji","year":"2017","journal-title":"Am Heart Assoc Sci Sessions"},{"issue":"7","key":"10.1016\/j.disamonth.2023.101548_bib0052","doi-asserted-by":"crossref","first-page":"1943","DOI":"10.1016\/S0735-1097(98)00493-8","article-title":"Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy","volume":"32","author":"Yetman","year":"1998","journal-title":"J Am Coll Cardiol"},{"issue":"10","key":"10.1016\/j.disamonth.2023.101548_bib0053","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1136\/adc.59.10.971","article-title":"Hypertrophic cardiomyopathy: an important cause of sudden death","volume":"59","author":"McKenna","year":"1984","journal-title":"Arch Dis Child"},{"issue":"1","key":"10.1016\/j.disamonth.2023.101548_bib0054","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1161\/01.CIR.65.1.7","article-title":"Hypertrophic cardiomyopathy in infants: clinical features and natural history","volume":"65","author":"Maron","year":"1982","journal-title":"Circulation"},{"key":"10.1016\/j.disamonth.2023.101548_bib0055","doi-asserted-by":"crossref","first-page":"213","DOI":"10.3389\/fphys.2018.00213","article-title":"Distinct ECG phenotypes identified in hypertrophic cardiomyopathy using machine learning associate with arrhythmic risk markers","volume":"9","author":"Lyon","year":"2018","journal-title":"Front Physiol"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0056","doi-asserted-by":"crossref","first-page":"3105","DOI":"10.1111\/apa.16045","article-title":"Sudden cardiac death in childhood hypertrophic cardiomyopathy is best predicted by a combination of electrocardiogram risk-score and HCMRisk-Kids score","volume":"110","author":"\u00d6stman-Smith","year":"2021","journal-title":"Acta Paediatr"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0057","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.jacc.2009.02.071","article-title":"Outcome of patients with hypertrophic cardiomyopathy and a normal electrocardiogram","volume":"54","author":"McLeod","year":"2009","journal-title":"J Am Coll Cardiol"},{"issue":"1","key":"10.1016\/j.disamonth.2023.101548_bib0058","doi-asserted-by":"crossref","DOI":"10.4081\/cardiogenetics.2019.7951","article-title":"Risk stratification in hypertrophic cardiomyopathy: time to think about the electrocardiogram","volume":"9","author":"Skinner","year":"2019","journal-title":"Cardiogenetics"},{"issue":"25","key":"10.1016\/j.disamonth.2023.101548_bib0059","doi-asserted-by":"crossref","first-page":"e159","DOI":"10.1016\/j.jacc.2020.08.045","article-title":"2020 AHA\/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American college of cardiology\/American heart association joint committee on clinical practice guidelines","volume":"76","author":"Ommen","year":"2020","journal-title":"J Am Coll Cardiol"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0060","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1161\/CIRCULATIONAHA.113.007094","article-title":"Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy","volume":"130","author":"Chan","year":"2014","journal-title":"Circulation"},{"issue":"1","key":"10.1016\/j.disamonth.2023.101548_bib0061","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/1532-429X-14-13","article-title":"Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy","volume":"14","author":"Maron","year":"2012","journal-title":"J Cardiovasc Magn Reson"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0062","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1056\/NEJM199703133361107","article-title":"The management of hypertrophic cardiomyopathy","volume":"336","author":"Spirito","year":"1997","journal-title":"N Engl J Med"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0063","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1172\/JCI44972","article-title":"Rapamycin reverses hypertrophic cardiomyopathy in a mouse model of LEOPARD syndrome-associated PTPN11 mutation","volume":"121","author":"Marin","year":"2011","journal-title":"J Clin Invest"},{"issue":"17","key":"10.1016\/j.disamonth.2023.101548_bib0064","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1016\/j.jacc.2019.01.066","article-title":"Hypertrophic cardiomyopathy in Noonan syndrome treated by MEK-inhibition","volume":"73","author":"Andelfinger","year":"2019","journal-title":"J Am Coll Cardiol"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0065","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.ahj.2012.04.018","article-title":"Outcomes in children with noonan syndrome and hypertrophic cardiomyopathy: a study from the pediatric cardiomyopathy registry","volume":"164","author":"Wilkinson","year":"2012","journal-title":"Am Heart J"},{"key":"10.1016\/j.disamonth.2023.101548_bib0066","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1016\/j.amjcard.2015.06.037","article-title":"Surgical