{"id":"46035","type":"article","url":"https://hartvaat.nl/2026/04/17/massieve-linkerventrikelhypertrofie-bij-kinderen-met-hypertrofische-cardiomyopat/","title":"Massieve linkerventrikelhypertrofie bij kinderen met hypertrofische cardiomyopathie (multiregisteranalyse)","title_en":"The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis","category":"algemeen","category_label":"Algemeen","professions":["cardioloog"],"tags":[],"journal":"Circulation","doi":"https://www.ahajournals.org/doi/abs/10.1161/CIRCULATIONAHA.126.078843","source_url":"https://doi.org/https://www.ahajournals.org/doi/abs/10.1161/CIRCULATIONAHA.126.078843","authors":["Robert Przybylski"],"significance":8,"published":"2026-07-15","source_date":"2026-04-17","image":"","kennis":["https://hartvaat.nl/kennis/ritmestoornissen/sport-en-ritmestoornissen/","https://hartvaat.nl/kennis/cardiometabool/obesitas-en-hart/"],"congress":"","summary_en":"Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but its natural history is poorly understood. This analysis from two large international registries (SHaRe and IPHCC) compares children with HCM with and without massive LVH — defined as maximal wall thickness ≥30 mm or a z-score ≥+20 before age 18 — using serial wall-thickness measurements and composite outcomes for major ventricular arrhythmia events (sudden cardiac death, aborted sudden cardiac death or appropriate ICD therapy) and heart failure. The study characterises the course and risk profile of this severe phenotype to support risk stratification and treatment decisions such as ICD implantation.","created":"2026-07-03T20:27:08Z","updated":"2026-07-04T19:27:01Z","licence":"Citeer vrij, met bronvermelding en een link naar hartvaat.nl (de url van het record). Samenvattingen zijn redactioneel werk van HartVaat; de oorspronkelijke publicaties blijven van hun uitgevers (doi). Geen medisch advies.","body_markdown":"Massieve linkerventrikelhypertrofie (LVH) is een risicofactor voor plotse hartdood bij kinderen met hypertrofische cardiomyopathie (HCM), maar het natuurlijke beloop is nauwelijks bekend. Deze analyse uit twee grote internationale registers (SHaRe en IPHCC) vergelijkt kinderen met HCM mét en zónder massieve LVH — gedefinieerd als een maximale wanddikte ≥30 mm of een z-score ≥+20 vóór de leeftijd van 18 jaar — met seriële wanddiktemetingen en samengestelde uitkomsten voor ernstige ventriculaire ritmestoornissen (plotse hartdood, afgebroken plotse hartdood of terechte ICD-therapie) en hartfalen. De studie brengt het beloop en risicoprofiel van deze ernstige fenotypegroep in kaart, ter ondersteuning van de risicostratificatie en behandelkeuzes zoals een ICD.","abstract_original":"BACKGROUND:Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but little is understood about its natural history.METHODS:Patients with pediatric-onset HCM identified from 2 registries (SHaRe [Sarcomeric Human Cardiomyopathy Registry] and IPHCC [International Paediatric Hypertrophic Cardiomyopathy Consortium]) with or without massive LVH were compared. Massive LVH was defined as absolute maximal left ventricular wall thickness (MLVWT) ≥30 mm or MLVWTzscore ≥+20 at <18 years of age. Data from SHaRe and IPHCC include encounters from January 1960 through March 2024 and January 1970 through March 2024, respectively. Demographic, clinical, and serial MLVWT data were collected. Composite outcomes included major ventricular arrhythmia event (sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter defibrillator therapy); heart failure (HF) event (left ventricular ejec"}