TABLE 2.
Fontan Cross-Sectional Study Patient Characteristics by Age at Enrollment
| Overall | <9 years | 9 to <11 years |
11 to <15 years |
≥15 years | |||
|---|---|---|---|---|---|---|---|
| Characteristic | N | Mean ± SD Median or % |
Mean ± SD Median or % |
Mean ± SD Median or % |
Mean ± SD Median or % |
Mean ± SD Median or % |
P-value |
| N | 546 | 138(25%) | 120(22%) | 169(31%) | 119(22%) | ||
| Age at enrollment, yr | 11.9±3.4 | 7.9±0.7 | 10.0±0.6 | 12.8±1.2 | 17.0±1.1 | ||
| Age at Fontan, yr | 3.4±2.1 | 3.0±1.3 | 3.0±1.5 | 3.2±1.9 | 4.6±3.0 | <.001 | |
| Age at volume unloading surgery, yr | 1.6±1.6 | 0.9±0.9 | 1.1±1.0 | 1.4±1.0 | 3.4±2.1 | <.001 | |
| Male | 546 | 60% | 58% | 62% | 65% | 55% | .305 |
| Race | 544 | .014 | |||||
| White | 80% | 70% | 80% | 82% | 87% | ||
| Black | 10% | 14% | 7% | 11% | 8% | ||
| Asian | 3% | 4% | 3% | 2% | <1% | ||
| Other | 8% | 13% | 10% | 4% | 3% | ||
| Hispanic | 517 | 7% | 8% | 10% | 4% | 6% | .318 |
| Growth | |||||||
| Percentile for stature-for-age | 544 | 26 | 22 | 31 | 22 | 31 | 0.242* |
| Z-score for stature-for-age | 544 | −0.7±1.3 | −0.7±1.4 | −0.6±1.3 | −0.8±1.3 | −0.6±1.0 | 0.316 |
| Percentile for weight-for-age | 545 | 37 | 36 | 27 | 29 | 48 | 0.021* |
| Z-score for weight-for-age | 545 | −0.4±1.4 | −0.4±1.2 | −0.5±1.4 | −0.6±1.5 | −0.2±1.2 | 0.085 |
| Body mass index z-score | 544 | −0.05±1.13 | 0.07±0.97 | −0.23±1.25 | −0.15±1.24 | 0.13±0.99 | 0.030 |
| Fontan Type | 513 | <.001 | |||||
| Atriopulmonary connection | 13% | <1% | 3% | 15% | 36% | ||
| TCPC intracardiac lateral tunnel | 59% | 52% | 62% | 70% | 50% | ||
| TCPC extracardiac lateral tunnel | 13% | 26% | 19% | 4% | 3% | ||
| TCPC extracardiac conduit | 13% | 21% | 15% | 9% | 6% | ||
| Other | 2% | 0% | <1% | 2% | 5% | ||
| Ventricular type | 546 | 0.023 | |||||
| Left Ventricular | 49% | 46% | 38% | 53% | 57% | ||
| Right Ventricular | 34% | 36% | 41% | 34% | 24% | ||
| Mixed | 18% | 19% | 22% | 13% | 19% | ||
| Stage II surgery performed | 546 | 75% | 93% | 88% | 78% | 35% | <.001 |
| Predominant rhythm | 518 | 0.255 | |||||
| Normal sinus rhythm | 67% | 63% | 69% | 71% | 63% | ||
| Atrial escape | 9% | 11% | 10% | 7% | 9% | ||
| Junctional escape | 6% | 9% | 6% | 5% | 3% | ||
| Paced | 8% | 10% | 4% | 9% | 11% | ||
| Other | 11% | 8% | 12% | 9% | 15% | ||
| Currently on pacemaker | 546 | 13% | 12% | 12% | 11% | 20% | 0.087 |
| Serology | |||||||
| Brain natriuretic peptide, pg/mL | 510 | 13 (7, 26) |
11 | 11 | 14 | 17 | 0.020* |
