Table 3

Association of clinical features, cardiopulmonary exercise and CMR with MACE (ventricular tachycardia/heart failure/transplant/death) in unrepaired Ebstein’s anomaly patients during 3.5±2.6 years follow-up

Patient factors (n=79)Per*HR95% CIp Value†
Clinical features
 Age at inclusion↑1 year0.9640.908 to 1.0240.236
 Male gender0.4070.074 to 2.2280.300
 NYHA class>2 7.659 1.535 to 38.204 0.013
 Previous atrial tachyarrhythmia 11.155 1.299 to 95.813 0.028
 Atrial septal defect/patent foramen ovale1.2790.233 to 7.0040.777
 QRS durationms1.0160.980 to 1.0540.393
 QRS fractionation1.0000.063 to 15.9881.000
 Accessory pathway0.2360.043 to 1.2970.097
 Oxygen saturation at rest↓1%1.1290.891 to 1.4310.314
 Brain natriuretic peptide (n=50)↑1 pmol/L0.9810.841 to 1.1440.807
 Cardiothoracic ratio>652.1820.227 to 20.9850.499
Cardiopulmonary exercise capacity (n=50)
 Heart rate reserve↓1 bpm1.0330.971 to 1.1890.302
 Per cent predicted VO2 ↓1%1.0810.978 to 1.1930.128
 VE/VCO2 slope0.9530.837 to 1.0830.459
Right heart CMR measures 
 Functional right atrial indexed volume↑5 mL/m2 1.0090.983 to 1.0370.493
 Native right atrial indexed volume↑5 mL/m2 1.0080.979 to 1.0380.587
 Atrialised RV indexed volume↑5 mL/m2 1.0710.953 to 1.2030.252
 Tricuspid regurgitant fraction↑1%0.9980.949 to 1.0500.953
 Apical septal leaflet displacement↑1 mm1.0420.984 to 1.1040.160
 Apical septal leaflet displacement/LV septal length↑1%1.0400.989 to 1.0930.124
 Functional RV end diastolic volume index↑5 mL/m2 1.0210.989 to 1.0930.427
 Functional RV stroke volume index↓10 mL/m2 1.0180.845 to 1.2800.904
 Functional RV ejection fraction↓5% 2.058 1.168 to 3.623 0.012
Left heart CMR measures
 LV end diastolic volume index↑5 mL/m2 0.9250.744 to 1.1490.481
 LV stroke volume index↓10 mL/m2 2.817 1.121 to 7.092 0.028
 LV ejection fraction↓5% 2.347 1.348 to 4.082 0.003
 Cardiac index↓100 mL/min/m2 1.171 1.002 to 1.366 0.047
Combined right and left heart CMR measures
 Functional RV/LV end diastolic indexed volume ratio↑1 unit1.1780.645 to 2.1500.594
 Total right/left volume index↑1 unit1.0590.807 to 1.3900.678
 Severity index volume↑1 unit1.4020.388 to 5.0560.606
  • *Unit change in the parameter tested for hazard analysis is based on clinical relevance.

  • †p Values are derived from univariable Cox proportional hazard analysis.

  • CMR, cardiovascular magnetic resonance; LV, left ventricular; MACE, major adverse cardiovascular event; NYHA, New York Heart Association; RV, right ventricular.

  • Significant univariable predictors of MACE are formatted bold and italic.