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.
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.
Absolute Event Rate: 0% vs 0%
Abstract Introduction We noted the presence of localised hypertrophy of the inferolateral basal wall of the left ventricle (LV) in women with Marfan syndrome (MFS) and mitral valve prolapse (MVP). Objective To describe this basal inferolateral hypertrophy (BILH), report its prevalence, study the relationship with MVP, mitral annular disjunction (MAD) and assess its prognosis in patients with MFS. Method In this prospective cohort study, we included consecutive patients seen in our centre with a pathogenic variant of FBN1, aged 14 years and without previous thoracic surgery. Using transthoracic echocardiography, we measured the diastolic thickness of the basal and mid segments of the LV inferolateral wall and report presence or absence of MVP and MAD. BILH was defined as the conjunction of an inferolateral basal segment thickness ≥12mm and a basal to mid segment wall thickness ratio ≥1.5. Patients were followed for clinical events: aortic events, mitral valve surgery, symptomatic ventricular tachycardia or ICD implantation and cardiovascular death. Results 241 patients with MFS were included. MVP was found in 38.1%, MAD in 21.5% and BILH in 7.9% of patients. BILH was 4.6 times more frequent in patients with bivalvular MVP and 3.1 times more frequent in patients with MAD and was associated with a longer QTc (mean: 426 ms vs. 411 ms, p=0.022). BILH was observed almost exclusively in women (17/19). In the 2 men with BILH, a bivalvular MVP was present in both and MAD in one. In women, BILH, but not MVP or MAD, was associated with the occurrence of clinical events after more than 6 years of follow-up. When the entire population was included (men and women), significance was lost. Conclusions Localized basal left ventricular hypertrophy is present in women with MFS and is associated with MVP and MAD. Its presence is associated with subsequent clinical events and therefore has prognostic implications.
Milleron et al. (Sat,) reported a other. 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.