Introduction
Methods
Study population and inclusion criteria
Statistical analysis
Results
Comparison between patients with Fontan circulation and patients with rTOF
Fontan circulation (n = 189) | rTOF (n = 36) | p-value | |
---|---|---|---|
Male | 121 (64%) | 17 (47%) | 0.06 |
Median age at CMR, yrs | 16.1 (11.0, 23.2) | 15.7 (11.1, 23.7) | 0.86 |
Mean BSA at CMR, m2 | 1.49 ± 0.44 | 1.49 ± 0.39 | 1.00 |
Thoracic duct visualization | < 0.001 | ||
Complete/Nearly complete | 122 (65%) | 8 (22%) | |
Incomplete/Not visualized | 67 (35%) | 28 (79%) | |
Maximum thoracic duct diameter, mm | 2.50 (2.10, 3.10) | 1.95 (1.70, 2.60) | 0.002 |
Thoracic duct tortuosity | 0.17 | ||
None/Mild | 119 (63%) | 17 (47%) | |
Moderate/Severe | 50 (27%) | 3 (8%) | |
3D sequence with artifact or of poor quality | 17 (9%) | 4 (11%) | 0.75 |
3D sequence with limited field of view | 6 (3%) | 0 (0%) | 0.59 |
Variable | None/mild (n = 119) | Moderate/severe (n = 50) | p-value |
---|---|---|---|
Male | 78 (66%) | 33 (66%) | 1.00 |
Primary cardiac diagnosis | 0.05 | ||
Tricuspid atresia | 24 (20%) | 6 (12%) | |
HLHS | 39 (33%) | 18 (36%) | |
DILV | 10 (8%) | 6 (12%) | |
DORV | 25 (21%) | 3 (6%) | |
Unbalanced CAVC | 6 (5%) | 4 (8%) | |
Small right heart | 6 (5%) | 3 (6%) | |
Other | 9 (8%) | 10 (20%) | |
Heterotaxy syndrome | 1.00 | ||
Yes | 22 (19%) | 9 (18%) | |
No | 97 (82%) | 41 (82%) | |
Median age at Fontan, years | 2.7 (2.2, 3.6) | 3.0 (2.3, 3.7) | 0.23 |
Fontan type | 0.04 | ||
Lateral tunnel | 86 (72%) | 26 (52%) | |
Extracardiac | 25 (21%) | 17 (34%) | |
RA-PA | 7 (6%) | 5 (10%) | |
Other | 1 (1%) | 2 (4%) | |
Fontan revision | 1.00 | ||
Yes | 5 (4%) | 2 (4%) | |
No | 114 (96%) | 48 (96%) | |
Ventricular morphology | 0.08 | ||
Right | 57 (50%) | 20 (42%) | |
Left | 44 (38%) | 15 (31%) | |
Both | 14 (12%) | 13 (27%) | |
Median age at MRI, years | 17.1 (12.3, 24.5) | 15.2 (10.4, 22.6) | 0.27 |
BSA at MRI | 1.54 ± 0.42 | 1.46 ± 0.49 | 0.32 |
Clinical variables and thoracic duct characteristics in patients with Fontan circulation
Clinical event | Event | No event | p-value |
---|---|---|---|
Plastic bronchitis, n | 2 | 187 | |
Age at CMR, years | 7.1 ± 1.4 | 18.5 ± 10.0 | 0.11 |
Maximum thoracic duct diameter, mm | 2.8 (0.7) | 2.8 (0.1) | 0.95 |
Tortuosity | 0.51 | ||
None/mild | 1 (50%) | 118 (71%) | |
Moderate/severe | 1 (50%) | 49 (29%) | |
PLE, n | 4 | 185 | |
Age at CMR, years | 12.9 ± 3.9 | 18.5 ± 10.1 | 0.27 |
Maximum thoracic duct diameter, mm | 4.1 (0.5) | 2.7 (0.1) | 0.005 |
Tortuosity | 0.08 | ||
None/mild | 1 (25%) | 118 (72%) | |
Moderate/severe | 3 (75%) | 47 (29%) | |
NYHA class ≥ II, n | 21 | 168 | |
Age at CMR, years | 21.1 ± 11.7 | 18.0 ± 9.8 | 0.19 |
Maximum thoracic duct diameter, mm | 2.9 (0.2) | 2.7 (0.1) | 0.50 |
Tortuosity | 0.02 | ||
None/mild | 9 (45%) | 110 (74%) | |
Moderate/severe | 11 (55%) | 39 (26%) | |
Major thrombotic event, n | 17 | 166 | |
Age at CMR, years | 23.4 ± 17.0 | 17.9 ± 9.0 | 0.20 |
Maximum thoracic duct diameter, mm | 2.4 (0.3) | 2.8 (0.01) | 0.13 |
Tortuosity | 1.00 | ||
None/mild | 11 (73%) | 102 (69%) | |
Moderate/severe | 4 (27%) | 46 (31%) | |
Arrhythmia history, n | 52 | 137 | |
Age at CMR, years | 23.6 ± 12.8 | 16.3 ± 8.0 | < 0.001 |
Maximum thoracic duct diameter, mm | 2.5 (0.2) | 2.8 (0.1) | 0.12 |