| Echocardiographic Measures | |||||||
| Heart rate z-score | 437 | −0.20±0.98 | −0.27±0.95 | −0.05±0.93 | −0.20±0.98 | −0.27±1.04 | 0.318 |
| End-diastolic volume z-score | 414 | −0.7±1.9 | −0.5±1.8 | −0.6±1.9 | −1.0±1.5 | −0.4±2.5 | 0.134 |
| End-systolic volume z-score | 414 | 0.2±2.4 | 0.4±2.1 | 0.2±2.4 | −0.1±2.1 | 0.6±3.3 | 0.206 |
| Ejection fraction z-score | 414 | −0.9±2.0 | −0.8±2.0 | −0.6±1.9 | −0.9±2.1 | −1.2±2.1 | 0.265 |
| Stroke volume z-score | 414 | −1.1±1.8 | −0.9±1.9 | −1.0±1.7 | −1.3±1.5 | −1.0±2.2 | 0.246 |
| Mass z-score | 406 | 1.0±2.3 | 0.7±2.0 | 0.9±2.3 | 0.8±2.1 | 1.7±2.7 | 0.011 |
| Ejection fraction, % | 414 | 59±10 | 59±10 | 60±10 | 59±11 | 57±11 | 0.252 |
| Mass:volume ratio g/mL | 406 | 1.21±0.39 | 1.16±0.41 | 1.20±0.38 | 1.23±0.38 | 1.26±0.38 | 0.321 |
| Mass:volume ratio z-score | 406 | 2.65±3.22 | 1.97±3.06 | 2.41±2.97 | 2.86±3.15 | 3.49±3.63 | 0.011 |
| dP/dtic, mm Hg/s | 449 | 1125 (802,1700) |
1257 (N=115) |
1134 (N=106) |
1114 (N=134) |
997 (N=94) |
0.027* |
| Tei index (by Tissue Doppler) | 462 | 0.64±0.19 | 0.60±0.17 (N=117) |
0.62±0.14 (N=99) |
0.63±0.17 (N=144) |
0.70±0.24 (N=102) |
<.001 |
| E’, cm/sec | 452 | 9.3±3.3 | 9.5±3.3 (N=113) |
9.6±2.9 (N=97) |
9.0±3.3 (N=141) |
9.5±3.8 (N=101) |
0.517 |
| E:A ratio | 344 | 1.48 (1.21,1.92) |
1.45 (N=88) |
1.46 (N=80) |
1.55 (N=105) |
1.48 (N=71) |
0.883* |
| E:E’ ratio | 297 | 7.79 (5.94,9.89) |
8.03 (N=74) |
7.15 (N=66) |
8.12 (N=94) |
7.63 (N=63) |
0.216* |
| Systemic ventricular FP rate, cm/sec | 143 | 64±20 | 63±16 (N=39) |
64±19 (N=40) |
69±24 (N=41) |
57±15 (N=23) |
0.107 |
| Restrictive pattern present | 344 | 52% | 52% | 54% | 56% | 44% | 0.421 |
| Diastolic dysfunction grade | 327 | 0.438† | |||||
| Normal | 28% | 23% | 27% | 25% | 38% | ||
| Impaired relaxation | 9% | 12% | 8% | 9% | 8% | ||
| Pseudonormalization | 41% | 45% | 47% | 42% | 29% | ||
| Restrictive | 22% | 20% | 18% | 24% | 26% | ||
| Overall AV valve regurgitation | 528 | 0.541† | |||||
| None | 26% | 27% | 17% | 33% | 22% | ||
| Mild | 55% | 53% | 59% | 50% | 60% | ||
| Moderate | 19% | 19% | 23% | 17% | 17% | ||
| Severe | <1% | 0% | <1% | 0% | 0% | ||
| Semilunar valve regurgitation | 314 | 0.148† | |||||
| None | 51% | 57% | 52% | 54% | 37% | ||
| Mild | 40% | 37% | 42% | 36% | 50% | ||
| Moderate | 9% | 7% | 6% | 10% | 13% | ||
| Cardiac MRI | |||||||
| N | 161 | 161 | 33 | 37 | 50 | 41 | |
| End-diastolic volume /BSA , mL/m | 85±25 | 96±26 | 89±29 | 81±20 | 79±23 | 0.011 | |
