Introduction
Materials and methods
Study population
CMR protocol
Biventricular and biatrial morphology and function quantification
LV and RV global strain analysis
Quantification of chest wall deformity
Subgroup analysis of patients with chest wall deformity
Statistical analysis
Results
Demographics of MFS patients compared to controls
Controls (n = 20) | All patients (n = 40) | p value | |
---|---|---|---|
Demographics | |||
Age, years | 16 ± 4 | 16 ± 3 | 0.77 |
Male, % | 12 | 23 | > 0.99 |
Weight, kg | 61 ± 15 | 62 ± 20 | 0.90 |
Height, m | 1.70 ± 0.12 | 1.79 ± 0.16 | 0.03 |
BMI, kg/m2 | 21 ± 3 | 19 ± 4 | < 0.05 |
BSA, m2 | 1.70 ± 0.26 | 1.77 ± 0.35 | 0.44 |
CMR parameters | |||
Heart rate at CMR, bpm | 73 ± 14 | 71 ± 11 | 0.50 |
LVEF, % | 62 ± 5 | 61 ± 5 | 0.66 |
LV mass, g/m2 | 54 ± 10 | 55 ± 12 | 0.85 |
LVEDV, ml/m2 | 82 ± 12 | 86 ± 19 | 0.38 |
LVESV, ml/m2 | 31 ± 8 | 33 ± 8 | 0.56 |
LVSV, ml/m2 | 50 ± 7 | 51 ± 11 | 0.62 |
LAEDV, ml/m2 | 11 ± 3 | 14 ± 7 | 0.05 |
LAESV, ml/m2 | 29 ± 8 | 28 ± 11 | 0.65 |
RVEF, % | 62 ± 6 | 55 ± 10 | < 0.01 |
RVEDV, ml/m2 | 81 ± 10 | 75 ± 17 | 0.08 |
RVESV, ml/m2 | 32 ± 7 | 34 ± 10 | 0.35 |
RVSV, ml/m2 | 50 ± 5 | 41 ± 12 | < 0.001 |
RAEDV, ml/m2 | 20 ± 8 | 19 ± 7 | 0.42 |
RAESV, ml/m2 | 34 ± 9 | 33 ± 10 | 0.68 |
Chest wall dimensions | |||
A-Haller, mm | 229 ± 17 | 227 ± 23 | 0.81 |
C-Haller, mm | 89 ± 13 | 77 ± 25 | 0.02 |
Haller index | 2.6 ± 0.3 | 3.4 ± 1.5 | < 0.01 |
Global LV myocardial strain | |||
LV GLS, % | − 20 ± 3 | − 17 ± 3 | < 0.01 |
LV GCS, % | − 22 ± 3 | − 19 ± 3 | < 0.0001 |
LV GRS, % | 37 ± 8 | 35 ± 7 | 0.31 |
Global RV myocardial strain | |||
RV GLS, % | − 23 ± 3 | − 21 ± 3 | < 0.05 |
RV GCS, % | − 12 ± 3 | − 15 ± 3 | < 0.01 |
Cardiac morphology and function of MFS patients compared to controls
Global myocardial strain in MFS patients compared to controls
CMR characteristics of MFS patients with chest wall deformity
Haller index < 3.25 (n = 23) | p value | Haller index ≥ 3.25 (n = 17) | p value | Controls (n = 20) | |
---|---|---|---|---|---|
Demographics | |||||
Age, years | 17 ± 3 | 0.04 | 14 ± 3 | 0.23 | 16 ± 4 |
Male, % | 14 | 0.75 | 9 | 0.75 | 12 |
Weight, kg | 70 ± 20 | < 0.01 | 51 ± 15 | 0.05 | 61 ± 15 |
Height, m | 1.83 ± 0.16 | 0.11 | 1.74 ± 0.16 | 0.35 | 1.70 ± 0.12 |
BMI, kg/m2 | 21 ± 4 | < 0.001 | 16 ± 3 | < 0.0001 | 21 ± 3 |
BSA, m2 | 1.89 ± 0.33 | < 0.01 | 1.61 ± 0.30 | 0.32 | 1.70 ± 0.26 |
CMR parameters | |||||
HR at CMR, bpm | 71 ± 13 | 0.89 | 71 ± 7 | 0.51 | 73 ± 14 |
LVEF, % | 61 ± 5 | 0.98 | 61 ± 5 | 0.71 | 62 ± 5 |
LV mass, g/m2 | 55 ± 11 | 0.90 | 54 ± 14 | 0.94 | 54 ± 10 |
LVEDV, ml/m2 | 87 ± 17 | 0.76 | 85 ± 22 | 0.60 | 82 ± 12 |
LVESV, ml/m2 | 34 ± 8 | 0.38 | 31 ± 7 | 0.97 | 31 ± 8 |
LVSV, ml/m2 | 53 ± 12 | 0.23 | 49 ± 10 | 0.60 | 50 ± 7 |
LAEDV, ml/m2 | 16 ± 7 | 0.07 | 12 ± 6 | 0.64 | 11 ± 3 |
LAESV, ml/m2 | 31 ± 11 | 0.01 | 22 ± 8 | 0.02 | 29 ± 8 |
RVEF, % | 58 ± 8 | 0.01 | 50 ± 11 | < 0.001 | 62 ± 6 |
RVEDV, ml/m2 | 75 ± 16 | 0.93 | 75 ± 19 | 0.23 | 81 ± 10 |
RVESV, ml/m2 | 32 ± 8 | 0.09 | 37 ± 12 | 0.09 | 32 ± 7 |
RVSV, ml/m2 | 44 ± 11 | 0.08 | 37 ± 12 | < 0.001 | 50 ± 5 |
RAEDV, ml/m2 | 20 ± 7 | 0.26 | 17 ± 6 | 0.21 | 20 ± 8 |
RAESV, ml/m2 | 36 ± 10 | 0.06 | 29 ± 10 | 0.14 | 34 ± 9 |
Chest wall dimensions | |||||
A-Haller, mm | 226 ± 20 | 0.71 | 229 ± 28 | > 0.99 | 229 ± 17 |
C-Haller, mm | 94 ± 15 | < 0.0001 | 54 ± 15 | < 0.0001 | 89 ± 13 |
Haller index | 2.5 ± 0.4 | < 0.0001 | 4.6 ± 1.7 | < 0.001 | 2.6 ± 0.3 |
Global LV myocardial strain | |||||
LV GLS, % | − 18 ± 3 | 0.03 | − 16 ± 2 | < 0.001 | − 20 ± 3 |
LV GCS, % | − 19 ± 2 | 0.21 | − 18 ± 4 | < 0.001 | − 22 ± 3 |
LV GRS, % | 35 ± 8 | 0.50 | 34 ± 7 | 0.22 | 37 ± 8 |
Global RV myocardial strain | |||||
RV GLS, % | − 22 ± 3 | 0.45 | − 21 ± 2 | < 0.05 | − 23 ± 3 |
RV GCS, % | − 15 ± 4 | 0.73 | − 14 ± 3 | 0.02 | − 12 ± 3 |