The Prevalence and Association of Exercise Test Abnormalities With Sudden Cardiac Death and Transplant-Free Survival in Childhood Hypertrophic Cardiomyopathy.

التفاصيل البيبلوغرافية
العنوان:	The Prevalence and Association of Exercise Test Abnormalities With Sudden Cardiac Death and Transplant-Free Survival in Childhood Hypertrophic Cardiomyopathy.
المؤلفون:	Conway J; Department of Pediatrics, Stollery Children's Hospital, Edmonton, Canada (J.C.)., Min S; Genetics and Genome Biology, Hospital for Sick Children, Toronto, Canada (S. Min, S. Mital)., Villa C; Department of Pediatrics, Cincinnati Children's Hospital, OH (C.V.)., Weintraub RG; Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia (R.G.W.)., Nakano S; Department of Pediatrics, Children's Hospital Colorado, Aurora (S.N.)., Godown J; Department of Pediatrics, Monroe Carrell Jr Children's Hospital at Vanderbilt, Nashville, TN (J.G.)., Tatangelo M; Ted Rogers Computational Program, Peter Munk Cardiac Centre, University Health Network, Toronto, Canada (M.T.)., Armstrong K; Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada (K.A.)., Richmond M; Department of Pediatrics, Morgan Stanley Children's Hospital, Columbia University Medical Centre, New York, NY (M.R.)., Kaufman B; Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA (B.K.)., Lal AK; Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City (A.K.L.)., Balaji S; Department of Pediatrics, Oregon Health and Science University, Portland (S.B.)., Power A; Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX (A.P., N.B.H.)., Baez Hernandez N; Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX (A.P., N.B.H.)., Gardin L; Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Canada (L.G.)., Kantor PF; Department of Pediatrics, Children's Hospital of Los Angeles, CA (P.F.K.)., Parent JJ; Department of Pediatrics, Riley Children's Hospital, Indianapolis, IN (J.J.P.)., Aziz PF; Department of Pediatrics, Cleveland Clinic Children's Hospital, OH (P.F.A.)., Jefferies JL; Department of Pediatrics, University of Tennessee Health Sciences Centre, Memphis (J.L.J.)., Dragulescu A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Canada (A.D., A.J., L.B., S. Mital)., Jeewa A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Canada (A.D., A.J., L.B., S. Mital)., Benson L; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Canada (A.D., A.J., L.B., S. Mital)., Russell MW; Department of Pediatrics, University of Michigan Health System, Ann Arbor (M.W.R.)., Whitehill R; Department of Pediatrics, Children's Healthcare of Atlanta, GA (R.W.)., Rossano J; Department of Pediatrics, Children's Hospital of Philadelphia, PA (J.R.)., Howard T; Department of Pediatrics, Texas Children's Hospital, Houston (T.H.)., Mital S; Genetics and Genome Biology, Hospital for Sick Children, Toronto, Canada (S. Min, S. Mital).; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Canada (A.D., A.J., L.B., S. Mital).; Ted Rogers Centre for Heart Research, Toronto, Canada (S. Mital).
المصدر:	Circulation [Circulation] 2023 Feb 28; Vol. 147 (9), pp. 718-727. Date of Electronic Publication: 2022 Nov 06.
نوع المنشور:	Journal Article; Research Support, Non-U.S. Gov't
اللغة:	English
بيانات الدورية:	Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 0147763 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1524-4539 (Electronic) Linking ISSN: 00097322 NLM ISO Abbreviation: Circulation Subsets: MEDLINE
أسماء مطبوعة:	Publication: Hagerstown, MD : Lippincott Williams & Wilkins Original Publication: [Dallas, Tex., etc., American Heart Association, etc.]
مواضيع طبية MeSH:	Exercise Test* , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic*/surgery, Male ; Female ; Humans ; Cohort Studies ; Prevalence ; Death, Sudden, Cardiac/epidemiology ; Death, Sudden, Cardiac/etiology ; Arrhythmias, Cardiac/etiology ; Risk Factors
مستخلص:	Background: Hypertrophic cardiomyopathy (HCM) can be associated with an abnormal exercise response. In adults with HCM, abnormal results on exercise stress testing are predictive of heart failure outcomes. Our goal was to determine whether an abnormal exercise response is associated with adverse outcomes in pediatric patients with HCM. Methods: In an international cohort study including 20 centers, phenotype-positive patients with primary HCM who were <18 years of age at diagnosis were included. Abnormal exercise response was defined as a blunted blood pressure response and new or worsened ST- or T-wave segment changes or complex ventricular ectopy. Sudden cardiac death (SCD) events were defined as a composite of SCD and aborted sudden cardiac arrest. Using Kaplan-Meier survival, competing outcomes, and Cox regression analyses, we analyzed the association of abnormal exercise test results with transplant and SCD event-free survival. Results: Of 724 eligible patients, 630 underwent at least 1 exercise test. There were no major differences in clinical characteristics between those with or without an exercise test. The median age at exercise testing was 13.8 years (interquartile range, 4.7 years); 78% were male and 39% were receiving beta-blockers. A total of 175 (28%) had abnormal test results. Patients with abnormal test results had more severe septal hypertrophy, higher left atrial diameter z scores, higher resting left ventricular outflow tract gradient, and higher frequency of myectomy compared with participants with normal test results ( P <0.05). Compared with normal test results, abnormal test results were independently associated with lower 5-year transplant-free survival (97% versus 88%, respectively; P =0.005). Patients with exercise-induced ischemia were most likely to experience all-cause death or transplant (hazard ratio, 4.86 [95% CI, 1.69-13.99]), followed by those with an abnormal blood pressure response (hazard ratio, 3.19 [95% CI, 1.32-7.71]). Exercise-induced ischemia was also independently associated with lower SCD event-free survival (hazard ratio, 3.32 [95% CI, 1.27-8.70]). Exercise-induced ectopy was not associated with survival. Conclusions: Exercise abnormalities are common in childhood HCM. An abnormal exercise test result was independently associated with lower transplant-free survival, especially in those with an ischemic or abnormal blood pressure response with exercise. Exercise-induced ischemia was also independently associated with SCD events. These findings argue for routine exercise testing in childhood HCM as part of ongoing risk assessment.
التعليقات:	Comment in: Circulation. 2023 Jul 4;148(1):73. (PMID: 37399261)
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فهرسة مساهمة:	Keywords: cardiomyopathy, hypertrophic; death, sudden, cardiac; defibrillators, implantable; exercise test; pediatrics
تواريخ الأحداث:	Date Created: 20221106 Date Completed: 20230301 Latest Revision: 20230715
رمز التحديث:	20230715
مُعرف محوري في PubMed:	PMC9977414
DOI:	10.1161/CIRCULATIONAHA.122.062699
PMID:	36335467
قاعدة البيانات:	MEDLINE

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تدمد:	1524-4539
DOI:	10.1161/CIRCULATIONAHA.122.062699