Table 1. Pregnancy outcomes in women with Marfan's syndrome following aortic root replacement from survey and Marfan's clinics a .
Patient (pregnancy number following ARR) | Marfan's criteria | Indication for ARR | Age at ARR (y) | Fetal outcome | Delivery type | Anesthesia | AD related to pregnancy | BB | Chronic HTN | Hypertensive disorder of pregnancy b | Smoking | Breast feeding ≥ 2 wk |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 (1) | Scoliosis, thumb sign, MVP, pes planus, lens dislocation, pectus carinatum | AA | 28 | Term | Cesarean | Epidural | No | + | + | − | − | + |
1 (2) | Therapeutic abortion | SVD | None | No | + | + | − | − | NA | |||
1 (3) | Term | Cesarean | Epidural | No | + | + | − | − | + | |||
2 (1) | Scoliosis, thumb sign, pectus excavatum | AA | 31 | Term | SVD | Epidural | No | + | + | − | − | − |
3 (1) | FBN1 mutation, lens dislocation, pneumothorax, thumb sign, FH of AD | AA | 31 | Miscarriage | SVD | General | No | + | − | − | − | NA |
3 (2) | Miscarriage | SVD | General | No | + | − | − | − | NA | |||
3 (3) | Term | SVD | Epidural | No | + | − | − | − | + | |||
4 (1) | FBN1 mutation, MVP, thumb sign, pectus excavatum | AA | 24 | Term | SVD | Epidural | No | − | − | − | − | + |
4 (2) | Term | SVD | Epidural | No | − | − | − | − | + | |||
5 (1) | FBN1 mutation, MVP, pectus excavatum, thumb sign, FH of AD | AA | 32 | Miscarriage | SVD | None | No | + | − | − | − | NA |
5 (2) | Preterm | Cesarean | General | No | + | − | − | − | + | |||
6 (1) | Pectus carinatum, pes planus | AA | 27 | Preterm | Cesarean | Epidural | No | + | − | − | NA | + |
7 (1) | FBN1 mutation, scoliosis, pectus carinatum | AA | 22 | Term | Cesarean | Epidural | No | + | − | − | NA | − |
8 (1) | FBN1 mutation, FH of Marfan's syndrome | AA | 22 | Miscarriage | SVD | General | No | + | − | − | − | NA |
8 (2) | Term | Cesarean | Epidural | No | + | − | − | − | − | |||
8 (3) | Term | Cesarean | Epidural | No | + | − | − | − | − | |||
9 (1) | FBN1 mutation, AA | AA | 22 | Preterm | SVD | Epidural | No | + | + | − | + | +, 6 mo |
9 (2) | Preterm | Forceps assisted | Epidural | No, descending TAA 3 y after 2 nd pregnancy | + | + | − | + | +, 3 mo | |||
9 (3) | Preterm | Forceps assisted | Epidural | No | + | + | − | + | +, 3 mo | |||
10 (1) | FBN1 mutation, dural ectasia, AA | AA | 26 | Term | Cesarean | Spinal | No | + | − | − | − | +, > 12 mo |
11 (1) | Ghent systemic score 9, AA | AD | 29 | Term | Forceps assisted | Epidural | No, c Fatal type B dissection 1-y postpartum | + | − | +, Preeclampsia | − | +, 12 mo |
12 (1) | FBN1 mutation, AA, FH of AD | AA | 24 | Term | Cesarean | Epidural | No | + | − | − | − | + |
13 (1) 13 (2) |
AA, scoliosis, FH of Marfan's syndrome | AA | 34 | Miscarriage | Vacuum aspiration | Paracervical block | No | + | − | − | − | NA |
Term | SVD | Epidural | No | + | − | − | − | + | ||||
14 (1) 14 (2) |
AA, FBN1 mutation, FH of Marfan's, high systemic score | AD | 31 | Term | SVD | Epidural | No | + | − | − | − | − |
Term | SVD | Epidural | No | + | − | − | − | − |
Abbreviations: AA, Aortic Aneurysm; AD, aortic dissection; ARR, aortic root replacement;; BB, β -blocker; FH, family history; HTN, hypertension; MVP, mitral valve prolapse; NA, missing data or not applicable; SVD, spontaneous vaginal delivery; TAA, thoracic aortic aneurysm; +, present; −, absent
Washington University in St. Louis School of Medicine and Johns Hopkins University.
Hypertensive disorder of pregnancy: gestational hypertension, pre-eclampsia, eclampsia.
Smoking data was available for 18 live births.