Purpose: The purpose of this review was to identify the short-term and long-term outcomes of pregnancy in women suffering from congenital heart disease (CHD). Methods: An integrative review is used to identify the pregnancy outcomes in women with CHD. Data search was between 2010 and 2020 using Google Scholar, Scopus, Web of Science, Science Direct, Pub Med, Medline, CINAHL, EBSCO, Cochrane, and EBSCO host. Sixteen articles met the eligibility criteria. Results: The sixteen reviewed articles utilized descriptive retrospective and prospective design. Themes were short-term outcomes that include; cardiac maternal and obstetric outcomes, and long-term outcomes. Conclusion: Previous uncertainty about the ability of CHD patients to successfully become pregnant and deliver safely has replaced by the recognition that a large number can have excellent outcomes. However, these patients do continue to have higher cardiac, obstetric, and fetal risks than the general population. This illuminates the importance of preconception counseling and risk assessment for women suffering from CHD regarding the expected maternal and fetal outcomes. Moreover, there is a need for providing accurate and appropriate education about pregnancy and delivery options.
Congenital heart disease embraces a wide range of heart defects, ranging from benign lesions such as atrial septal defects to very complex conditions, such as transposition of the great vessels or univentricular heart. CHD was defined as “a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance” [
Traditionally, women suffering from CHD were recommended to avoid pregnancy [
The results of this ILR can help in presenting synthesized evidence, in addition to adding more were information to the body of literature about pregnancy outcomes in women suffering from CHD. Moreover, having such information can help nurses and providers in providing care and education for these patients, as the identifying of outcomes of pregnancy in CHD patients will make it easier for nurses to provide an individualized care and support based on patients’ expected outcomes. This ILR will increase the awareness of nursing administrators and educators about pregnancy outcomes in women suffering from CHD.
Recently, a growing number of research that addressed pregnancy outcomes among women suffering from CHD have emerged. However, the available evidence is built mostly on reviewing patients’ records in a single center with a small sample size [
In this review, the search was accomplished on January 2020 using different databases that include: Google Scholar, Scopus, Science Direct, Pub MED, Medline, CINAHL, EBSCO, Cochrane, ERIC uses the keywords of “congenital heart disease”, “pregnancy”, “adverse events” and “outcomes”. Initially, in a separated manner from each other, then each new search added a new keyword until including all keywords. Later, these words were searched in combination with each other. The Boolean operator “OR” and “AND” were applied separately and in combination with the keywords to expand results. However, the searching process was limited to the following inclusion criteria: 1) written in English, 2) published between 2010 and 2020 to ensure a contemporary view of pregnancy outcomes, and 3) no restriction to the research design. The experimental designs, mixed-method approach, quantitative, qualitative, and descriptive studies were considered and examined to identify outcomes. However, review papers and incomplete reports in the form of editorials, opinion pieces, and conference abstracts have excluded.
The principal investigator searched the literature. Two thousand and seven hundred articles were retrieved and the initial evaluation of their title abstracts took place. After evaluating and removing the duplicated articles, only 650 were found related to the topic of interest. Then, related articles were printed and read in full, following a secondary evaluation, 16 articles were exactly identified to cover the inclusion criteria. As a result, those sixteen articles were included in this review (see
| Author/s | Purpose | Sample size | Settings | Design | Findings |
|---|---|---|---|---|---|
| Ntiloudi et al., 2017 [ | Highlight the nature and the rate of pregnancy complications in a single-center patient population. | 117 pregnancies from 52 ACHD patients | AHEPA University Hospital, Greec | Retrospective and observational design | Of 117 pregnancies from 52 ACHD patients (age at pregnancy 6.6 - 28.3 years), 10 were therapeutically aborted. A proportion of 41.1% of the remaining 107 pregnancies were complicated either with cardiac (3.7%), obstetric (15.0%), or fetal/neonatal (11.2%) adverse events or with spontaneous abortion (17.8%). Hospitalization during pregnancy was required in 10 patients. Elective cesarean sections were initially planned for 31% of the 87 pregnancies, which were finally completed, while 8% required an emergency cesarean section, mostly for obstetric reasons. NYHA class deterioration after pregnancy was detected in 9.3% of our cohort. Modified WHO class III/IV, prior medication use, and higher body mass index (BMI) were related to cardiac complications during pregnancy or NYHA deterioration. |
