Table 2.
Cardiovascular Status: Cardiac Magnetic Resonance, Exercise Stress Test and Electrocardiogram
| CARDIAC MAGNETIC RESONANCE | Total N=148 |
ND* N=108 |
22q11.2DS† N=26 |
P-value |
|---|---|---|---|---|
| RV ejection fraction (%)‡ | 60±8 | 60±8 | 60±10 | 0.98 |
| Pulmonary regurgitant fraction (%) | 34±17 | 35±16 | 28±19 | 0.11 |
| RV end-diastolic volume (mL/m2) | 114±39 | 117±40 | 104±35 | 0.04§ |
| RV end-diastolic volume (mL/m2) adjusted | 113(87; 144) | 115 (90; 149) | 106 (82; 138) | 0.40 |
| RV end-diastolic volume Z score | 3.1 ± 2.9 | 3.4 ± 2.8 | 1.8 ± 2.8 | 0.0093* |
| RV end-systolic volume (mL/m2) | 48±20 | 49±19 | 43±23 | 0.062 |
| RV cardiac index (L/min/m2) | 5.5±1.5 | 5.7±1.4 | 5.1±1.5 | 0.043 |
| RV stroke volume (mL/m2) | 69.7±19 | 72±18.5 | 61±18 | 0.01 |
| Indexed RV mass (grams/m2) | 76±23 | 77 ±24 | 68±21 | 0.10 |
| LV ejection fraction (%)∥ | 69±7 | 70 ±7 | 66±8 | 0.05 |
| LV cardiac output (L/min/m2) | 3.7±0.7 | 3.7±0.7 | 3.6±0.8 | 0.61 |
| EXERCISE STRESS TEST | N=156 | N=129 | N=27 | |
| Maximal effort (RER≥1.1)# | 94 (63) | 84 (68) | 10 (40) | 0.012 |
| Forced vital capacity (Liters) | 2.3±0.9 | 2.41±0.9 | 1.5±0.6 | <0.0001 |
| % Predicted forced vital capacity | 77±16 | 81± 14 | 62±16 | <0.0001 |
| Cardiovascular Response at Peak Exercise | ||||
| mVO2 (mL/kg/min)** | 32±8 | 33±8 | 24±7 | <0.0001 |
| Predicted mVO2 (%) | 76±18 | 80±17 | 61±17 | <0.0001 |
| Maximum work (watts) | 112 ± 48 | 117 ± 49 | 83 ± 28 | 0.0041 |
| % Predicted maximum work | 83 ± 23 | 86 ± 22 | 64 ± 18 | 0.0002 |
| Oxygen pulse (ml oxygen/beat) | 7.4 ± 2.9 | 7.8 ± 2.9 | 5.4 ± 2.2 | <0.0001 |
| Oxygen pulse/m2 (ml oxygen/beat/m2) | 5.5 ± 1.4 | 5.8 ± 1.3 | 4.4 ± 1.2 | <0.0001 |
| Breathing reserve (%) | 16 ±13 | 15 ± 13 | 19 ± 10 | 0.12 |
| Maximum heart rate (beats per minute) | 180 ± 15 | 182 ± 14 | 175 ± 17 | 0.08 |
| Anaerobic Threshold Measurements | ||||
| VO2 at anaerobic threshold (mL/kg/min)** | 21 ± 5 | 21 ± 5 | 19 ± 6 | 0.036 |
| Predicted VO2 at anaerobic threshold (%) | 86 ± 18 | 87 ± 17 | 80 ± 21 | 0.085 |
| ELECTROCARDIOGRAM | N=155 | N=129 | N=26 | |
| PR interval | 137 ± 25 | 135 ± 24 | 147 ± 25 | 0.04 |
| QRS duration | 126 ± 25 | 125 ± 25 | 130 ± 24 | 0.44 |
| QTc interval†† | 446 ± 26 | 446 ± 27 | 450 ± 25 | 0.47 |
| Right bundle branch block | 106(84) | 88(85) | 18(75) | 0.037 |
Data are expressed as mean (± standard deviation), or number (percentage).
*
ND = non-deleted
†
22q11.2DS = 22q11.2 deletion syndrome
‡
RV = right ventricle
§
RV end-diastolic volume adjusted for reoperations to limit pulmonary insufficiency (RV to pulmonary artery conduit or pulmonary valve replacement).
∥
LV = left ventricle
#
RER = respiratory exchange ratio
**
mVO2 = maximum oxygen consumption. VO2 indicates oxygen consumption
††
QTc = QT interval corrected for heart rate