Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC)
Oktay Tutarel , Lucia Baris, Werner Budts, Mohamad Gamal Abd-El Aziz, Csilla Liptai, David Majdalany, Silvana Jovanova, Alexandra Frogoudaki, Heidi M Connolly, Mark R Johnson, Aldo P Maggioni , Roger Hall, Jolien W Roos-Hesselink on behalf of the ROPAC Investigators Group
Heart 2022;108:117–123.
http:// dx. doi.org/ 10. 1136/ heartjnl-2020-318685
Take Home Points:
- Maternal mortality was zero
- Heart failure and arrhythmias are the most common complication of pregnancy
- There appears to be no major change in the systemic RV due to pregnancy
Commentary
Transposition of the great arteries (TGA) repaired with an atrial switch and congenitally corrected transposition of the great arteries (CCTGA) represent a cohort of the more complex patients with congenital heart disease often with difficult to manage heart failure and arrhythmias. There have been concerns about women with a systemic right ventricle becoming pregnant and the sequalae on the right ventricle. The ROPAC registry as part of a wider review of pregnancy outcomes offers some insight into this cohort.
Methods
From 2007 to 2018 pregnant patients with a variety of cardiac conditions were enrolled prospectively and from this cohort women with TGA or CCTGA were then identified making sure only those with a systemic RV were included and baseline characteristics recorded.
End points for analysis were maternal death, arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischaemic coronary events and thromboembolic events. Secondary end points were adverse foetal or neonatal outcomes.
Results
A total of 162 women were analysed as part of the study, TGA- 121 and CCTGA-41. The majority of women (73%) had relatively well-preserved systemic RV function, identified as greater than 40% with less than 5% of women having signs of heart failure pre-pregnancy and most being in their first pregnancy. Major maternal outcomes are shown below:
Outcome |
All |
CCTGA |
TGA |
p-value |
Maternal mortality |
0 |
0 |
0 |
Na |
Hospital admission |
9.8% |
19.5% |
6.6% |
0.03 |
Heart failure |
9.8% |
12.2% |
9.1% |
0.56 |
SVT |
3.1% |
0 |
4.1% |
0.33 |
VT |
3.7% |
4.9% |
0.8% |
0.64 |
Thromboembolism |
1.8% |
4.9% |
0.8% |
0.16 |
The most common mode of delivery was a vaginal delivery for women with a systemic RV (51.5%). There was no significant change in the amount of tricuspid regurgitation in women with scan pre-and post-pregnancy nor in RV dimensions. Adverse maternal outcomes on univariate analysis were associated with women with pre-existing heart failure symptoms or systemic ventricular function less than 40%.
Obstetric and foetal outcomes are shown below:
Outcome |
All |
CCTGA |
TGA |
p-value |
Caesarean section (LSCS) |
48.5% |
61% |
44.6% |
0.07 |
LSCS for cardiac reasons |
32.9% |
56% |
22.2% |
0.01 |
Foetal death |
0.6% |
0% |
0.8% |
1 |
Neonatal death |
0% |
0% |
0% |
Na |
Premature birth |
21% |
20% |
21.5% |
1 |
Growth retardation |
4.3% |
2.4% |
5% |
0.68 |
Lower weight babies were associated as expected with maternal medication usage(p=0.01).
Discussion
This study showed good outcomes for women with a systemic right ventricle thought with symptomatic heart failure observed in 10% of women. Compared to historical data adverse outcomes in ROPAC were lower which is useful in the counselling of women. Compared to previous studies, the lack of progression in RV dysfunction was reassuring. Surprisingly, CCTGA was associated with more hospital admissions and more LSCS for cardiac reasons that TGA which was the opposite to previously reported studies and reasons for this are unclear thought the amount of women with a pacemaker was higher in the CCTGA group.
Further studies with more detailed echo follow up are needed to assess the systemic RV in pregnancy.