Table 1.
Characteristics of patients receiving AVR at 1-20 year of age for congenital aortic valve disease by valve substitute
| Patient characteristics | Total N = 911 | R-AVR N = 416 (45.7%) | M-AVR N = 391 (42.9%) | T-AVR N = 104 (11.4%) | p-value a |
|---|---|---|---|---|---|
| Males | 669 (73.4) | 294 (70.7) | 300 (76.7) | 75 (72.1) | 0.14 |
| Age at AVR-Median (IQR) | 13.4 (8.4 – 16.5) | 12.6 (7.0-16.1) | 13.9 (9.3-16.9) | 13.9 (8.7-16.5) | <0.01 |
| 1 - 5 years | 143 (15.7) | 84 (20.2) | 40 (10.2) | 19 (18.3) | |
| 6 – 10 years | 199 (21.8) | 95 (22.8) | 85 (21.7) | 19 (18.3) | |
| 11 - 20 years | 569 (62.5) | 237 (57.0) | 266 (68.0) | 66 (63.5) | |
| Era of AVR (~tertiles) | <0.001 | ||||
| 1982 - 1994 | 265 (29.1) | 40 (9.6) | 192 (49.1) | 33 (31.7) | |
| 1995 - 1998 | 348 (38.2) | 194 (46.6) | 119 (30.4) | 35 (33.7) | |
| 1999 - 2003 | 298 (32.7) | 182 (43.8) | 80 (20.5) | 36 (34.6) | |
| Valve hemodynamics at AVR | <0.001 | ||||
| Stenosis | 105 (11.5) | 50 (12.0) | 47 (12.0) | 8 (7.7) | |
| Regurgitation | 279 (30.6) | 59 (14.2) | 174 (44.5) | 46 (44.2) | |
| Both | 527 (57.9) | 307 (73.8) | 170 (43.5) | 50 (48.1) | |
| Other left heart obstruction b | 242 (26.6) | 107 (25.7) | 110 (28.1) | 25 (24.0) | 0.32 |
| Present at 1 level | 169 (18.6) | 70 (16.8) | 84 (21.5) | 15 (14.4) | |
| Present at 2 levels | 65 (7.1) | 33 (7.9) | 22 (5.6) | 10 (9.6) | |
| Present at 3 levels | 8 (0.9) | 4 (1.0) | 4 (1.0) | 0 (0.0) | |
| Genetic conditions (all) | 68 (7.5) | 22 (5.3) | 32 (8.2) | 14 (13.5) | 0.01 |
| Down syndrome | 11 (1.2) | 3 (0.7) | 7 (1.8) | 1 (1.0) | 0.38 |
| Otherc | 57 (6.3) | 19 (4.6) | 25 (6.4) | 13 (12.5) | 0.01 |
| Concurrent procedures | 123 (13.5) | 35 (8.4) | 75 (19.2) | 13 (12.5) | <0.001 |
| Konno procedure | 103 (11.3) | 34 (8.2) | 58 (14.8) | 11 (10.6) | 0.01 |
| Mitral valve repair | 24 (2.6) | 0 (0.0) | 22 (5.6) | 2 (1.9) | <0.001 |
| Arch repair | 7 (0.8) | 2 (0.5) | 4 (1.0) | 1 (1.0) | 0.55 |
| Pacemaker placementd | 44 (4.8) | 17 (4.1) | 24 (6.1) | 3 (2.9) | 0.27 |
| Prior cardiac interventions | 528 (58.0) | 210 (50.5) | 253 (64.7) | 65 (62.5) | <0.001 |
| Balloon valvuloplasty | 142 (15.6) | 99 (23.8) | 30 (7.7) | 13 (12.5) | <0.001 |
| Aortic valve repair | 326 (35.8) | 143 (34.4) | 143 (36.6) | 40 (38.5) | 0.67 |
| Aortic arch repair | 100 (11.0) | 50 (12.0) | 38 (9.7) | 12 (11.5) | 0.69 |
| Pacemaker placement | 20 (2.2) | 2 (0.5) | 17 (4.4) | 1 (1.0) | <0.001 |
| Complex heart disease e | 82 (9.0) | 4 (1.0) | 68 (17.4) | 10 (9.6) | <0.001 |
p-values comparing the distribution of categorical characteristics across valve types were estimated using chi-squared or Fisher’s exact test when at least one expected cell count was less than 5 (left heart obstruction, Down’s syndrome, concurrent mitral valve repair, arch repair or pacemaker placement); Kruskal-Wallis test was used for continuous factors since normality was not assumed
Left heart obstruction was considered as stenosis at the mitral valve, sub-aortic area, or aortic arch
Other syndromes include Digeorge, Goldenhar, Klippel-Feil, Noonan, Osteogenesis Imperfecta, Pierre-Robin, Triple X, Trisomy 15, Turner Syndrome, and Vater Syndrome
During or up to 30 days after AVR procedure
Includes: double outlet right ventricle, dextro- or levo-transposed great arteries, tetralogy of Fallot, truncus arteriosus communis, pulmonary atresia