| Warrick et al., 2015 [ | 1) Assess prevalence of congenital heart disease (CHD), 2) Describe outcomes of pregnancies in women with CHD, 3) Compare outcomes in women with and without CHD, and 4) Characterize neonatal outcomes in pregnancies complicated by CHD. | 117 pregnancies in 110 women with CHD | University of Colorado Hospital | Retrospective cohort study | We identified 117 pregnancies in 110 women with CHD. Parturient with CHD were more likely to have operative vaginal delivery (P = 0.0001), neonatal ICU admissions (P = 0.003), and had prolonged hospital stays. Occurrence of CHD in neonates was 6%. Moderate-to-severe valvular disease was associated with increased rates of operative vaginal delivery, early initiation of neuraxial labor analgesia, cardiac complications (including arrhythmia and use of diuretics), prolonged hospital stay, and maternal ICU admission. However most deliveries and births were uncomplicated; and there were one case each of maternal mortality and fetal death after birth. |
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| Schlichting et al., 2019 [ | Compare comorbidities and adverse cardiovascular, obstetric, and fetal events during delivery between pregnant women with and without CHDs in the United States. | Women with and without CHDs were compared in 22,881,691 deliveries. There were 17,729 deliveries to women with CHDs | A nationally representative sample of discharges from hospitals in the United States | Stratified, cross-sectional observational design | There were approximately 22,881,691 deliveries in the United States between 2008 and 2013. Among the delivery hospitalizations, 17,729 were to women with CHDs, Women with CHD had higher comorbidities in comparison with women without CHDs. cardiac events including heart failure, arrhythmia, and thromboembolic events were significantly greater among pregnant women with CHDs than pregnant women without. Women with CHDs are at an increased risk for numerous cardiovascular, obstetric, and fetal events at the time of delivery. The odds of an adverse maternal cardiac event during delivery were 2.4 to 27.6 times higher for women with CHDs compared with women without. Among the cardiac events examined, the odds of heart failure during delivery were the highest, Women with CHDs also showed significantly increased odds of other adverse obstetric events, including pre-term delivery, hemorrhage, and placental abruption. Infants born to mothers with CHDs were more likely to possess birth defects or exhibit growth restriction at birth, but less likely to experience fetal distress. These infants may experience growth restriction. |
| Karamlou et al., 2011 [ | Determined the national prevalence of GUCH parturients, their diagnostic makeup, and whether they have increased risk of peripartum complications, maternal or fetal death. | A total of 39.9 million births occurred, 26,973 (0.07%) of which were GUCH | Nationwide Inpatient Sample (NIS) is a stratified, Cross-sectional database in the US | Cross-sectional retrospective design | The GUCH parturients, especially those with VSD, have increased risk of mortality and peripartum complications compared with other age-matched women. The GUCH women had a significantly higher prevalence of all peripartum complications studied compared with non-GUCH women, including preterm delivery (10.2% vs 7.2%), induction (35.6% vs 32.5%), surgically assisted delivery (11.8% vs 7.9%), and caesarean birth (33.6% vs 27.2%) (p = 0.001 for all). Hospital length of stay was similar for both GUCH and non GUCH women and was 2.5 days. |
| Ramage et al., 2019 [ | To assess the association of ACHD and its subtypes with pregnancy, maternal, and neonatal outcomes. | 2114 women with ACHD | Population-level administrative data from the Discharge Abstract Database (DAD) in Canada | Cross-sectional retrospective design | In this large cross-sectional study of 2114 women with adult congenital heart disease in Canada, maternal and neonatal outcomes varied by subtype of maternal heart condition. Adult congenital heart disease was associated with increased odds of adverse maternal, neonatal, and pregnancy outcomes during labor and delivery hospitalization. |
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| Cauldwell et al., 2016 [ | Identify the factors associated with an increased post-partum blood loss in women with congenital heart disease (CHD). | 366 nulliparous women with CHD | Chelsea and Westminster Hospital, UK | A retrospective study design | Women with CHD are at increased risk of post-partum hemorrhage (PPH). Only women with a Fontan circulation were still significantly associated with higher blood loss at delivery. Data support vaginal delivery as the default for women with cardiac disease. As it is associated with a lower risk of post-partum hemorrhage and a better fetal outcome. |
| Yadav et al., 2018 [ | To study pregnancy outcomes in operated vs non-operated cases of congenital heart disease cases during pregnancy. | 55 patients of CHD, 29 women had no cardiac surgery and 26 had cardiac surgery | Obstetric unit of a tertiary center unit, India | Retrospective study design | There was no difference in cardiac complications, NYHA deterioration and need of cardiac drugs in the two groups. Obstetric complications and mode of delivery were also similar in the two groups. Birth weight was also similar in the both groups. Stillbirth rate and other neonatal complications were also similar in two groups. |