ventricular septal myectomy for patients with noonan syndrome and symptomatic left ventricular outflow tract obstruction","volume":"116","author":"Poterucha","year":"2015","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0067","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1161\/CIRCHEARTFAILURE.114.001105","article-title":"Danon disease: clinical features, evaluation, and management","volume":"7","author":"D'souza","year":"2014","journal-title":"Circ Heart Fail"},{"key":"10.1016\/j.disamonth.2023.101548_bib0068","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1007\/s10545-011-9359-8","article-title":"Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management","volume":"34","author":"Braunlin","year":"2011","journal-title":"J Inherit Metab Dis"},{"key":"10.1016\/j.disamonth.2023.101548_bib0069","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1136\/heart.86.5.570","article-title":"Heart transplantation in children with mitochondrial cardiomyopathy","volume":"86","author":"Bonnet","year":"2001","journal-title":"Heart"},{"issue":"13","key":"10.1016\/j.disamonth.2023.101548_bib0070","doi-asserted-by":"crossref","first-page":"287","DOI":"10.21037\/atm.2019.04.67","article-title":"Progress and challenges of gene therapy for Pompe disease","volume":"7","author":"Ronzitti","year":"2019","journal-title":"Ann Transl Med"},{"key":"10.1016\/j.disamonth.2023.101548_bib0071","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1016\/j.omtm.2020.05.034","article-title":"A novel gene therapy approach for GSD III using an AAV vector encoding a bacterial glycogen debranching enzyme","volume":"18","author":"Lim","year":"2020","journal-title":"Mol Ther Methods Clin Dev"},{"issue":"535","key":"10.1016\/j.disamonth.2023.101548_bib0072","doi-asserted-by":"crossref","first-page":"eaax1744","DOI":"10.1126\/scitranslmed.aax1744","article-title":"Systemic AAV9.LAMP2B injection reverses metabolic and physiologic multiorgan dysfunction in a murine model of Danon disease","volume":"12","author":"Manso","year":"2020","journal-title":"Sci Transl Med"},{"issue":"21","key":"10.1016\/j.disamonth.2023.101548_bib0073","doi-asserted-by":"crossref","first-page":"2232","DOI":"10.1161\/CIRCULATIONAHA.106.644682","article-title":"Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction","volume":"114","author":"Maron","year":"2006","journal-title":"Circulation"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0074","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/0002-9149(86)90118-9","article-title":"Clinical significance and therapeutic implications of the left ventricular outflow tract pressure gradient in hypertrophic cardiomyopathy","volume":"58","author":"Maron","year":"1986","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0075","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1161\/01.CIR.26.2.166","article-title":"Studies on digitalis. V. Comparison of the effects of ouabain on left ventricular dynamics in valvular aortic stenosis and hypertrophic subaortic stenosis","volume":"26","author":"Braunwald","year":"1962","journal-title":"Circulation"},{"issue":"20","key":"10.1016\/j.disamonth.2023.101548_bib0076","doi-asserted-by":"crossref","DOI":"10.1056\/NEJM196611172752006","article-title":"Beta-adrenergic receptor blocking drugs. Mechanisms of action and clinical applications","volume":"275","author":"Epstein","year":"1966","journal-title":"N Engl J Med"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0077","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1161\/01.CIR.35.5.847","article-title":"Amelioration of angina pectoris in idiopathic hypertrophic subaortic stenosis with beta-adrenergic blockade","volume":"35","author":"Cohen","year":"1967","journal-title":"Circulation"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0078","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1016\/0002-9149(73)90012-X","article-title":"Hypertrophic subaortic stenosis. Clinical and hemodynamic effects of long-term propranolol therapy","volume":"31","author":"Stenson","year":"1973","journal-title":"Am J Cardiol"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0079","doi-asserted-by":"crossref","first-page":"1813","DOI":"10.1016\/S0735-1097(99)00421-0","article-title":"A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose beta-adrenoceptor antagonist treatment","volume":"34","author":"Ostman-Smith","year":"1999","journal-title":"J Am Coll Cardiol"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0080","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1161\/01.CIR.60.6.1208","article-title":"Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. II. Effects on exercise capacity and symptomatic