Echocardiographic assessment of cardiac function and morphology in patients with generalised lipodystrophy

Because cardiomegaly has been observed in lipodystrophic patients we studied cardiac morphology and function with one- and two-dimensional echocardiography in addition to general cardiologic examination in a series of seven patients. Muscular hypertrophy with increased chamber size and myocardial indentations were found. Two patients had asymmetrical septal hypertrophy (ASH), and two patients demonstrated systolic anterior movement (SAM) of the mitral valve. Wall motion analysis showed anomalities in four patients during contraction, in three during the early relaxation phase. Since pathological findings, probably increasing with age, were made in the majority of the patients, these findings add an additional unfavourable aspect to the syndrome.Abbreviations ASH asymmetrical septal hypertrophy - SAM systolic anterior movement - CGL congenital generalised lipodystrophy - ATL acquired total lipoatrophy - LA/Ao left atrial/aortic ratio - NRDD normalised rate of diameter decrease - NRID reduced rate of increased diameter - HOCM hypertrophic obstructive cardiomyopathy - VCF velocity of circumferencial fiber shortening