CARDIAC FINDINGS IN GENERALIZED LIPODYSTROPHY

The cardiac findings in eight patients, two of whom were female, with total lipodystrophy (Berardinelli-Seip's disease) are reported. One of them had the acquired form of the disease. Four patients died at a me an age of 32 years. As far as we know, at least three of them most lik ely died for cardiac reasons, one shortly after recovering from an att empted suicide. All eight patients had hypertrophic hearts, mostly wit h deranged diastolic, but also systolic, function. One had pulmonary h ypertension. We conclude that generalized lipodystrophy is a serious d isease with cardiac affection leading to cardiac dysfunction and early death. There is no specific cardiac treatment, and the treatment shou ld be according to the general guidelines for patients with hypertroph ic, dysfunctioning hearts.