Cardiac steatosis and left ventricular hypertrophy in patients with generalized lipodystrophy as determined by magnetic resonance spectroscopy and imaging

Michael D. Nelson, Ronald G. Victor, Edward W. Szczepaniak, Vinaya Simha, Abhimanyu Garg, Lidia S. Szczepaniak

Research output: Contribution to journalArticle

34 Citations (Scopus)

Abstract

Generalized lipodystrophy is a rare disorder characterized by marked loss of adipose tissue with reduced triglyceride storage capacity, leading to a severe form of metabolic syndrome including hypertriglyceridemia, insulin resistance, type 2 diabetes mellitus, and hepatic steatosis. Recent echocardiographic studies suggest that concentric left ventricular (LV) hypertrophy is another characteristic feature of this syndrome, but the mechanism remains unknown. It has recently been hypothesized that the LV hypertrophy could be an extreme clinical example of "lipotoxic cardiomyopathy": excessive myocyte accumulation of triglyceride leading to adverse hypertrophic signaling. To test this hypothesis, the first cardiac magnetic resonance study of patients with generalized lipodystrophy was performed, using magnetic resonance imaging and localized proton spectroscopy to detect excessive triglyceride content in the hypertrophied myocytes. Six patients with generalized lipodystrophy and 6 healthy controls matched for age, gender, and body mass index were studied. As hypothesized, myocardial triglyceride content was threefold higher in patients than controls: 0.6 ± 0.2% versus 0.2 ± 0.1% (p = 0.004). The presence of pericardial fat was also found, representing a previously undescribed adipose depot in generalized lipodystrophy. Patients with generalized lipodystrophy, compared with controls, also had a striking degree of concentric LV hypertrophy, independent of blood pressure: LV mass index 101.0 ± 18.3 versus 69.0 ± 17.7 g/m2, respectively (p = 0.02), and LV concentricity 1.3 ± 0.3 versus 0.99 ± 0.1 g/ml, respectively (p = 0.04). In conclusion, these findings advance the lipotoxicity hypothesis as a putative underlying mechanism for the dramatic concentric LV hypertrophy found in generalized lipodystrophy.

Original languageEnglish (US)
Pages (from-to)1019-1024
Number of pages6
JournalAmerican Journal of Cardiology
Volume112
Issue number7
DOIs
StatePublished - Oct 1 2013

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Congenital Generalized Lipodystrophy
Left Ventricular Hypertrophy
Magnetic Resonance Spectroscopy
Magnetic Resonance Imaging
Triglycerides
Muscle Cells
Hypertriglyceridemia
Cardiomyopathies
Type 2 Diabetes Mellitus
Insulin Resistance
Protons
Adipose Tissue
Spectrum Analysis
Body Mass Index
Fats
Blood Pressure
Liver

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Cardiac steatosis and left ventricular hypertrophy in patients with generalized lipodystrophy as determined by magnetic resonance spectroscopy and imaging. / Nelson, Michael D.; Victor, Ronald G.; Szczepaniak, Edward W.; Simha, Vinaya; Garg, Abhimanyu; Szczepaniak, Lidia S.

In: American Journal of Cardiology, Vol. 112, No. 7, 01.10.2013, p. 1019-1024.

Research output: Contribution to journalArticle

Nelson, Michael D. ; Victor, Ronald G. ; Szczepaniak, Edward W. ; Simha, Vinaya ; Garg, Abhimanyu ; Szczepaniak, Lidia S. / Cardiac steatosis and left ventricular hypertrophy in patients with generalized lipodystrophy as determined by magnetic resonance spectroscopy and imaging. In: American Journal of Cardiology. 2013 ; Vol. 112, No. 7. pp. 1019-1024.
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