status","volume":"60","author":"Rosing","year":"1979","journal-title":"Circulation"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0081","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1536\/ihj.27.701","article-title":"Comparable effects of oral diltiazem and verapamil in the treatment of hypertrophic cardiomyopathy. Double-blind crossover study","volume":"27","author":"Toshima","year":"1986","journal-title":"Jpn Heart J"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0082","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.116.005152","article-title":"Safety of outpatient initiation of disopyramide for obstructive hypertrophic cardiomyopathy patients","volume":"6","author":"Adler","year":"2017","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.disamonth.2023.101548_bib0083","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/0002-9149(62)90373-9","article-title":"Hemodynamic alterations in idiopathic hypertrophic subaortic stenosis induced by sympathomimetic drugs","volume":"10","author":"Braunwald","year":"1962","journal-title":"Am J Cardiol"},{"issue":"10","key":"10.1016\/j.disamonth.2023.101548_bib0084","doi-asserted-by":"crossref","first-page":"663","DOI":"10.2165\/00148581-200305100-00002","article-title":"Management of hypertrophic cardiomyopathy in children","volume":"5","author":"Seggewiss","year":"2003","journal-title":"Paediatr Drugs"},{"issue":"1","key":"10.1016\/j.disamonth.2023.101548_bib0085","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1161\/01.CIR.52.1.88","article-title":"Operative treatment in hypertrophic subaortic stenosis. Techniques, and the results of pre and postoperative assessments in 83 patients","volume":"52","author":"Morrow","year":"1975","journal-title":"Circulation"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0086","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1016\/j.jacc.2015.06.1333","article-title":"Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction","volume":"66","author":"Maron","year":"2015","journal-title":"J Am Coll Cardiol"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0087","first-page":"324","article-title":"Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy: US nationwide inpatient database","volume":"2","author":"Kim","year":"2017","journal-title":"JAMA Cardiol"},{"issue":"8969","key":"10.1016\/j.disamonth.2023.101548_bib0088","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0140-6736(95)91267-3","article-title":"Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy","volume":"346","author":"Sigwart","year":"1995","journal-title":"Lancet"},{"issue":"22","key":"10.1016\/j.disamonth.2023.101548_bib0089","doi-asserted-by":"crossref","first-page":"2415","DOI":"10.1161\/01.CIR.98.22.2415","article-title":"Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: results with respect to intraprocedural myocardial contrast echocardiography","volume":"98","author":"Faber","year":"1998","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.disamonth.2023.101548_bib0090","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.amjcard.2003.09.034","article-title":"Conduction system abnormalities in patients with obstructive hypertrophic cardiomyopathy following septal reduction interventions","volume":"93","author":"Qin","year":"2004","journal-title":"Am J Cardiol"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0091","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1136\/heartjnl-2013-304276","article-title":"Atrial fibrillation and thromboembolism in patients with hypertrophic cardiomyopathy: systematic review","volume":"100","author":"Guttmann","year":"2014","journal-title":"Heart"},{"issue":"16","key":"10.1016\/j.disamonth.2023.101548_bib0092","doi-asserted-by":"crossref","first-page":"e147","DOI":"10.1016\/j.jacc.2013.05.019","article-title":"2013 ACCF\/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation\/American heart association task force on practice guidelines","volume":"62","author":"Yancy","year":"2013","journal-title":"J Am Coll Cardiol"},{"issue":"8","key":"10.1016\/j.disamonth.2023.101548_bib0093","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1016\/j.amjcard.2017.12.044","article-title":"Impact of advanced therapies for improving survival to heart transplant in patients with hypertrophic cardiomyopathy","volume":"121","author":"Rowin","year":"2018","journal-title":"Am J Cardiol"},{"issue":"12","key":"10.1016\/j.disamonth.2023.101548_bib0094","first-page":"764","article-title":"Prevention of sudden cardiac death by the implantable cardioverter-defibrillator","volume":"128","author":"Hindricks","year":"2018","journal-title":"Pol Arch Intern Med"},{"key":"10.1016\/j.disamonth.2023.101548_bib0095","doi-asserted-by":"crossref","first-page":"2010","DOI":"10.1093\/eurheartj\/eht439","article-title":"A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD)","volume":"35","author":"O'Mahony","year":"2014","journal-title":"Eur