Paeds Vivas · cardiology
Cardiomyopathies in children — branching viva
Branching viva from the definition and morphological classification of the paediatric cardiomyopathies, through the hypertrophic sudden-death lane, the dilated heart-failure and transplant lane, the tachycardiomyopathy trap, and the family cascade-screening and surveillance strategy.
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Study tools
Target exams
RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar in clinic. The consultant asks you to talk through four children: a 14-year-old runner who collapsed during a race whose father died suddenly at 38, a three-month-old in heart failure with a heart rate of 230, a well sibling of a patient with hypertrophic cardiomyopathy whose genetic test is positive but whose echo is normal, and a nine-year-old with restrictive physiology and giant atria.
Station opening
Examiner: "Define the paediatric cardiomyopathy and reproduce the morphological classification." [2]
Strong candidate (must-hit)
- Defines a cardiomyopathy as an intrinsic disease of myocardial structure or function, distinct from secondary hypertensive, valvular, or congenital disease, citing the 2006 AHA and 2008 ESC definitions; reproduces the five morphological types (dilated, hypertrophic, restrictive, left ventricular non-compaction, arrhythmogenic) with their echo hallmarks and approximate shares; explains that morphology is the bedside frame that drives the differential, the symptoms, and the complication to watch for, with the genotype layered on top to refine risk and family screening. [2]
Weak candidate
- "A cardiomyopathy is a big heart." [2]
References7ShowHide
- [2]Maron BJ; Towbin JA; Thiene G; Antzelevitch C; Corrado D; Arnett D; et al Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement. Circulation, 2006.PMID 16567565
- [4]Maron BJ Hypertrophic cardiomyopathy: a systematic review. JAMA, 2002.PMID 11886323
- [5]Maron BJ; Shen WK; Link MS; Epstein AE; Almquist AK; Daubert JP; et al Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med, 2000.PMID 10666426
- [6]Bagnall RD; Weintraub RG; Ingles J; Duflou J; Yeates L; Lam L; et al A Prospective Study of Sudden Cardiac Death among Children and Young Adults. N Engl J Med, 2016.PMID 27332903
- [7]Shaddy RE; Boucek MM; Hsu DT; Boucek RJ; Canter CE; Mahony L; et al Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA, 2007.PMID 17848651
- [8]Burkett EL; Hershberger RE Clinical and genetic issues in familial dilated cardiomyopathy. J Am Coll Cardiol, 2005.PMID 15808750
- [9]Alexander PMA; Nugent AW; Daubeney PEF; Lee KJ; Sleeper LA; Schuster T; et al Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood: Results From a National Population-Based Study. Circulation, 2018.PMID 29490994