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• W3138698154 abstract "HomeCirculationVol. 143, No. 11Response by Mital et al to Letter Regarding Article, “A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy” Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBResponse by Mital et al to Letter Regarding Article, “A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy” Seema Mital, MD, Katey R. Armstrong, MBChB, MD, Ryan J. Butts, MD, Jennifer Conway, MD, Steve Fan, PhD, PStat, Letizia Gardin, MD, Paul F. Kantor, MBBCh, Beth Kaufman, MD, Myriam Lafreniere-Roula, PhD, Anastasia Miron, HBA, Marc E. Richmond, MD, MS, Joseph W. Rossano, MD, Mark W. Russell, MD, Chet Villa, MD, Robert G. Weintraub, MBBS and PRIMaCY Investigators Seema MitalSeema Mital https://orcid.org/0000-0002-7643-4484 Department of Pediatrics and Genetics and Genome Biology Program (S.M.), Hospital for Sick Children, University of Toronto, Ontario, Canada. Search for more papers by this author , Katey R. ArmstrongKatey R. Armstrong Department of Pediatrics, British Columbia Children’s Hospital, Vancouver, Canada (K.R.A.). Search for more papers by this author , Ryan J. ButtsRyan J. Butts Department of Pediatrics, Children’s Medical Center of Dallas, TX (R.J.B.). Search for more papers by this author , Jennifer ConwayJennifer Conway Department of Pediatrics, Stollery Children’s Hospital, Edmonton, Alberta, Canada (J.C.). Search for more papers by this author , Steve FanSteve Fan Ted Rogers Computational Program, Ted Rogers Centre for Heart Research, University Health Network, Toronto, Ontario, Canada (S.F., M.L.-R.). Search for more papers by this author , Letizia GardinLetizia Gardin Department of Pediatrics, Children’s Hospital of Eastern Ontario, Ottawa, Canada (L.G.). Search for more papers by this author , Paul F. KantorPaul F. Kantor Department of Pediatrics, Children’s Hospital of Los Angeles, CA (P.F.K.). Search for more papers by this author , Beth KaufmanBeth Kaufman Department of Pediatrics, Lucile Packard Children’s Hospital, Stanford, Palo Alto, CA (B.K.). Search for more papers by this author , Myriam Lafreniere-RoulaMyriam Lafreniere-Roula Department of Pediatrics, Morgan Stanley Children’s Hospital, Columbia University College of Physicians and Surgeons, NY (M.E.R.). Ted Rogers Computational Program, Ted Rogers Centre for Heart Research, University Health Network, Toronto, Ontario, Canada (S.F., M.L.-R.). Search for more papers by this author , Anastasia MironAnastasia Miron Search for more papers by this author , Marc E. RichmondMarc E. Richmond https://orcid.org/0000-0001-5491-8085 Search for more papers by this author , Joseph W. RossanoJoseph W. Rossano https://orcid.org/0000-0002-8284-0673 Department of Pediatrics, Children’s Hospital of Philadelphia, PA (J.W.R.). Search for more papers by this author , Mark W. RussellMark W. Russell Department of Pediatrics, C.S. Mott Children’s Hospital, Ann Arbor, MI (M.W.R.). Search for more papers by this author , Chet VillaChet Villa https://orcid.org/0000-0002-8195-0694 The Heart Institute, Cincinnati Children’s Hospital, OH (C.V.). Search for more papers by this author , Robert G. WeintraubRobert G. Weintraub Department of Cardiology, The Royal Children’s Hospital, Melbourne, Victoria, Australia (R.G.W.). Murdoch Children’s Research Institute, University of Melbourne, Victoria, Australia (R.G.W.). Search for more papers by this author and PRIMaCY Investigators Search for more papers by this author Originally published15 Mar 2021https://doi.org/10.1161/CIRCULATIONAHA.120.051632Circulation. 2021;143:e788–e789In Response:Our article described age-specific risk factors for sudden cardiac death (SCD) in an international pediatric hypertrophic cardiomyopathy (HCM) cohort.1 Grace et al, in their letter, state that SCD incidence is lower. The studies cited included patients ages 10 to 45 years in whom the event rate would be expected to be different than our early onset pediatric cohort. It is important to note that ours was not a population study to define SCD incidence but rather to risk-stratify patients followed in hospitals where implantable cardioverter-defibrillator decisions are made. We appreciate that, as patients age, other causes of death emerge.2 However, in children, SCD remains the leading cause of death.1,3 There was no difference in the freedom from SCD in our patients diagnosed before versus after 2000.Regarding appropriate shocks as a surrogate outcome, this was consistent with our goal of developing a prediction model for events that cause SCD, need resuscitation, or trigger appropriate implantable cardioverter-defibrillator shocks. In that