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Table 2 Comparison between control subjects and patients with Marfan syndrome with and without valvular disease and/or cardiovascular surgery

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

  Control subjects Patients with Marfan syndrome p value p value
N = 40 No surgery Surgery Control versus MFS-1 MFS-1 versus MFS-2
No valvular disease Valvular disease
N = 55 (MFS-1) N = 31 (MFS-2)
Female (%) 22 (55) 31 (56.4) 17 (54.8) 0.895 0.891
Age (%) 37.9 ± 14.4 35.1 ± 14.7 38.5 ± 13.7 0.370 0.295
Height (cm) 173 ± 10 183.2 ± 11 183.9 ± 9.5  < 0.001 0.771
Weight (kg) 68.3 ± 9.2 74.2 ± 17.9 74.1 ± 18.3 0.041 0.987
BSA (m2) 1.8 ± 0.1 1.9 ± 0.2 1.9 ± 0.2 0.001 0.923
SBP (mmHg) 121.1 ± 12.7 121 ± 12.6 127.3 ± 15.9 0.899 0.049
DBP (mmHg) 73.7 ± 9.6 71.6 ± 70 67 ± 10.1 0.361 0.067
BB Use (%)a 0 (0) 32 (58.2) 25 (80.6) n.a 0.034
Ao sinus (mm) 30.5 ± 2.9 39.8 ± 5.1 41.1 ± 6.6b  < 0.001 0.498
z-score sinus  − 0.8 ± 0.7 2.5 ± 1.4 3.3 ± 1.1b  < 0.001 0.094
LVEDDi (mm/m2) 24.7 ± 2.4 24.8 ± 3.4 26.7 ± 3.5 0.795 0.020
LVEDDi ≥ 30 mm/m2 (%) 0 (0) 5 (9.1) 4 (12.9) 0.050 0.717
iLVM 86.9 ± 20.2 81 ± 34.7 86.2 ± 23.04 0.331 0.486
LVEF (%) 68.3 ± 7.2 66 ± 7.2 62.5 ± 7.6 0.131 0.034
LVEF < 55% 0 (0) 2 (3.6) 5 (16.1) 0.507 0.093
mw-FS (%) 21 ± 7 21 ± 5 16 ± 6 0.778 0.004
RWT 0.45 (0.40–0.50) 0.37 (0.32–0.45) 0.33 (0.29–0.40)  < 0.001 0.060
E/A 1.5 (1.2–2) 1.6 (1.3–2) 1.6 (1.2–2.1) 0.351 0.975
E/E’ 6.4 (5.1–7.6) 7.8 (6.6–9.3) 8.6 (7–12.8)  < 0.001 0.051
LAVi (ml/m2) 21.2 ± 5.5 21.4 ± 1.3 26.4 ± 11.3 0.945 0.038
MVP (%)c 1 (2.5) 8 (14.5) 8 (26.7) 0.045 0.185
TAPSE (mm) 24.5 ± 2.8 22.1 ± 4.3 19.5 ± 3.8 0.003 0.010
TAPSE ≤ 16 mm (%) 0 (0) 3 (6.4) 6 (20.7) 0.250 0.077
NT-ProBNP (pg/ml) 30 (19–44.5) 53.5 (30–74.2) 129 (82–235.2) 0.001  < 0.001
Min HR (bpm) 50.5 (44–55.5) 45 (41.5–49) 48 (44–55) 0.004 0.026
Average HR (bpm) 74.2 ± 8.3 64 (56.5–71.5) 67 (58.5–73)  < 0.001 0.205
QRS width (ms) 80 (80–90) 96 (86–104) 98 (86–106)  < 0.001 0.639
QTc (ms) 380.2 ± 24.5 414 (388–433.5) 426 (407–445)  < 0.001 0.028
QTc > 460 ms (%) 0 (0) 0 (0) 4 (12.9) n.a n.a
SVES/24 h 2 (0.25–4.7) 7 (2–37.5) 17 (1–56)  < 0.001 0.560
Atrial runs (%) 2 (5) 14 (26.4) 12 (41.4) 0.015 0.318
VES/24 h 0 (0–5.7) 6 (1–69.5) 14 (1.5–373.5)  < 0.001 0.312
VE (%) 5 (12.5) 14 (26.4) 13 (41.9) 0.099 0.090
Vent couplets (%) 2 (5) 9 (17) 8 (25.8) 0.077 0.270
NSVT (%) 0 (0) 5 (9.1) 5 (17.2) 0.050 0.273
SDNN (ms) 147 (116–185.2) 185 (156.2–219.2) 132 (95.4–191) 0.001 0.003
RMSDD (ms) 53 (36.2–84) 82.5 (66.2–82.5) 59.3 (42.7–112)  < 0.001 0.040
  1. Values are given as mean ± SD, median (IQR) or number (%)
  2. Ao aortic, BB beta-blocker, BSA body surface area, DBP diastolic blood pressure, Dec time deceleration time, HR heart rate, LAVi left atrium volume index, LVEDDi left ventricular end diastolic diameter index, LVEF left ventricular ejection fraction, MFS Marfan syndrome, MVP mitral valve prolapse, mw-FS mid-wall fractional shortening, NSVT non-sustained ventricular, RMSDD mean squared difference of successive NN intervals, RWT relative wall thickness, SBP systolic blood pressure, SDNN standard deviation of the NN interval, SVES supraventricular extrasystoles, VES ventricular extrasystoles, TAPSE tricuspid annular plane systolic excursion, VE ventricular ectopy
  3. aBeta-blocker alone or in combination
  4. bOnly those patients with valvular pathology without aortic root replacement are considered for the mean value of the sinus and the z-score
  5. cOnly those patients with true mitral valve prolapse considered here. Those with mitral valve bulging were not included in this calculation