A comprehensive echocardiogram study in elher danlos syndrome

Background and Purpose: Ehlers-Danlos syndrome (EDS) is a group of inherited disorders marked by extremely loose joints, hyperelastic skin that bruises easily, and easily damaged blood vessels. The aim of this study is to determine the echocardiographic findings in Ehlers-Danlos syndrome (EDS) patients. Methods: 139 EDS patients were selected randomly from our clinic and underwent echocardiogram. Echocardiogram results were reviewed comprehensively in all the patients and performed cumulative distribution of results in top 10 percentile to see the abnormalities. Results: Out of 139 patients (n=139), 96% are females (n=133; age 31.88±11.36), 4% are males (n=6; age23.83±9.19). Our findings are as follows (mean ± standard deviation) RVDD (2.27 cm ± 0.20 cm), LVED (4.45 cm ± 0.43 cm), IVS (0.79 cm ± 0.11 cm), LVEF (61.9% ± 2.48%), LVES (2.93 cm ± 0.25 cm), LVPW (0.82 cm ± 0.12 cm), LVOT Diam (1.82 cm ± 0.21 cm) AO Root (2.70 cm ± 0.33 cm), LA Diam (3.17 cm ± 0.33 cm), AV Velocity (1.11 m/sec ± 0.14 m/sec), LVOT Vel (0.82 m/sec ± 0.31 m/sec), Mean Gradient (2.92mmHg ± 0.68 mmHg), Peek Gradient (4.81 mmHg ± 1.40 mmHg), AVA (2.85 cm2 ± 0.34 cm2), E Velocity (0.77 m/sec ± 0.17 m/sec), A Velocity (0.64 m/sec ± 0.10 m/sec), E/A Ratio (1.42 ± 0.34), Mean Gradient (0.81 mmHg ± 0.27 mmHg), Peak Gradient (2.22 mmHg ± 0.81 mmHg), PHT (48.21 m/sec ± 11.16 m/sec), MVDT (157.40 m/sec ± 30.33 m/sec), MVA (4.72 cm2 ± 1.16 cm2), Mital Regurg Mild in 87% patients TV Velocity (0.69 m/ sec ± 0.16 m/sec), RAP (est.) (10 mmHg ± 0 mmHg), TR Velocity (20.88 m/sec ± 6.62 m/ sec), RSVP (est.) (29.08 ± 6.54), TR Mild in 83% Patients. PV Vel (0.92 m/sec ± 0.23 m/ sec). Top 10 percentile parameters are follows RVDD 2.53 cm, LVED 5.00 cm, IVS 0.93 cm, LVEF 65.08%, LVES 3.25 cm, LVPW 0.96 cm, LVOT Diam 2.09 cm, Ao Root 3.12 cm, LA Diam 3.59 cm, AV Velocity 1.29 m/sec, LVOT Vel 1.22 m/sec, Mean Gradient 3.79 mmHg, Peek Gradient 6.60 mmHG, AVA 3.29 cm2, E Velocity 0.99 m/sec, A Velocity 0.77 m/sec, E/A Ratio 1.86, Mean Gradient 1.16 mmHg, Peak Gradient 3.25 mmHg, PHT 62.51 m/sec, MVDT 207.80 m/sec, MVA 6.21 cm2, TV Velocity 0.90 m/sec, RAP (est) 10mmHg, TR Velocity 29.36 m/sec, RSVP (est.) 37.46, PV Vel 1.21 m/sec. Conclusion: Our study attempts to define Echo characteristics of a large cohort of patients with Ehlers Danlos Syndrome. In this subgroup no ventricular or atrial enlargement is seen. There is no increased prevalence of mitral valve prolapse seen. The increased LVOT and aortic velocities may be related with hyperadrenergic state that happens in these patients. Diastolic parameters are preserved.