Tutarel O, Baris L, Budts W, Gamal Abd-El Aziz M, Liptai C, Majdalany D, Jovanova S, Frogoudaki A, Connolly HM, Johnson MR, Maggioni AP, Hall R, Roos-Hesselink JW; ROPAC Investigators Group.Heart. 2021 Apr 28:heartjnl-2020-318685. doi: 10.1136/heartjnl-2020-318685. Online ahead of print.PMID: 33911009
Take Home Points:
In women with a systemic right ventricle (sRV) after atrial switch for transposition of the great arteries (121 patients) or congenitally corrected transposition of the great arteries (ccTGA, 41 patients), in this international prospective observational registry, pregnancy was well tolerated with favourable maternal and fetal outcomes with:
- 26 (21%) having at least 1 major adverse cardiac event (MACE)
- 16 (9.8%) having heart failure needing treatment
- 11 (6.8%) having arrthymia (5 atrial and 6 ventricular) needing treatment
- 4 (2.5%) having other events
- No deterioration in sRV was seen in those who had pre and post pregnancy echoes
Pre-pregnancy signs of heart failure and sRV ejection fraction <40% predicted MACE.
Commentary from Dr Simon MacDonald (London, UK) section editor of ACHD Journal Watch:
There is uncertainty about how a systemic right ventricle supports the systemic circulation and adapts to the volume changes seen in pregnancy. This is important in patients with transposition of the great arteries (TGA) who have had the atrial switch operation (Mustard or Senning operation) and those who have congenitally corrected transposition of the great arteries (ccTGA), this study helping inform management of pregnancy for these women.
They used an international, prospective, observational registry of pregnant patients with cardiac disease called the European Society of Cardiology (ESC) EURObservational Research Program(EORP) Registry on Pregnancy and Cardiac disease (ROPAC). They studied all pregnancies in women with a sRV enrolled in this database from January 2007 to January 2018. Baseline characteristics were collected before pregnancy, including echocardiography where available.
The primary combined endpoint taken was major adverse cardiac event (MACE), defined as maternal death, supra-ventricular or ventricular arrhythmias needing treatment, heart failure (requiring hospital admission, new or change in treatment), aortic dissection, endocarditis, ischemic coronary event and other thromboembolic events.
5739 women were in the registry, 162 had a sRV (121 post atrial switch and 41 ccTGA). Baseline characteristics are shown in table 1:
Pregnancy outcomes were as in table 2:
Obstetric and fetal outcomes were as in table 3. 48.5% had a caesarean section and there was 1 fetal death in a woman with TGA, none in ccTGA women. There were no maternal deaths. 26 of the 79 women having a caesarean did so for cardiac reasons, such as reported heart failure (23%) or severity of cardiac disease (34%).
There were 34 premature births with 15 of these induced, 12 spontaneous and 7 unknown. This was not significantly associated with heart failure or NYHA class but was with maternal cardiac medication use (p=0.01). 17.1% of ccTGA women had low birthweight infants, 18.2% with TGA. Pre-pregnancy signs of heart failure, a RVEF <40% were predictors of MACE, being primigravida reduced the risk, as seen in figure 1:
The authors conclude that the majority of women with sRV tolerate pregnancy well with low rates of MACE and without maternal or neonatal mortality. This was a large prospective study, compared to previous smaller retrospective studies. Numbers were too small for multivariate analysis and obstetric and fetal complications seemed less than that reported in other smaller studies however. Serial echo data was only available on a limited number as a caution. The authors also note that most of the women studied had TGA with palliative Mustard or Senning operation. This will change as the arterial switch operation is the predominant operation now for this condition, with the left ventricle supporting the systemic circulation, and the proportion of woman with ccTGA and systemic right ventricle will proportionately increase.