| All Patients (n = 147) | Patients with MPBF (n = 53) | Patients without MPBF (n = 94) | P Value |
---|---|---|---|---|
Age at Fontan (yr)+ | 3.2 [2.2–6] | 2.9 [2–4.8] | 3.3 [2.3–7.6] | 0.21 |
Age at CMR (yr) | 21.8 [16.5–30.6] | 20.7 [16.5–25] | 23.2 [16.6–32.2] | 0.20 |
Time since Fontan (yr) | 17.2 [13.1–22.9] | 16.5 [13.1–20.9] | 17.9 [12.9–23.9] | 0.23 |
Time between CMR and CPET (mos) | 2.8 [0–13.8] | 1 [0–14.5] | 4.5 [0–11.5] | 0.10 |
Body surface area at CMR (m2) | 1.7 [1.5–1.9] | 1.7 [1.5–1.8] | 1.7 [1.5–2] | 0.52 |
Cardiac Diagnosis | Â | Â | Â | 0.14 |
 Tricuspid atresia | 37 (25%) | 14 (26%) | 23 (24%) |  |
 Double-inlet left ventricle | 24 (16%) | 7 (13%) | 17 (18%) |  |
 HLHS | 25 (17%) | 14 (26%) | 11 (12%) |  |
 Unbalanced AV canal | 9 (6%) | 4 (8%) | 5 (5%) |  |
 Double-outlet right ventricle | 25 (17) | 9 (17%) | 16 (17%) |  |
 Complex 2 ventricle | 13 (9%) | 2 (4%) | 11 (12%) |  |
 Hypoplastic TV/RV | 6 (4%) | 0 (0%) | 6 (7%) |  |
 Pulmonary atresia/IVS | 2 (1%) | 1 (2%) | 1 (1%) |  |
 Mitral atresia | 6 (4%) | 2 (4%) | 4 (4%) |  |
Levocardia | 122 (83%) | 78 (83%) | 44 (83%) | 0.90 |
HLHS only | 25 (17%) | 14 (26%) | 11 (12%) | 0.04 |
Heterotaxy | 17 (12%) | 8 (16%) | 9 (10%) | 0.23 |
Genetic diagnosis | 11 (7%) | 5 (9%) | 6 (6%) | 0.45 |
Dominant ventricular morphology | Â | Â | Â | 0.68 |
 Left ventricle | 58 (40%) | 19 (42%) | 39 (42%) |  |
 Right ventricle | 49 (33%) | 20 (32%) | 29 (31%) |  |
 2 ventricles | 40 (27%) | 14 (26%) | 26 (27%) |  |
History of neonatal surgery | 79 (54%) | 34 (64%) | 45 (48%) | 0.06 |
History of Glenn operation | 75 (51%) | 33 (62%) | 39 (41%) | 0.02 |
History of Damus-Kaye-Stansel | 42 (29%) | 21 (40%) | 21 (22%) | 0.04 |
Bilateral Glenn operation | 13 (8.8%) | 5 (10%) | 8 (10%) | 0.50 |
Fontan Type | Â | Â | Â | 0.43 |
 Lateral tunnel | 98 (67%) | 41 (77%) | 57 (60%) |  |
 RA-Pulmonary artery | 31 (21%) | 8 (15%) | 23 (25%) |  |
 Extracardiac | 11 (8%) | 3 (6%) | 8 (11%) |  |
 RA-RV Fontan | 5 (3.4%) | 1 (2%) | 4 (4%) |  |
History of any PA interventiona | 34 (23%) | 20 (38%) | 13 (14%) | 0.004 |
 History of PA intervention before Fontan | 12 (8%) | 10 (19%) | 2 (2%) | 0.001 |
 History of PA intervention at Fontan | 3 (2%) | 1 (2%) | 2 (2%) | 0.70 |
 History of PA intervention after Fontan | 19 (13%) | 10 (19%) | 9 (10%) | 0.09 |
Pulmonary vein stenosisa | 5 (3%) | 4 (7%) | 1 (1%) | 0.06 |