Baseline characteristics of the female patients after Fontan repair (n = 38)
| *These may differ from the type at menarche due to repeat Fontan procedures; †including three third operations. | |
| AP, atriopulmonary; AV, atrioventricular; RA, right atrial; RV, right ventricular. | |
| Primary cardiac malformation | |
| Tricuspid atresia | 18 (47.4%) |
| Double inlet left ventricle | 7 (18.4%) |
| Pulmonary atresia with intact ventricular septum | 3 (7.9%) |
| Double outlet right ventricle | 3 (7.9%) |
| Complete AV septal defect | 2 (5.3%) |
| Other | 5 (18.5%) |
| Palliative procedures before Fontan | |
| Blalock–Taussig shunt | 16 (42.1%) |
| Waterston shunt | 3 (7.9%) |
| Classic Glenn shunt | 2 (5.3%) |
| Rashkind atrioseptostomy | 1 (2.6%) |
| Pulmonary artery banding | 1 (2.6%) |
| None | 15 (39.4%) |
| Median age at first palliation (years) | 3.5 (range 1–25) |
| Type of Fontan repair (at menarche/at inclusion*) | |
| AP connection (RA-AP) | 22 (57.9%)/23 (60.5%) |
| AV connection (RA-RV) | 8 (21.1%)/5 (13.2%) |
| Total cavopulmonary connection | 3 (7.9%)/10 (26.3%) |
| None | 5 (13.2%)/0 |
| Median age at (primary) Fontan repair (years) | 7.5 (range 3–25) |
| Number of repeat Fontan operations | 20† |
| Sequelae at inclusion | |
| Atrial flutter/atrial fibrillation | 19 (50.0%) |
| Permanent pacemaker | 6 (15.7%) |
| Protein C deficiency | 6 (15.7%) |
| Thromboembolic complications | 4 (10.5%) |
| Protein-losing enteropathy | 1 (2.6%) |
| New York Heart Association class at inclusion | |
| I/II | 36 (94.7%) |
| III/IV | 2 (5.3%) |
| Echocardiography at inclusion | |
| Severe impairment of systemic ventricular function | 5 (13.2%) |
| Severe (grade III–IV) systemic AV valve regurgitation | 6 (15.7%) |