Tortuosity | 0.35 | ||
None/mild | 35 (76%) | 84 (68%) | |
Moderate/severe | 11 (24%) | 39 (32%) |
Variable | None/mild (n = 119) | Moderate /severe (n = 50) | p-value | ||
---|---|---|---|---|---|
n | Value | n | Value | ||
CMR | |||||
EDV/BSA, ml/m2 | 86 | 116 ± 33.9 | 35 | 132.3 ± 45.7 | 0.06 |
ESV/BSA, ml/m2 | 84 | 57.3 ± 26.0 | 32 | 70.0 ± 29.9 | 0.03 |
Mass/BSA, g/m2 | 77 | 64.5 ± 22.7 | 28 | 75.6 ± 37.0 | 0.15 |
EF, % | 84 | 52.4 ± 8.6 | 32 | 49.2 ± 8.4 | 0.09 |
APC, % | 20 | 16.8 ± 9.6 | 8 | 21.0 ± 9.5 | 0.31 |
Moderate or worse AVVR (echo) | 85 | 9 (11%) | 39 | 7 (18%) | 0.26 |
Cath | |||||
Mean Fontan pressure, mmHg | 27 | 15.0 ± 3.0 | 18 | 16.5 ± 3.6 | 0.14 |
PVR, WUm2 | 26 | 2.16 ± 0.77 | 17 | 1.72 ± 0.83 | 0.09 |
Max EDP, mmHg | 26 | 8.96 ± 2.60 | 17 | 10.53 ± 2.65 | 0.06 |
CPET | |||||
Peak RER | 34 | 1.20 ± 0.09 | 11 | 1.18 ± 0.09 | 0.42 |
Peak VO2, mL × kg−1 × min−1 | 34 | 24.1 ± 7.2 | 11 | 20.8 ± 7.2 | 0.19 |
% predicted VO2 max | 34 | 62.4 ± 14.0 | 11 | 58.6 ± 16.0 | 0.46 |
% predicted peak O2 pulse | 34 | 76.5 ± 20.9 | 11 | 72.0 ± 22.1 | 0.54 |
% predicted peak work rate | 33 | 71.5 ± 17.8 | 11 | 65.2 ± 23.3 | 0.35 |
Relative VO2 at VAT mL × kg−1 × min−1 | 34 | 14.3 ± 3.9 | 11 | 13.7 ± 4.5 | 0.69 |
%VAT/Predicted peak VO2 | 34 | 37.3 ± 7.9 | 11 | 39.3 ± 12.6 | 0.63 |
VE/VCO2 slope | 34 | 35.4 ± 7.5 | 11 | 30.9 ± 7.4 | 0.09 |
LAB | |||||
Creatinine, mg/dL | 52 | 0.70 ± 0.20 | 35 | 0.61 ± 0.22 | 0.04 |
Albumin, mg/dL | 49 | 4.4 ± 0.6 | 33 | 4.4 ± 0.5 | 0.71 |
Absolute Lymphocyte Count, K cells/µL | 13 | 0.76 ± 0.36 | 9 | 1.80 ± 0.78 | 0.003 |
Variable | Mean ± SD | Partial correlation | ||
---|---|---|---|---|
n | R | p-value | ||
CMR | ||||
Years since initial Fontan | 14.6 ± 8.8 | 171 | − 0.08 | 0.30 |
EDV/BSA, ml/m2 | 120.9 ± 37.4 | 122 | 0.06 | 0.51 |
ESV/BSA, ml/m2 | 60.9 ± 27.1 | 117 | 0.13 | 0.16 |
Mass/BSA, g/m2 | 67.4 ± 27.3 | 106 | 0.02 | 0.81 |
EF, %* | 51.4 ± 8.4 | 117 | − 0.22 | 0.02 |
APC, % | 18.5 ± 9.8 | 29 | − 0.19 | 0.32 |
Cath | ||||
Mean Fontan pressure, mmHg | 15.6 ± 3.3 | 46 | 0.16 | 0.30 |
PVR, WUm2 | 2.0 ± 0.8 | 44 | − 0.16 | 0.29 |
Max EDP, mmHg | 9.6 ± 3.0 | 44 | 0.28 | 0.07 |
CPET | ||||
Peak VO2, mL × kg−1 × min−1 | 24.2 ± 8.1 | 45 | − 0.20 | 0.20 |
% predicted VO2 max | 63.4 ± 16.0 | 45 | − 0.08 | 0.61 |
Peak RER | 1.2 ± 0.1 | 45 | − 0.01 | 0.96 |
% predicted peak O2 pulse | 77.2 ± 22.5 | 45 | 0.10 | 0.52 |
% predicted peak work rate | 71.7 ± 20.5 | 44 | − 0.17 | 0.28 |
Relative VO2 at VAT mL × kg−1 × min−1 | 14.5 ± 4.2 | 45 | − 0.10 | 0.50 |
%VAT/Predicted peak VO2 | 38.4 ± 9.5 | 45 | 0.03 | 0.85 |
VE/VCO2 slope | 34.4 ± 7.4 | 45 | − 0.27 | 0.07 |
Laboratory | ||||
Creatinine, mg/dL | 0.7 ± 0.2 | 88 | − 0.03 | 0.79 |
Albumin, mg/dL | 4.4 ± 0.6 | 83 | 0.09 | 0.44 |
Absolute Lymphocyte Count, K cells/μL | 1.2 ± 0.8 | 22 | − 0.27 | 0.24 |
Interobserver | Intraobserver | |||
---|---|---|---|---|
Percent agreement | Kappa (95% CI) | Percent agreement | Kappa (95% CI) | |
Thoracic duct visualization | 88% | 0.76 (0.58, 0.94) | 86% | 0.72 (0.53, 0.91) |
Thoracic duct tortuosity | 76% | 0.67 (0.51, 0.84) | 76% | 0.68 (0.50, 0.86) |
Mean difference ± SD | ICC (95% CI) | Mean difference ± SD | ICC (95% CI) | |
Thoracic duct diameter | 0.37 ± 0.65 | 0.78 (0.74, 0.81) | 0.41 ± 0.88 | 0.64 (0.57, 0.70) |