| Gußmann et al., 2013 [ | 1) Assess functional class during and after pregnancy. 2) Analysis a self-assessment of health, work capability and physical activity during the longest recorded possible follow-up period. | N = 103 | German Heart Centre Berlin, Germany | Retrospective study. The long-term data were acquired with a self-assessment questionnaire from each patient | Evaluate the short-term and long-term follow-up in simpler and more complex cardiac defects The short-term outcome data for obstetric, maternal cardiac and neonatal complications. Outcome data on maternal cardiac complications indicated that two-thirds of the patients had no cardiovascular events. The main cardiac symptom was the loss of functional class before and in pregnancy. Prematurity and small sizes for gestational age were the main neonatal complications. Two-thirds of the patients reported good outcomes in the self-assessments of health, work capability and physical activity in the long-term follow-up period. The health self-assessment correlated with physical activity. For the long-term outcomes, 18% of the patients with contraindications died. Only 25% of these patients answered the questionnaire. All of these patients suffered from pulmonary hypertension. |
| Kamiya, et al., 2012 [ | 1) Characterize the risk factors for pregnancy-associated cardiac events, and 2) to evaluate the long-term effects of pregnancy on the heart in women with repaired TOF. | 40 deliveries in 25 patients with repaired TOF | National Cerebral and Cardiovascular Center, Japan | Retrospective descriptive study | Cardiac Events occurred in 7 of 40 deliveries (17.5%) in 7 patients (28%): 1 case of new onset of non-sustained ventricular tachycardia (NSVT) requiring treatment, 2 of heart failure, and 4 of worsening heart failure and arrhythmias including SVT and NSVT. Obstetric Events: The mean week of delivery was shorter in patients with cardiac events than in those without cardiac events. Offspring Events: There were no neonatal deaths. The mean birth weight was 2665 g and was significantly lower for offspring of patients with cardiac events. The predictors of maternal cardiac events were a history of ablation for SVT and large CTR on chest radiograph. |
| Kampman et al., 2015 [ | To observe the incidence of cardiovascular complications in the first year postpartum and to compare cardiac function parameters pre-pregnancy and 1-year postpartum in women with CHD. | 172 pregnancies in 172 women | 8 participating centers, the Netherlands | A prospective multicenter cohort study | Cardiovascular events 1-year postpartum: Women with higher modified WHO risk classifications before pregnancy had higher cardiovascular events. Of the women with cardiovascular events postpartum, 63.6% had no history of cardiovascular events before pregnancy and 71.4% developed new arrhythmia. Women with cardiovascular events during pregnancy had a higher risk of cardiovascular events postpartum compared with women without cardiovascular events during pregnancy. The incidence of cardiovascular events 1-year postpartum was 6.4%. |
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| Pillutla et al., 2016 [ | To identify the cardiovascular and neonatal outcomes in pregnant women with high-risk CHD. | 61 pregnancies in 43 women | The Ahmanson/ University of California Los Angeles (UCLA) Adult Congenital Heart Disease Center (ACHDC), USA | Single-center retrospective analysis | Of all pregnancies, 31% were complicated by a cardiovascular event and that the most common cardiac events were either heart failure or arrhythmias. Development of any major cardiac complication during pregnancy was best predicted by antepartum maternal arrhythmia. |
| Bowater et al., 2013 [ | To examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients. | 31 pregnancies in 18 women | Queen Elizabeth Hospital, UK | Retrospective analysis | This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. There are increased risks in comparison with the general population, with a higher preterm delivery and low birth weight rates. There is also a higher cesarean section rate in this population, 39% vs. a 23% average for the hospital. |
| Assenza et al., 2013 [ | To better understand the quantitative volumetric changes associated with pregnancy in women with repaired tetralogy of Fallot (TOF), utilizing sequential cardiovascular magnetic resonance (CMR) imaging. | 30 pregnancies in 30 women, and 26 nulliparous women (Total 56) | Boston Children’s Hospital, USA | Retrospective analysis of records | Women with repaired TOF who have completed pregnancy appear to experience an accelerated rate of right ventricular remodeling, defined as an increase in end-diastolic volume; however RV systolic function does not deteriorate. |
| Ladouceur et al., 2016 [ | To describe maternal and fetal outcomes during pregnancy in patients with PAH-CHD. | 28 pregnancies in 20 women | Seven different French centers, France | Retrospective multi-center analysis | 28 pregnancies in 20 women (26 ± 6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks’ gestation (WG)), 8 abortions and 2 miscarriages. Six (33%) patients experienced severe cardiac events they had lower resting oxygen saturation. The most common cardiac complications during the complete pregnancies were heart failure (n = 4) and severe hypoxemia (n = 5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality = 5.0%). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred. |