Heart J"},{"issue":"10","key":"10.1016\/j.disamonth.2023.101548_bib0096","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1001\/jama.287.10.1308","article-title":"Hypertrophic cardiomyopathy: a systematic review","volume":"287","author":"Maron","year":"2002","journal-title":"JAMA"},{"issue":"11","key":"10.1016\/j.disamonth.2023.101548_bib0097","doi-asserted-by":"crossref","first-page":"2125","DOI":"10.1016\/j.jacc.2004.08.052","article-title":"Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy","volume":"44","author":"Maron","year":"2004","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.disamonth.2023.101548_bib0098","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.ppedcard.2017.03.003","article-title":"Prevention of sudden cardiac death in children and young adults","volume":"45","author":"Aro","year":"2017","journal-title":"Prog Pediatr Cardiol"},{"issue":"6","key":"10.1016\/j.disamonth.2023.101548_bib0099","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1016\/0002-9149(78)90687-2","article-title":"Long-term medical management of hypertrophic obstructive cardiomyopathy","volume":"42","author":"Frank","year":"1978","journal-title":"Am J Cardiol"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0100","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1111\/j.1472-8206.2010.00869.x","article-title":"Hypertrophic cardiomyopathy in childhood and adolescence - strategies to prevent sudden death","volume":"24","author":"Ostman-Smith","year":"2010","journal-title":"Fundam Clin Pharmacol"},{"issue":"5","key":"10.1016\/j.disamonth.2023.101548_bib0101","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1093\/eurheartj\/ehi108","volume":"26","author":"Corrado","year":"2005","journal-title":"Eur Heart J"},{"issue":"18","key":"10.1016\/j.disamonth.2023.101548_bib0102","doi-asserted-by":"crossref","first-page":"2196","DOI":"10.1093\/eurheartj\/ehl137","article-title":"Evidence for efficacy of the Italian national pre-participation screening programme for identification of hypertrophic cardiomyopathy in competitive athletes","volume":"27","author":"Pelliccia","year":"2006","journal-title":"Eur Heart J"},{"issue":"14","key":"10.1016\/j.disamonth.2023.101548_bib0103","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1136\/heartjnl-2011-300531","article-title":"Hypertrophic cardiomyopathy in children","volume":"98","author":"Moak","year":"2012","journal-title":"Heart"},{"issue":"8","key":"10.1016\/j.disamonth.2023.101548_bib0104","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1016\/j.jacc.2005.04.062","article-title":"Dilated-hypokinetic evolution of hypertrophic cardiomyopathy: prevalence, incidence, risk factors, and prognostic implications in pediatric and adult patients","volume":"46","author":"Biagini","year":"2005","journal-title":"J Am Coll Cardiol"},{"issue":"12","key":"10.1016\/j.disamonth.2023.101548_bib0105","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1016\/j.healun.2009.05.028","article-title":"Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant","volume":"28","author":"Gajarski","year":"2009","journal-title":"J Heart Lung Transpl"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0106","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/j.amjcard.2007.09.085","article-title":"Heart transplantation in hypertrophic cardiomyopathy","volume":"101","author":"Biagini","year":"2008","journal-title":"Am J Cardiol"},{"issue":"3","key":"10.1016\/j.disamonth.2023.101548_bib0107","first-page":"32","article-title":"Heart transplantation in patients with hypertrophic cardiomyopathy","volume":"2018","author":"Torres","year":"2018","journal-title":"Glob Cardiol Sci Pract"}],"container-title":["Disease-a-Month"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0011502923000287?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0011502923000287?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T01:02:59Z","timestamp":1757898179000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0011502923000287"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4]]},"references-count":107,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2023,4]]}},"alternative-id":["S0011502923000287"],"URL":"https:\/\/doi.org\/10.1016\/j.disamonth.2023.101548","relation":{},"ISSN":["0011-5029"],"issn-type":[{"value":"0011-5029","type":"print"}],"subject":[],"published":{"date-parts":[[2023,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Sudden cardiac death in childhood hypertrophic cardiomyopathy","name":"articletitle","label":"Article Title"},{"value":"Disease-a-Month","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.disamonth.2023.101548","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"101548"}}