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. 2020 Jul 31;41(6):1248–1298. doi: 10.1007/s00246-020-02408-w
Total (n = 276) Type 1: Absent central PA (n = 45) Type 2: incompletely arborizing PA (n = 152) Type 3: normally arborizing PA (n = 48) Type 4: PDA or anomalous PA (n = 31) p value
Demographic features
 Female sex 141 (51%) 22 (49%) 72 (47%) 27 (56%) 20 (65%) 0.30
 Age (Months) 3.7 (1.0,6.0) 4.7 (2.5,6.4) 4.6 (1.7,6.2) 1.6 (0.1,4.1) 1.3 (0.2,4.4) <0.001
Weight (kg) 5.2 (3.7,6.5) 5.6 (4.3,6.6) 5.3 (4.0,6.5) 4.2 (3.5,6.2) 4.8 (3.6,5.7) 0.13
PA and MAPCA measurements
 Intrapericardial PA diameter (mm) 2.1 (0.8,3.2) 0 (0,0) 2.6 (1.8,3.9) 2.6 (2.1,3.2) 0.6 (0,1.4) <0.001
 Intrapericardial PA index (mm2/m2 29 (4,64) 0 (0, 0) 40 (18,89) 44 (29,70) 4 (0,22) <0.001
 Modified Nakata (mm2/m2) 56 (16,97) 0 (0,0) 60 (27,105) 63 (43,82) 118 (72,162) <0.001
 MAPCA index (mm2/m2) 71 (33,129) 154 (119,207) 80 (49,122) 0 (0,39) 35 (20,63) <0.001
 TNPAI (mm2/m2) 148 (99,195) 154 (119, 207) 165 (111, 204) 80 (51, 116) 165 (121, 225) <0.001
First surgery
 Complete repair 182 (66%) 34 (76%) 109 (72%) 20 (42%) 19 (61%) <0.001
 Aortopulmonary window 35 (13%) 0 (0%) 10 (7%) 25 (52%) 0 (0%)
 Other palliation 59 (21%) 11 (24%) 33 (22%) 3 (6%) 12 (39%)
 Current status
  Single-stage complete repair 182 (66%) 34 (76%) 109 (72%) 20 (42%) 19 (61%) <0.001
  Multi-stage complete repair 67 (24%) 7 (16%) 27 (18%) 25 (52%) 8 (26%)
  Unrepaired 12 (4%) 1 (2%) 6 (4%) 3 (6%) 2 (6%)
  Death without repair 15 (5%) 3 (7%) 10 (7%) 0 (0%) 2 (6%)
Complete repair (ever) 249 (90%) 41 (91%) 136 (89%) 45 (94%) 27 (87%) 0.76
RV:Ao at complete repair 0.33 (0.28,0.40) 0.31 (0.27,0.36) 0.33 (0.28,0.40) 0.31 (0.28,0.38) 0.34 (0.25,0.40) 0.40