| Silversides et al., 2018 [ | 1) Examine cardiac complications during pregnancy and their temporal trends; and 2) Derive a risk stratification index. | 1938 pregnancies were included | 2 large Canadian tertiary care hospitals. (Toronto and Vancouver) Canada | Prospective descriptive design | Cardiac complications occurred in 16% of pregnancies and were primarily related to arrhythmias and heart failure. Although the overall rates of cardiac complications during pregnancy did not change over the years, the frequency of pulmonary edema decreased. Ten predictors of maternal cardiac complications were identified: 5 general predictors (prior cardiac events or arrhythmias, poor functional class or cyanosis, high-risk valve disease/left ventricular outflow tract obstruction, systemic ventricular dysfunction, no prior cardiac interventions); 4 lesion-specific predictors (mechanical valves, high-risk aortopathies, pulmonary hypertension, coronary artery disease); and 1 delivery of care predictor (late pregnancy assessment). These 10 predictors were incorporated into a new risk index (CARPREG II [Cardiac Disease in Pregnancy Study]). |
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| Balint et al., 2010 [ | To examine the frequency and determinants of late cardiac events (LCE) in a large cohort of women with CHD. | 405 pregnancies in 318 women | A tertiary care referral hospital, Canada | Prospective descriptive design | The median follow-up time was 2.6 years after pregnancy. LCE occurred after 12% (50/405) of pregnancies. The 5-year rate of LCE was higher in women with adverse cardiac events during pregnancy than in those without. Women at highest risk for LCE were those with functional limitations, cyanosis, ventricular dysfunction and left ventricular outflow tract obstruction. Three women (0.007%) died after pregnancy. The most common LCEs were arrhythmias which occurred in 35 women. Sixty-two pregnancies (15%) were followed by therapeutic cardiac interventions; the most common indications for intervention were arrhythmia and/or congestive heart failure (40%) or deterioration in functional capacity (38%). Approximately 1/10 pregnancies were followed by a LCE. |
The quality of methods used in the included articles was assessed by using criteria for assessing the quality of quantitative studies, which was recommended by Kmet et al. (2014). The checklist comprised of 14 questions with a corresponding scoring system as follows: (Yes = 2); (Partially = 1); (No = 0); and not applicable [
The quality score of each article was calculated by summing the total score of items and dividing it by the highest possible total score after removing non-applicable items. The calculated summary score for each article can range between zero and two (see
Following Whittemore and Knafl’s (2005) methodological approach of conducting an integrative review, analysis has four phases. 1) Data reduction: represents a process that divides the primary sources into subgroups to facilitate analysis. 2) Data display: showing data as they appeared in the research matrix (data extraction sheet), to enhance comparison. 3) Data comparison: examining data to identify patterns, relationships, and themes, by which variables can be grouped together and a conceptual map can be drawn. 4) Conclusion drawing and verification: conclusion drawing occurs when the inquirer identifies patterns and relationships at a more abstract level, and generalizes the findings to populations of interest [
The reviewed articles showed that women suffering from CHD can get pregnancy safely without any adverse events [
In the reviewed studies, pregnancy in women with CHD showed a satisfactory outcomes for both of the mothers and the fetus [
Congenital heart diseases not only pose a cardiac impact on women, but it has obstetric impacts, too. Many adverse obstetric outcomes were reported in the literature; such as pregnancy-induced hypertension (PIH: systolic blood pressure (BP) ≥ 140 mm Hg or diastolic BP ≥ 90 mm Hg after 20 weeks of gestation), premature labor (labor before 37 weeks of gestation), DVT/PE, placenta previa, placenta abruption and postpartum hemorrhage (PPH) (blood loss in vaginal delivery ≥ 800 ml or in cesarean delivery (CS) ≥ 1, 500 ml) [
Postpartum hemorrhage occurs overall at a rate of 21% in CHD [
Ladouceur et al. (2017) recently assessed obstetric and fetal outcomes of 28 pregnancies among 20 women suffering from pulmonary arterial hypertension associated with congenital heart disease. Overall, 33% of pregnancies were associated with adverse cardiac events with a severe reduction in resting oxygen saturation [
The risk of fetal and neonatal complications is higher among pregnant women with CHD as compared to healthy women [
Cardiac events were reported as new onset or worsening of arrhythmia requiring treatment, heart failure which is diagnosed through the following criteria (a decline in New York Heart Association (NYHA) class, pulmonary congestion confirmed by chest radiography, the requirement for diuretic therapy), endocarditis, or thromboembolic events during pregnancy to one month after delivery [
Arrhythmia and heart failure are the most commonly occurring cardiac adverse event during pregnancy [