| End-systolic volume /BSA13, mL/m2 | 37±16 | 41±17 | 38±19 | 36±16 | 35±14 | 0.395 | |
| Ejection fraction, % | 57±10 | 58±8 | 59±9 | 56±12 | 56±9 | 0.476 | |
| Stroke volume /BSA, mL/ m2 | 51±14 | 52±12 | 52±14 | 48±14 | 51±15 | 0.500 | |
| Mass/BSA13, g/m2 | 72±21 | 75±17 | 70±18 | 68±19 | 76±26 | 0.182 | |
| Mass:volume ratio g/mL | 0.89±0.31 | 0.83±0.24 | 0.83±0.29 | 0.87±0.25 | 1.01±0.40 | 0.036 | |
| Exercise Performance Measures | |||||||
| N | 412 | 68 | 95 | 152 | 97 | ||
| Peak VO2, mL/kg/min | 403 | 26±7 | 27±8 (N=65) |
28±7 (N=94) |
26±6 (N=148) |
25±7 (N=96) |
0.028 |
| Percent predicted peak VO2 | 403 | 65±16 | 67±19 | 68±17 | 65±15 | 59±14 | <.001 |
| Peak VO2 consumption at AT, mL/kg/min | 317 | 19±6 | 24±8 (N=35) |
20±7 (N=65) |
19±6 (N=131) |
16±5 (N=86) |
<.001 |
| Percent predicted VAT | 317 | 78±25 | 95±30 | 82±26 | 77±22 | 69±20 | <.001 |
| Maximum heart rate, bpm | 405 | 154±23 | 152±22 (N=64) |
156±24 (N=95) |
157±21 (N=150) |
150±26 (N=96) |
0.122 |
| Measures of Functional Status | |||||||
| CHQ-PF Physical Summary Score | 511 | 45.3±11.9 | 45.7±12.1 | 45.8±10.7 | 45.4±12.6 | 44.1±11.7 | 0.689 |
| CHQ-PF Psychosocial Summary Score | 511 | 47.2±10.8 | 47.9±10.6 | 47.2±11.1 | 45.5±10.9 | 49.1±10.5 | 0.052 |
Percentages may not add to 100 due to rounding
P-value is from analysis of variance for continuous outcomes and chi-square test for categorical outcomes unless otherwise specified
Kruskal-Wallis test p-value
Mantel-Haenszel test for linear trend p-value
N = number of subjects for whom data are available
BMI = Body Mass Index
TCPC = Total Cavopulmonary Connection
CHQ-PF = Child Health Questionnaire-Parent Form
dP/dtic = Estimated maximum first derivative of ventricular pressure
E’ = Tissue Doppler peak early diastolic velocity
E:A ratio = Ratio of early to late atrioventricular valve diastolic velocities
E:E’ ratio = Ratio of atrioventricular valve to Tissue Doppler peak early diastolic velocity
FP = Flow propagation
BSA=Body surface area
AV = Atrioventricular
VO2 = Rate of oxygen consumption
AT = Anaerobic threshold
VAT = Peak VO2 consumption at anaerobic threshold
Stage II = Superior cavopulmonary anastomosis
MRI = Magnetic resonance imaging
Restrictive pattern E/A>2, or 1<E/A<2 and DT<140 ms
Diastolic dysfunction grades: 0, Normal = [(1<E/A≤2) and (DT≥140 ms) and (E/E’≤10)]; 1, Impaired relaxation = [E/A=1]; 2, Pseudonormalization = [(1<E/A≤2) and [(DT<140 ms) or (E/E’ >10) or (FP<55 cm/s)]]; 3. Restrictive = [E/A>2]