234 Evaluation of left ventricular function in patients with Marfan syndrome using conventional Doppler-echocardiography, tissue Doppler and two-dimensional strain imaging

BackgroundEarly impairment of left ventricular systolic and diastolic function in Marfan syndrome (MFS) has been suggested but remains uncertain.Methods179 patients were consecutively screened for MFS. Diagnosis was confirmed in 92, ruled out in 35 and uncertain in 52. Clinical and echocardiographic characteristics of 35 Healthy and 79 MFS patients were compared. Patients with previous surgery or ongoing pregnancy were excluded.ResultsGroups were comparable for age and gender (mean age 31±14, male,45%). Overall MFS compared with healthy patients had larger aortic dimensions (Valsalva 41±7 vs 36±4mm, p<0.001), left ventricular volumes (end diastolic (EDV) 118±33 vs 86±26mL, p<0.01 and end systolic (ESV) 43±15 vs 30±9mL, p<0.01), diastolic dimensions (51±1 vs 48±1, p=0.04) and tended to have larger LV systolic dimensions(p=0.06). Adjustment for BSA lessened differences between groups but EDVI and ESVI remained significantly larger in MFS than healthy patients (both p<0.01). No difference in fractional shortening, Teicholz and Simpson ejection fraction was observed between groups (for all p=NS). Assessment of diastolic function with E/A and deceleration time showed no difference between groups (for all p=NS). However MFS compared with controls had lower early diastolic (Ea: 9.7±3 vs 14.5±4cm/s, p<0.001) and systolic velocities (Sm:8.5±2 vs10.2±3cm/s, p=0.003). Despite lower velocities, global and regional longitudinal systolic function (P=NS for all) quantified by 2D strain was not different between groups.These cardiac abnormalities observed in MFS increased with the severity of the aortic root dilatation (table).ConclusionIn comparison with controls MFS patients have apparent similar systolic and diastolic LV function. However comprehensive echocardiographic evaluation using most of the available current tools shows mild impairment of LV function, which seems related to the degree of aortic root involvement.VariablesControlsMFS with Valsalva≤40mmMFS with Valsalva>40mmP valueSm cm/s10±39±28±2P=0.002Ea cm/s15±411±49±2P<0.001EDVI mL/m250±1253±1269±16P