In a contradictory to the traditional thinking that pregnancy outcomes will be better in women with CHD who underwent a cardiac repair surgery; Yadav and his colleagues (2018) found no statistically significant differences in the cardiac, maternal and neonatal outcomes between the operated CHD and non-operated CHD population who pose the same baseline characteristics [
Limited studies have assessed the effect of pregnancy on long-term outcomes in women suffering from CHD. To assess the cardiac outcomes in women suffering from CHD after pregnancy, Balint and colleagues (2010) conducted a prospective study recruiting a consecutive sample of pregnant women who were referred for follow up or consultation in a cardiac center. The data were collected throughout 1995-2007, antenatal, perinatal and postnatal data were collected from these pregnant women. At the first antenatal visit, comprehensive examinations and investigations to assess the cardiac conditions were performed as baseline data. Also, during pregnancy; any cardiac complications were recorded, and any obstetric or fetal complications were recorded too; to be studied as predictors with any late cardiac complications after pregnancy completion. The late cardiac complications were defined as: sudden cardiac death, pulmonary edema, cardiac arrhythmias (Tachy/Bradyarrhythmias), stroke or transient ischemic attacks [
To follow the long-term outcomes one-year post-delivery, Kampman et al. (2015) conducted a prospective multi-center cohort study. They found that the complications after one year of delivery are rare; the overall incidence rate was 6.4%. The occurrence of post-partum complications was higher in women with a mechanical prosthetic valve, in women who use cardiac medications, pre-pregnancy, especially beta-blockers and anti-coagulants, and in women with a history of arrhythmias [
Gußmann et al. (2013) conducted an analysis of a self-assessment of health, work capability, and physical activity during the longest possible follow-up period; the follow-up period reached up to eleven years. In this analysis, 107 women were followed, two-thirds of the patients reported good outcomes in the self-assessments of health, work capability and physical activity in the long-term follow-up period. The health self-assessment correlated positively with the physical activity. For the long-term outcomes, eighteen percent of the women with contraindications to pregnancy died, and all patients (with contraindications to pregnancy) who responded to the follow up questionnaire developed pulmonary hypertension. The results of this study suggest to continue in the advisement of contradictory of pregnancy considering not only the short-term outcomes, but also the long-term outcomes [
In this review, the following themes were identified: short-term outcomes that are divided into three sub-categories: maternal, cardiac, and fetal outcomes [
The calculated scores for the rigor of the research articles ranged between 0.60 and 0.73, with a mean average of 0.65. In addition, the minimum score of the included studies was 0.60 indicating adequate rigor of the included studies; therefore, no studies were excluded based on the calculated scores.
There are some limitations of the reviewed articles. Out of the 16 reviewed articles, thirteen research articles were conducted in a retrospective manner over a variable period. Of them, eleven had taken place in a single center or hospital to evaluate the cardiac and fetal outcomes for women suffering from CHD [
As most of the reviewed articles are conducted in retrospective or so-called survivor cohorts, there is a need for using more robust designs to get more valid results. Moreover, for accurate estimations of serious and relatively rare complications a large-scale (multicenter and international) prospective registration remains needed.
Several limitations of the present ILR need to be discussed. The quality of the review depends on the design of the articles included. The study designs mostly based on reviewing the patients’ records retrospectively, and few prospective studies to follow the long-term outcomes; therefore, the results need to be judged with caution. Selection bias is introduced by excluding articles based on the earlier-mentioned criteria. Underreporting of complications may also be an important problem.
The last decades show a progressive advancement in the management of congenital heart disease; that results in large numbers of women with repaired CHD, who are able now to successfully become pregnant and deliver healthy offspring. Previous uncertainty about the ability of these patients to successfully become pregnant and deliver safely has been replaced by the recognition that a large number can have excellent outcomes. However, despite this, these patients do continue to have higher cardiac, obstetric, and fetal risks than the general population. This highlights the importance of preconception counseling regarding the increased risk of fetal congenital heart disease with affected mothers and referrals for genetic assessment and counseling where appropriate, moreover, providing the appropriate and accurate advice for women about the potential adverse events that they may encounter on short-term and long-term basis.
The authors declare no conflicts of interest regarding the publication of this paper.
Al Obieat, H., Khalaf, I. and Al Momani, M.Sh. (2020) The Outcomes of Pregnancy with Congenital Heart Disease: An Integrative Literature Review. Open Journal of Nursing, 10, 473-489. https://doi.org/10.4236/ojn.2020.105033