Why the study?
Localised hypertrophy of the inferolateral basal wall of the LV was noted in women with Marfan syndrome and MVP, prompting investigation into its prevalence, relationship with MVP and MAD, and prognostic value.
Does the presence of basal inferolateral hypertrophy (BILH) predict adverse clinical events in patients with Marfan syndrome?
Population
241 consecutive MFS patients aged >14 years with a pathogenic FBN1 variant and no prior thoracic surgery
Comparison
Presence vs absence of basal inferolateral hypertrophy
Design
Prospective single-centre cohort study
Follow-up
More than 6 years
Key result
Basal inferolateral LV hypertrophy occurred in 7.9% of Marfan patients, mainly women, and was linked to higher clinical events risk over 6 years independent of MVP or MAD.
Authors
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May warrant closer monitoring in Marfan patients; hypothesis-generating and requires prospective validation.
Does the presence of basal inferolateral hypertrophy (BILH) predict adverse clinical events in patients with Marfan syndrome?
Localized basal inferolateral left ventricular hypertrophy is a frequent echocardiographic finding in women with Marfan syndrome and mitral valve prolapse, and it is associated with a higher risk of adverse clinical events.
Milleron et al. (2025) studied this question. Basal inferolateral LV hypertrophy occurred in 7.9% of Marfan patients, mainly women, and was linked to higher clinical events risk over 6 years independent of MVP or MAD.
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