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Table 1 Patient Demographic and Clinical Characteristics

From: Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity

  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     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     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     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
  1. Abbreviations: AV atrioventricular, CMR cardiovascular magnetic resonance, CPET cardiopulmonary exercise test, HLHS hypoplastic left heart syndrome, IVS intact ventricular septum, MPBF maldistribution of pulmonary blood flow, PA pulmonary artery, RA right atrium, RV right ventricle, TV tricuspid valve
  2. aPrior to CMR. + Data presented as median [interquartile range]