Clinical characteristics of all patients offered preconception counselling (PCC)†
| Trying for pregnancy (n=19)§ | Considering future pregnancy (n=16)‡ | Decided against pregnancy (n=15)* | Declining PCC (n=3) | |
|---|---|---|---|---|
| Age at PCC (mean), years | 29.4 | 23.6 | 29.5 | 33.5 |
| Underlying anatomy, tricuspid atresia/other | 8/11 | 11/5 | 7/8 | 2/1 |
| Systemic ventricle morphology (left, right) | 15/4 | 14/2 | 13/2 | 3/0 |
| Fontan type, TCPC/AP Fontan | 13/6 | 11/5 | 11/8 | 1/2 |
| Age at Fontan completion (mean±SD), years | 8.9 (4.0) | 6.6 (2.6) | 8.8 (5.7) | 5.6 (1.1) |
| Prior thromboembolism | 2 | 0 | 1 | 0 |
| CARPREG score prior to pregnancy, 0/1/≥2 | 4/10/5 | 6/10/2 | 4/5/2 | 1/2 |
| Prior atrial arrhythmia | 12 | 4 | 7 | 1 |
| New York Heart Association Functional Class prior to pregnancy (NYHA 1/2/3/4) | 16/3/0/0 | 16/0/0/0 | 14/1/0/0 | 3/0/0/0 |
| Oxygen saturations <92% prior to pregnancy | 8 | 4 | 8 | 1 |
| Haemoglobin (g/dL) at PCC (mean) at PCC | 14.4 | 14.1 | 14.7 | 13.7 |
| Systemic ventricle morphology (left/right) | 15/4 | 14/2 | 13/2 | 3/0 |
| Systemic Ventricular Function Grade: good/mildly impaired/moderately impaired/poor | 18/0/1/0 | 15/0/1/0 | 11/2/2/0 | 3/0/0/0 |
*Includes 1 patient with a diagnosis of premature ovarian failure.
†The total population included 12 double outlet right ventricle, 10 double inlet left ventricle, 4 pulmonary atresia, ventricular septal defect with major aortopulmonary artery collaterals.
‡One had Bjork modification.
§Two had Bjork modification.
¶The CARPREG study proposed a prognostic index that is applicable to underlying both congenital and acquired cardiovascular disease. Adverse cardiac events that were recorded include pulmonary oedema, arrhythmia, stroke or cardiac death and were able to be predicted by four factors: (1) prior episodes of heart failure, (2) arrhythmias prior to pregnancy, (3) baseline functional class >II or cyanosis; left heart obstructive lesions, (4) poor systemic ventricular ejection fraction <40%. Each of the above was assigned an individual point to each predictor to produce a mortality risk index: an index of 0, 1 or ≥2 correlated with maternal mortality rates of 5%, 27% and 75%, respectively.
CARPREG, cardiac disease in pregnancy; TCPC, total cavopulmonary connection.