Table 5.
Fontan Cross-Sectional Study Patient Characteristics by Superior Cavopulmonary Connection (Stage II Surgery) Performed
| With Stage II | Without Stage II | |||
|---|---|---|---|---|
| Characteristic | Mean ± SD Median Or % |
Mean ± SD Median Or % |
P-value | Age-adjusted pvalue |
| N | N=408 | N=138 | ||
| Age at enrollment, yr | 10.3 | 15.7 | <.001* | − |
| Age at Fontan, yr | 3.5±2.0 | 3.2±2.3 | 0.318 | <.001 |
| Age at volume unloading surgery, yr | 0.8 | 2.8 | <.001* | <.001 |
| Male | 60% | 60% | 1.00 | .497 |
| Race | 0.024 | .491 | ||
| White | 78% | 86% | ||
| Black | 11% | 9% | ||
| Asian | 2% | 3% | ||
| Other | 9% | 2% | ||
| Hispanic | 7% | 5% | 0.550 | .588 |
| Growth | ||||
| Percentile for stature-for-age | 24 | 31 | 0.085* | .195 |
| Z-score for stature-for-age | −0.7±1.3 | −0.5±1.1 | 0.056 | .054 |
| Percentile for weight-for-age | 32 | 48 | <.001* | .005 |
| Z-score for weight-for-age | −0.5±1.4 | −0.1±1.1 | 0.001 | <.001 |
| Body mass index z-score | −0.13±1.17 | 0.18±1.00 | 0.004 | .003 |
| Fontan type | <.001 | .022 | ||
| Atriopulmonary connection | 7% | 33% | ||
| TCPC Intracardiac lateral tunnel | 61% | 56% | ||
| TCPC extracardiac lateral tunnel | 16% | 3% | ||
| TCPC extracardiac conduit | 16% | 2% | ||
| Other | <1% | 7% | ||
| Ventricular type | <.001 | .108 | ||
| Left Ventricular | 44% | 62% | ||
| Right Ventricular | 40% | 16% | ||
| Mixed | 16% | 22% | ||
| Currently on pacemaker | 14.2% | 13.8% | 1.000 | .059 |
| Serology | ||||
| BNP, pg/ml† | 11.8 | 17.6 | 0.001* | .066 |
| Predominant rhythm | 0.772 | .292 | ||
| Normal sinus rhythm | 66% | 70% | ||
| Atrial escape | 9% | 9% | ||
| Junctional escape | 6% | 4% | ||
| Paced | 9% | 6% | ||
| Other | 10% | 11% | ||
| Echo | ||||
| Heart rate z-score | −0.17±0.99 | −0.29±0.92 | 0.267 | .247 |
| End-diastolic volume z-score | −0.6±1.8 | −0.9± 2.3 | 0.167 | .111 |
| End-systolic volume z-score | 0.3± 2.2 | −0.0±3.0 | 0.400 | .190 |
| Ejection fraction z-score | −0.9±2.1 | −0.9±2.0 | 0.983 | .340 |
| Stroke volume z-score | −1.0±1.8 | −1.3±1.8 | 0.086 | .168 |
| Mass z-score | 1.0±2.1 | 0.8±2.6 | 0.323 | .008 |
| Ejection fraction, % | 59±11 | 58±10 | 0.966 | .343 |
| Mass:volume ratio, g/ml | 1.21±0.39 | 1.21±0.39 | 0.957 | .316 |
| Mass:volume ratio z-score | 2.57±3.10 | 2.92±3.63 | 0.356 | .380 |
| dp/dtic, mmHg/s | 1182 (N=341) |
1030 (N=108) |
0.044* | .368 |
| Tei index (by Tissue Doppler) | 0.6 (N=346) |
0.6 (N=116) |
0.303* | .175 |
| E’, cm/sec | 9.5±3.4 (N=338) |
9.0±3.2 (N=114) |
0.226 | .293 |
| E: A ratio | 1.48 (N=247) |
1.48 (N=97) |
0.985* | .530 |
| E: E’ ratio | 7.79 (N=215) |
7.80 (N=82) |
0.400* | .177 |
| Systemic ventricular FP rate, cm/sec | 65±21 | 60±15 | 0.111 | .219 |
| Restrictive pattern present | 53% | 49% | 0.472 | .932 |
| Diastolic dysfunction grade | 0.280 | .830 | ||
| Normal | 25% | 33% | ||
| Impaired relaxation | 9% | 9% | ||
| Pseudonormalization | 44% | 33% | ||
| Restrictive | 21% | 24% | ||
| Overall AV valve regurgitation grade | 0.725 | .406 | ||
| None | 25% | 29% | ||
| Mild | 56% | 54% | ||
| Moderate | 19% | 17% | ||
| Severe | <1% | 0% | ||
| Semilunar valve regurgitation grade | 0.830 | .482 | ||
| None | 52% | 48% | ||
| Mild | 39% | 44% | ||
| Moderate | 9% | 8% | ||
| Cardiac MRI | ||||
| N | 108 | 53 | ||
| End-diastolic volume / BSA1.3, ml/m2 | 87±27 | 81±21 | 0.109 | .658 |
| End-systolic volume / BSA1.3, ml/m2 | 38±17 | 36±14 | 0.391 | .739 |
| Ejection Fraction, % | 57±10 | 56±9 | 0.555 | .986 |
| Stroke volume/ BSA, mL/m2 | 51±14 | 50±13 | 0.596 | .884 |
| Mass / BSA1.3, g/m2 | 73±21 | 69±19 | 0.205 | .130 |
| Mass:volume ratio g/mL | 0.88±0.28 | 0.90±0.36 | 0.751 | .455 |
| Exercise Performance Measurements | ||||
| Peak VO2, ml/kg/min | 27±7.0 | 25±7 | 0.015 | .207 |
| Percent predicted peak VO2 | 66±17 | 60±15 | <.001 | .067 |
| Peak VO2 consumption at AT, ml/kg/min | 20±7 | 17±6 | 0.001 | .658 |
| Percent predicted VAT | 81±26 | 72±21 | 0.001 | .346 |
| Max heart rate, bpm | 155±24 | 153 ± 22 | 0.390 | .490 |
| Measures of Functional Status | ||||
| CHQ-PF Physical Summary Score | 45.3±12.1 | 45.3±11.3 | 0.974 | .555 |
| CHQ-PF Psychosocial Summary Score | 46.5±11.1 | 49.3±9.8 | 0.010 | .008 |
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
Wilcoxon rank sum test p-value
There was significant age-adjusted interaction of Stage II status and ventricular morphologic subtype (p=0.008), with lower BNP for subjects who underwent Stage II surgery compared with those who did not in the LV subgroup (log BNP 2.51±0.07 vs. 2.98±0.11, p<.001), but no difference in BNP by Stage II surgery status for subjects with RV or mixed type morphology.
N = number of subjects for whom data are available
See Table 1 footnote for abbreviations legend