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Table 1 Baseline characteristics of the patients with Marfan syndrome

From: Myocardial disease and ventricular arrhythmia in Marfan syndrome: a prospective study

Parameter MFS-1 (N = 55) MFS_2 (N = 31) p value
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 scorea 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
  1. ACEi angiotensin converting enzyme inhibitor, Afib atrial fibrillation, AoRR aortic root replacement, ARB angiotensin II receptor blocker antagonist, BB beta-blocker, BMI body mass index, EL ectopia lentis, HTA arterial hypertension, MV mitral valve, MVP mitral valve prolapse, SVT supraventricular tachycardia, VE ventricular ectopy
  2. aMarfan systemic score is a scoring system which takes into consideration several characteristic features of MFS and assigns each of them a value between 1 and 3, 3 being the most specific for the disease. A score of ≥ 7 is considered abnormal and in combination with aortic disease and/or ectopia lentis is diagnostic of MFS
  3. bPatients reporting palpitations in the past were not included. Only confirmed atrial and ventricular ectopy for which treatment was implemented were considered