Pregnancy in women with a systemic right ventricle after surgically and congenitally corrected transposition of the great arteries

https://doi.org/10.1016/j.ejogrb.2010.12.021Get rights and content

Abstract

Objective

To determine maternal and neonatal outcomes in women with a systemic right ventricle (RV).

Study design

A retrospective (historical) cohort study of maternal and neonatal outcomes at a tertiary referral academic obstetric unit (Chelsea and Westminster Hospital, London).

Results

Nineteen pregnancies in 14 women with a systemic RV were compared with 76 controls. There were no maternal deaths. In the study group cardiac complications occurred in six (32%) pregnancies. Obstetric complications occurred in four (21%) case pregnancies, not significantly higher than in the control group. The rate of neonatal complication was significantly higher in the study population with neonatal complications in 12 (63%) case pregnancies compared with 13 (17%) control pregnancies. The median birthweight centile was 9 in the study population, significantly lower than the control population.

Conclusions

Our cohort study demonstrates high maternal and neonatal morbidity and low birthweight in the presence of a systemic RV. Cardiac complications were more common in women with RV dysfunction and arrhythmias prior to pregnancy. Preconception counselling and tertiary care during pregnancy for these patients is highly advisable.

Introduction

In both dextro-transposition of the great arteries (D-TGA) following atrial repair, and levo-transposition of the great arteries (L-TGA), often referred to as congenitally corrected transposition of the great arteries, the right ventricle (RV) supports the systemic circulation. This puts the RV under considerable strain and eventually results in cardiac failure.

D-TGA accounts for 5–7% of all congenital heart malformations. In D-TGA the aorta arises from the morphological RV, and the pulmonary artery from the morphological left ventricle. The atrial switch (Mustard or Senning) procedure introduced in the late 1950s enables survival beyond infancy. In this procedure, venous blood flow is re-directed within the atrial compartment leaving the morphological RV and tricuspid valve supporting the systemic circulation. In contrast, L-TGA is a rare cardiac anomaly in which both the atrioventricular valves and the great arteries are transposed. Systemic venous return enters the right atrium and passes into the left ventricle, which then ejects it into the pulmonary artery. Pulmonary venous return enters the left atrium which is connected to the right ventricle which then supports the systemic circulation. Long-term survival rates are good for both corrected D-TGA and L-TGA and a substantial cohort of women have now reached reproductive maturity and are embarking on pregnancy [1]. Dysfunction of the systemic RV and tricuspid regurgitation are, however, encountered in 30–50% of patients at a relatively early age [2].

During pregnancy and childbirth the systemic ventricle is exposed to dramatic alterations in cardiovascular physiology. The systemic vascular resistance falls and cardiac output increases secondary to an increased heart rate and stroke volume [3]. Systolic function in the normal heart is preserved throughout pregnancy but diminishes near term due to a reduced preload and, possibly, decreased contractility [4]. A systemic RV is predisposed to dysfunction and may not adequately respond to the demands placed upon it during pregnancy. Potentially RV dysfunction in this setting can be irreversible [5], and thus pregnancy in women with a systemic RV carries a risk of both short- and long-term maternal and neonatal complications [6], [7], [8], [9], [10]. To define these risks further we reviewed our own series, assessing pregnancy outcomes and current management.

Section snippets

Materials and methods

The study cohort consisted of all women with a diagnosis of D-TGA or L-TGA who delivered at Chelsea and Westminster Hospital between January 1996 and October 2010. Only those women whose pregnancies progressed to 24 weeks gestation were included in the study. To allow comparison with our overall population, controlling for changes in demographics and practice over time, data were also collected from the Ciconia Maternity Information System on the two women who delivered immediately before and

Results

Baseline maternal characteristics for cases and controls are shown in Table 1. A total of 19 pregnancies in 14 women were identified (cases) and compared with 76 controls. The women in the study population were younger than the controls (mean age at booking 29.1 (SD 5.67) range 21–40, and 31.6 (SD 5.86) range 16–46 years, respectively) and there was no statistically significant difference in parity (p = 0.5). The rates of smoking and illicit drug use were similar between the two groups.