regard, appropriate shocks even if they occurred for arrhythmias that may otherwise have self-terminated are an accepted outcome.2–4 Nonetheless, the use of a surrogate outcome was acknowledged in our limitations.Known Danon cases were excluded except for 1 patient with a PRKAG2 mutation who did not experience an SCD event. Non-SCD deaths included 5 heart failure-related, 2 posttransplant, 1 postmyectomy, and 1 perinatal asphyxia.We are surprised that Grace et al, in their letter, define primary HCM only as patients with a sarcomeric gene variant. Although sarcomeric gene variants are the most frequent cause, defining primary HCM through such a narrow lens ignores the genetic diversity of HCM. It also presumes that those who decline genetic testing, who harbor variants of uncertain significance, or who are gene elusive on testing should not be considered primary HCM. This effectively excludes almost 50% of families! Moreover, extracardiac manifestations are often absent in those with nonsarcomeric gene variants, as seen in our cohort and reported in many studies including Danon disease, which underscores the phenotypic variability in HCM.5 In practice, genetic information may not be available to physicians at the time of SCD risk assessment. Risk assessment approaches therefore need to be inclusive. We stand by our approach of including genotype into the prediction model as opposed to excluding patients by genotype. This is central to precision medicine as our understanding of the genetic heterogeneity of HCM grows.We disagree with Miron et al1 that our article showed enhanced risk in nonsarcomeric HCM given we did not compare sarcomeric with nonsarcomeric HCM. We also disagree with their questioning of the diligence in data compilation and analysis. Data accuracy was verified, and all SCD and non-SCD deaths were adjudicated by site leads before analyses. A competing-outcomes model was applied rather than a simple regression framework. Validation of our findings in an independent cohort provided the ultimate and definitive evidence for the reproducibility and validity of our results. We strongly believe that our foundational work provides a strong framework for evidence-based risk stratification in this vulnerable population and the basis to expand the model further as new evidence-based risk factors emerge.Disclosures Dr Conway is a medical monitor for the Pumpkin Trial and has received unrestricted education funding from Abbott. Dr Rossano is a consultant for Amgen, Bayer, Novartis, and CLS Behring. Dr Kantor is a consultant for Novartis and Astra-Zeneca. The other authors report no conflicts.Footnoteshttps://www.ahajournals.org/journal/circReferences1. Miron A, Lafreniere-Roula M, Steve Fan CP, Armstrong KR, Dragulescu A, Papaz T, Manlhiot C, Kaufman B, Butts RJ, Gardin L, et al.. A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy.Circulation. 2020; 142:217–229. doi: 10.1161/CIRCULATIONAHA.120.047235LinkGoogle Scholar2. Ho CY, Day SM, Ashley EA, Michels M, Pereira AC, Jacoby D, Cirino AL, Fox JC, Lakdawala NK, Ware JS, et al.. Genotype and lifetime burden of disease in hypertrophic cardiomyopathy: insights from the Sarcomeric Human Cardiomyopathy Registry (SHaRe).Circulation. 2018; 138:1387–1398. doi: 10.1161/CIRCULATIONAHA.117.033200LinkGoogle Scholar3. Norrish G, Ding T, Field E, Ziólkowska L, Olivotto I, Limongelli G, Anastasakis A, Weintraub R, Biagini E, Ragni L, et al.. Development of a novel risk prediction model for sudden cardiac death in childhood hypertrophic cardiomyopathy (HCM Risk-Kids).JAMA Cardiol. 2019; 4:918–927. doi: 10.1001/jamacardio.2019.2861CrossrefMedlineGoogle Scholar4. Balaji S, DiLorenzo MP, Fish FA, Etheridge SP, Aziz PF, Russell MW, Tisma S, Pflaumer A, Sreeram N, Kubus P, et al.. Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: An international multicenter study.Heart Rhythm. 2019; 16:1462–1467. doi: 10.1016/j.hrthm.2019.04.040CrossrefMedlineGoogle Scholar5. Lotan D, Salazar-Mendiguchía J, Mogensen J, Rathore F, Anastasakis A, Kaski J, Garcia-Pavia P, Olivotto I, Charron P, Biagini E, et al.; Cooperating Investigators‡. Clinical profile of cardiac involvement in danon disease: a multicenter European registry.Circ Genom Precis Med. 2020; 13:e003117. doi: 10.1161/CIRCGEN.120.003117LinkGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails March 16, 2021Vol 143, Issue 11 Advertisement Article InformationMetrics © 2021 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.120.051632PMID: 33720772 Originally publishedMarch 15, 2021 PDF download Advertisement SubjectsEmbolismVascular Disease" @default.
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