Table 1 Baseline characteristics of the patients with Marfan syndrome

Female (%)

31 (56.4)

17 (54.8)

0.891

Age

35.07 ± 14.7

38.5 ± 13.7

0.295

FBN1 variant type (%)

0.296

 Missense

29 (53.7)

13 (41.9)

 Frameshift

13 (24.1)

7 (35)

 Nonsense

9 (16.7)

6 (19.4)

 Splice-site

2 (28.6)

5 (71.4)

 De novo (%)

17 (30.9)

8 (26.7)

0.682

Systemic score^a

8.02 ± 3.3

8.7 ± 3

0.401

EL (%)

25 (48.1)

21 (70)

0.149

AoRR (%)

0

22 (71)

n.a

 Valve sparing (%)

16 (72.7)

 Valve replacement (%)

6 (27.3)

MVP (%)

0.393

 Bulging

17 (52.8)

7 (23.3)

 Prolapse

8 (15.1)

8 (26.7)

MV surgery (%)

0

3 (9.3)

n.a

 Valvuloplasty and ring (%)

1

 Bioprosthesis (%)

1

 Mechanical valve (%)

1

Atrial arrhythmia (%)^b

0.016*

 Afib (%)

0

4 (12.9)

 Other SVT (%)

2 (3.6)

2 5 (6.4)

Symp. VE (%)^b

1

2

0.261

Treatment

0.206

 None (%)

16 (29.1)

3 (9.7)

 BB alone (%)

19 (34.5)

16 (51.6)

 ARB alone (%)

6 (10.9)

3 (9.7)

 ACEi alone (%)

1 (1.8)

0

 BB + ARB (%)

13 (23.6)

8 (25.8)

 BB + ACEi (%)

0

1 (3.2)

BMI ≥ 25 kg/m² (%)

13 (23.6)

7 (23.3)

0.365

Smoking (%)

0.979

 Never

38 (69.1)

22 (71)

 Ex-smoker

11 (20)

6 (19.4)

 Current

6 (10.9)

3 (9.7)

AHT (%)

6 (10.9)

2 (6.5)

0.494

Hyperlipidaemia (%)

5 (9.1)

3 (9.7)

0.844

Diabetes (%)

3 (5.5)

0

0.186