{"id":"fc9aa44567b1","type":"article","url":"https://hartvaat.nl/2026/05/22/lge-granulariteit-op-cmr-voorspelt-sterfte-bij-eindstadium-hypertrofische-cardio/","title":"LGE-granulariteit op CMR voorspelt sterfte bij eindstadium hypertrofische cardiomyopathie","title_en":"","category":"hartfalen","category_label":"Hartfalen","professions":["cardioloog"],"tags":["biomarkers-cardiovasculair","cardiomyopathie-gerichte-therapie","gedilateerde-cardiomyopathie","hypertrofische-cardiomyopathie","laminopathie"],"journal":"Circulation. Cardiovascular imaging","doi":"10.1161/CIRCIMAGING.125.019408","source_url":"https://doi.org/10.1161/CIRCIMAGING.125.019408","authors":["Julien Hudelo","Jérôme Garot","Solenn Toupin","Suzanne Duhamel","Francesca Sanguineti","Philippe Garot","Thomas Hovasse","Stéphane Champagne","Thierry Unterseeh","Mariama Akodad","Antoinette Neylon","Trecy Gonçalves","Alexandre Unger","Jeremy Florence","Jean-Guillaume Dillinger","Christophe Tribouilloy","Alexis Hermida","Valérie Bousson","Théo Pezel","Yohann Bohbot"],"significance":7,"published":"2026-06-06","source_date":"2026-05-22","image":"","kennis":["https://hartvaat.nl/kennis/hartfalen/ischemische-cardiomyopathie/","https://hartvaat.nl/kennis/hartfalen/hypertrofische-cardiomyopathie/"],"congress":"","summary_en":"Retrospective multicenter cohort of 691 patients with end-stage HCM (LVEF <50%) from three French tertiary centres, followed for a median of 9 years. After 33% mortality (n=226), the LGE granularity model — combining extent (unique vs multiple), location (septal vs other) and pattern (subepicardial vs midwall) — provided independent prognostic value. Late gadolinium enhancement was associated with mortality after adjustment (HR 1.52). Each component independently predicted mortality: LGE extent (HR 2.85), septal location (HR 1.73), and midwall pattern (HR 4.15). The LGE granularity model refines risk stratification in advanced HCM.","created":"2026-07-03T10:33:00Z","updated":"2026-07-03T13:31:54Z","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":"Retrospectief multicenter cohort van 691 patiënten met eindstadium HCM (LVEF <50%) uit drie Franse universitaire centra, gemiddeld 9 jaar gevolgd. Na 33% mortaliteit (n=226) bleek het LGE-granulariteitsmodel — combinatie van uitgebreidheid (uniek vs meervoudig), lokalisatie (septaal vs anders) en patroon (subepicardiaal vs midwall) — onafhankelijk prognostisch. Late gadolinium enhancement was geassocieerd met sterfte na correctie (HR 1,52). Elk LGE-component was zelfstandig voorspellend: uitgebreidheid (HR 2,85), septale lokalisatie (HR 1,73) en midwall-patroon (HR 4,15). De granulariteit van LGE biedt verfijning van risicostratificatie bij deze gevorderde HCM-vorm.","abstract_original":"BACKGROUND: End-stage hypertrophic cardiomyopathy (HCM) is a distinct and advanced form of HCM, defined by a left ventricular ejection fraction <50% and associated with a markedly poor prognosis. Evidence on the prognostic relevance of late gadolinium enhancement (LGE) and its key features in end-stage HCM remains limited. The aim of our study was to evaluate the prognostic value of the LGE granularity model, including its location, extent, and pattern in patients with end-stage HCM. METHODS: All patients referred for cardiovascular magnetic resonance assessment of HCM at 3 French tertiary university hospitals between 2008 and 2024 were retrospectively screened, and all patients with a left ventricular ejection fraction <50% were included. The LGE granularity model was defined as a model combining LGE extent (unique versus multiple involvement), location (septal versus other), and pattern (subepicardial versus midwall). The primary end point was all-cause mortality. RESULTS: Among 2873 patients with HCM, 691 (24%) with end-stage HCM were included (52±7 years, 54% male). After a median follow-up of 9 years (interquartile range, 6-11), 226 patients died (33%). LGE was observed in 259 (37%) patients and was associated with mortality, even after adjustment for classical prognostic factors (hazard ratio, 1.52 [95% CI, 1.07-2.18]; P=0.02). Each LGE granularity component was independently associated with mortality after adjustment: LGE extent (hazard ratio, 2.85 [95% CI, 1.03-7.85]; P=0.02), septal location (hazard ratio, 1.73 [95% CI, 1.10-2.73]; P<0.001), and midwall pattern (hazard ratio, 4.15 [95% CI, 1.79-9.61]; P<0.001). CONCLUSIONS: In this large multicenter cohort of patients with end-stage HCM, the LGE granularity model integrating LGE extent, location, and pattern provided strong and independent prognostic value."}