Asymmetric hypertrophic cardiomyopathy in generalized lipodystrophy characterized by cardiovascular magnetic resonance

1. Asymmetric Hypertrophic Cardiomyopathy
in Generalized Lipodystrophy Characterized
by Cardiovascular Magnetic Resonance
Alessandra Scatteia, Carmen Pagano, Carmine Pascale, Pasquale Guarini, Gennaro
Marotta, Pasquale Perrone-Filardi, Santo Dellegrottaglie.
Alessandra Scatteia
Department of Advanced Biomedical
Sciences,
Federico II University,
Naples, Italy

2. Clinical Case
Current presentation
ü30 year old male
üCongenital generalized lipodystrophy
üEcho: asymmetric septal LVH
üECG: new inverted T waves in precordial leads
üReferred to CMR ? cardiomyopathy
Concurrent issues
üHepatic steatosis and hepatosplenomegaly
üImpaired fasting glucose
üThrombocytopenia
üMild hypertriglyceridemia

3. CMR Findings
Cine SSFP
Septal LVH
(22mm)
Area of
hypointensity
Hypo/hyper-
intensity
(mixed signal)
Area of
hyperintensity
LGE
FSE-T1
STIR-T2
Left ventricular hypertrophy with fibrosis

4. Discussion
• This case: cardiac involvement in congenital generalized lipodystrophy
• Lipodystrophies (generalized or partial) are rare diseases
• The loss of adipose tissue severe metabolic alterations
• insulin resistance
• hypertriglyceridemia
• fat deposits in lymphoreticular tissues
• Most frequent cardiac abnormality: symmetric LVH

5. Conclusion
• CMR can provide non-invasive myocardial tissue characterisation
• CMR can provide differential diagnosis in LVH phenotypes
(HCM, Fabry’s, sarcoidosis, etc)
• Cardiac involvement in lipodystrophy is associated with poor prognosis
Lypodistrophy or sarcomeric Hypertrophic Cardiomyopathy?

6. Declaration of Interest
I have nothing to declare