The impact of pulmonary valve replacement on pregnancy outcomes in women with tetralogy of Fallot
Graphical abstract
Introduction
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease [1]. Notwithstanding successful repair in childhood, important residual hemodynamic sequelae, including pulmonary valve regurgitation (PR), are prevalent and have been associated with late morbidity and increased mortality [2]. Pulmonary valve replacement (PVR) is often performed to restore valve function, to facilitate RV reverse remodeling, and to mitigate risk of adverse outcomes, although published data to support the latter are relatively scarce [3,4]. Survival after primary repair of TOF (rTOF) has improved considerably over recent decades and, as a result, increasing numbers of women are reaching childbearing age. Even in the absence of cardiac disease, anticipated hemodynamic changes in the context of pregnancy are substantial. These include elevation in heart rate and augmentation of stroke volume, in order to achieve an increase in cardiac output of up to 50%, which typically peaks in the third trimester. Less is known about precise physiologic adaptations to pregnancy in women with rTOF.
While it is recognized that pregnancy is generally uncomplicated in the majority of women with rTOF, these women remain at increased risk of adverse pregnancy outcomes as compared with the general population [[5], [6], [7]], with the highest risk observed in those with RV dysfunction and/or severe PR [[8], [9], [10]]. Studies focused on imaging have demonstrated unfavorable RV remodeling as a result of pregnancy in select mothers with rTOF, particularly when the RV is known to be substantially dilated preconceptually [[11], [12], [13], [14]]. Although some advocate for proactive preconceptual PVR in women with significant PR and concomitant RV dilation in order to attenuate risk of poor pregnancy outcomes, data to support this practice are lacking. Therefore, the objective of this study was to explore the impact of pre-pregnancy PVR on clinical outcomes of pregnancy and on serial CMR measurements. We hypothesized that mothers with pre-pregnancy PVR would have superior maternal/fetal outcomes and better preservation of ventricular dimensions following pregnancy as compared with mothers without PVR.
Section snippets
Study population
Women with rTOF receiving clinical care during pregnancy in Boston (Boston Children's Hospital and Brigham and Women's Hospital: Standardized Outcomes in Cardiovascular Care [STORCC] Initiative) or in Toronto (University Health Network and Mount Sinai Hospital) with sequential pre-pregnancy (baseline) and post-pregnancy (follow-up) CMR examinations between 2001 and 2018 were retrospectively identified. Candidates were included if a baseline and a follow-up CMR study were available for analysis
Baseline demographics
Of the 29 women who met inclusion criteria, 7 underwent PVR before pregnancy (PVR+) and 22 did not (PVR-). Among the 7 women in the PVR+ group, 5 women had surgical PVR and 2 had percutaneous pulmonary valve implantation. The majority of women had an underlying diagnosis of TOF with pulmonary stenosis (n = 24) and a smaller number had more complex anatomy consisting of pulmonary atresia (n = 4) or absent pulmonary valve (n = 1). The baseline characteristics of the PVR+ and the PVR- groups are
Discussion
To our knowledge, this is the first study to explore the association between PVR status and pregnancy outcomes in women with rTOF. In keeping with previous studies, cardiovascular, obstetric, and fetal/neonatal outcomes in this cohort of mothers with rTOF were relatively favorable as compared with other forms of moderately complex congenital heart disease [19]. Furthermore, adverse events did not differ according to the presence or absence of pre-pregnancy PVR. A notable observation in this
Conclusions
Clinical outcomes in rTOF mothers and their offspring did not differ according to PVR status. In women without PVR, significant enlargement in RV size was observed following pregnancy, whereas women with pre-pregnancy PVR had no appreciable change in CMR parameters after pregnancy. Future study of a larger population with a longer follow-up may further inform clinical practice regarding indications for pre-pregnancy PVR.
Grant support
Canadian Institutes of Health Research Operating Grant (MOP 119353) to RW. VD is supported in part by a grant through the Adult Congenital Heart Association. The STORCC initiative is funded by the Brigham and Women's Watkins Discovery Award and the Barton Weinberg Family Fund to AMV.
Author statement
Each author listed on the manuscript takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
Declaration of competing interest
None.
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