Eleven

Comments

With advances in paediatric cardiology and cardiac surgery, women with complex congenital heart disease are reaching their childbearing years and embarking on pregnancy. Although arterial switch is currently the mainstay surgical procedure, a substantial cohort of D-TGA patients following atrial repair are still to enter their childbearing years. Pregnancy imposes a significant physiological burden on an already compromised heart, affecting both maternal and neonatal outcomes. In our series

Conclusion

Our cohort study demonstrates a high maternal and neonatal morbidity and low birthweight in the presence of a systemic RV. Cardiac complications were seen in women with pre-existing RV dysfunction and a history of arrhythmias. Preconception counselling and tertiary care during pregnancy for these patients are highly advisable.

References (18)

There are more references available in the full text version of this article.

Cited by (27)

  • Impact of pregnancy on natural history of systemic right ventricle in women with transposition of the great arteries

    2022, International Journal of Cardiology
    Citation Excerpt :

    Previous small series showed more favorable outcome for women with CCTGA compared to those with TGA [30–33] However, earlier data from international prospective registry of pregnant women with SRV found no significant differences between two group, except for hospital admission rate for a cardiac reason that occurred more often in women with CCTGA than TGA (19.5% vs 6.6%, p = 0.03) [24]. Serious obstetric and neonatal adverse events are common in pregnant women with SRV [16,23,30], although recent data report most favorable outcomes than before [24]. In the older series women with SRV experienced up to 2-fold increase of spontaneous miscarriage compared to general population [16].

  • Successful pregnancies after transvenous cardiac resynchronization therapy in a woman with congenitally corrected transposition of the great arteries

    2017, Egyptian Heart Journal
    Citation Excerpt :

    Most women with ccTGA reach childbearing age. The morphologic right ventricle (RV) is not designed to support systemic circulation and particularly increased cardiac output during pregnancy and is predisposed to dysfunction.2–4 Cardiac resynchronization therapy (CRT) is supposed to stabilize if not to improve cardiac function in these patients.5–7

  • High-Risk Cardiac Disease in Pregnancy: Part II

    2016, Journal of the American College of Cardiology
    Citation Excerpt :

    Cardiac death appears to be rare. Other complications are New York Heart Association (NYHA) functional class deterioration, RV dysfunction and worsening of tricuspid regurgitation (TR) that may be persistent after pregnancy, high rates of prematurity (25% to 50%) and small for gestational age (up to 50%), as well as fetal and neonatal death (18–24). Similar complications are described in women with CCTGA, but the complication rates are reported to be lower (25,26).

  • Pregnancy and long-term cardiovascular outcomes in women with congenitally corrected transposition of the great arteries

    2014, International Journal of Gynecology and Obstetrics
    Citation Excerpt :

    The authors identified 15 (10.3%) pregnancies in women with a systemic RV but only 3 (2%) in women with ccTGA. Similarly, Gelson et al. [9] combined data on pregnancies in women with repaired d-TGA (n = 11) and in women with ccTGA (n = 3), and compared them with data from a control group. In that study, all cardiac complications that occurred in the systemic RV group related to women with d-TGA who had undergone the Mustard procedure [9].

  • Preconception counselling of the patient with systemic ventricular dysfunction

    2014, Progress in Pediatric Cardiology
    Citation Excerpt :

    Neonatal complications are also common. In a small descriptive study of 19 patients with a systemic right ventricle 60% had a neonatal complication — often related to preterm delivery or growth restriction [18]. When assessing these patients prior to pregnancy all of the other associated issues need to be reviewed — this includes baffle patency, baffle leaks and pulmonary vascular resistance.

View all citing articles on Scopus
View full text