Heart failure & cardiomyopathies
Evidence of cardiomyopathy associated with Marfan syndrome in children
Authors' conclusion
Ventricular dilation and reduced EF are common in children with MFS and occur with no/mild LV volume load, suggesting intrinsic cardiomyopathy. MVP may be associated with cardiomyopathy.
Study context
- Population
- Patients with Marfan syndrome <19 years old at first cardiac MRI60% male
- Participants
- 58 patients
- Outcome
- Ventricular size and function (biventricular volumes and ejection fraction (EF) vs normative paediatric cMRI values)
Methodological detailsMedian age at initial cardiac MRI (cMRI) 13.6 years (IQR 10.0-15.8 years)
- Median age at initial cMRI 13.6 years (IQR 10.0-15.8 years)
Main finding
As reported in the abstract
LV size and function with absent/mild LV volume load (n=44, 76%) · initial cMRI
Indexed left ventricular (LV) end-diastolic volume (EDV) z-score (median), significantly increased above normative values
1.8
IQR 0.6–3.5p<0.0001
Abnormal LVEF (<55%)
48%
21/44
Observational data: associations, not effects.
Further findings
Mitral valve prolapse (MVP) in the absence of volume loading
- MVP associated with larger ventricular volumes and lower LVEF
Longitudinal change in LVEF and EDV z-scores (serial cMRI) · mean follow-up 1.5 years
- No significant change
Original publication
Evidence of cardiomyopathy associated with Marfan syndrome in children. Heart. Published 30 May 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →