Association between pregnancy and long-term cardiac outcomes in individuals with congenital heart disease.

BACKGROUND: As early life interventions for congenital heart disease improve, more patients are living to adulthood and are considering pregnancy. Scoring and classification systems predict the maternal cardiovascular risk of pregnancy in the context of congenital heart disease, but these scoring systems do not assess the potential subsequent risks following pregnancy. Data on the long-term cardiac outcomes after pregnancy are unknown for most lesion types. This limits the ability of healthcare practitioners to thoroughly counsel patients who are considering pregnancy in the setting of congenital heart disease. OBJECTIVE: We aimed to evaluate the association between pregnancy and the subsequent long-term cardiovascular health of individuals with congenital heart disease. STUDY DESIGN: This was a retrospective longitudinal cohort study of individuals identifying as female who were receiving care in two adult congenital heart disease centers from 2014 to 2019. Patient data were abstracted longitudinally from a patient age of 15 years (or from the time of entry into the healthcare system) to the conclusion of the study, death, or exit from the healthcare system. The primary endpoint, a composite adverse cardiac outcome (death, stroke, heart failure, unanticipated cardiac surgery, or a requirement for a catheterized procedure), was compared between parous (at least one pregnancy >20 weeks' gestation) and nulliparous individuals. By accounting for differences in the follow-up, the effect of pregnancy was estimated based on the time to the composite adverse outcome in a proportional hazards regression model adjusted for the World Health Organization class, baseline cardiac medications, and number of previous sternotomies. Participants were also categorized according to their lesion type, including septal defects (ventricular septal defects, atrial septal defects, atrioventricular septal defects, or atrioventricular canal defects), right-sided valvular lesions, left-sided valvular lesions, complex cardiac anomalies, and aortopathies, to evaluate if there is a differential effect of pregnancy on the primary outcome when adjusting for lesion type in a sensitivity analysis. RESULTS: Overall, 711 individuals were eligible for inclusion; 209 were parous and 502 nulliparous. People were classified according to the World Health Organization classification system with 86 (12.3%) being classified as class I, 76 (10.9%) being classified as class II, 272 (38.9%) being classified as class II to III, 155 (22.1%) being classified as class III, and 26 (3.7%) being classified as class IV. Aortic stenosis, bicuspid aortic valve, dilated ascending aorta or aortic root, aortic regurgitation, and pulmonary insufficiency were more common in parous individuals, whereas dextro-transposition of the great arteries, Turner syndrome, hypoplastic right heart, left superior vena cava, and other cardiac diagnoses were more common in nulliparous individuals. In multivariable modeling, pregnancy was associated with the composite adverse cardiac outcome (36.4%% vs 26.1%%; hazard ratio, 1.83; 95% confidence interval, 1.25-2.66). Parous individuals were more likely to have unanticipated cardiac surgery (28.2% vs 18.1%; P=.003). No other individual components of the primary outcome were statistically different between parous and nulliparous individuals in cross-sectional comparisons. The association between pregnancy and the primary outcome was similar in a sensitivity analysis that adjusted for cardiac lesion type (hazard ratio, 1.61; 95% confidence interval, 1.10-2.36). CONCLUSION: Among individuals with congenital heart disease, pregnancy was associated with an increase in subsequent long-term adverse cardiac outcomes. These data may inform counseling of individuals with congenital heart disease who are considering pregnancy.

Transition in cardiology 2: Maternal and fetal congenital heart disease.

The number of women with congenital heart disease (CHD) reaching reproductive age has been increasing. Many women with CHDs are desirous of pregnancy, but they face issues regarding preconception, antepartum, and postpartum management. On the other hand, the fetal diagnosis of CHD has improved with advances in the technique and equipment for fetal echocardiography. Recently, experiences with fetal intervention have been reported in patients with severe CHD, such as critical aortic stenosis. Nevertheless, some types of CHD are challenge to diagnose prenatally, resulting in adverse outcomes. Medical care is part of the transitional care for women and fetuses with CHD during the perinatal period. Pre-conceptional and prenatal counseling play an important role in transitional care. Sex and reproductive education need to be performed as early as possible. We herein review the current status, important issues to be resolved, and the future of maternal and fetal CHD to relevant caregivers.

The Utility of Screening Fetal Echocardiograms Following Normal Level II Ultrasounds in Fetuses with Maternal Congenital Heart Disease.

INTRODUCTION: Fetal echocardiograms (F-echo) are recommended in all pregnancies when maternal congenital heart disease (CHD) is present, even if there was a prior level II ultrasound (LII-US) that was normal. The goal of this study was to evaluate if any diagnosis of a critical CHD was missed in a fetus with maternal CHD who had a normal LII-US. METHODS: A retrospective chart review of all F-echoes where the indication was maternal CHD between 1/1/2015 to 12/31/2022 was performed. Fetuses were included if they had a LII-US that was read as normal and had an F-echo. Critical CHD was defined as CHD requiring catheterization or surgical intervention < 1 month of age. RESULTS: A total of 296 F-echoes on fetuses with maternal CHD were evaluated, of which 175 met inclusion criteria. LII-US was performed at 19.8 ± 2.9 weeks gestational age and F-echo was performed at 24.2 ± 2.8 weeks gestational age. No patient with a normal LII-US had a diagnosis of a critical CHD by F-echo (negative predictive value = 100%). Evaluating those patients that had a negative LII-US, ten patients were diagnosed with non-critical CHD postnatally (negative predictive value = 94.3%). F-echo correctly diagnosed two of the ten missed LII-US CHD. CONCLUSIONS: Critical CHD was not missed with a normal LII-US in this at risk population. F-echo also missed the majority of CHD when a LII-US was read as normal. A cost-benefit analysis of screening F-echo in fetuses with maternal CHD should be conducted if a normal LII-US has been performed.

Impact of pregnancy on the cardiac health of women with prior surgeries for pulmonary valve anomalies.

OBJECTIVE: We assessed the impact of pregnancy on long-term cardiac outcomes in women with prior surgery for congenital pulmonary valve anomalies. STUDY DESIGN: Data on all reproductive age women with prior pulmonary valve repair or replacement, cared for at a tertiary institution over a 10-year period, were analyzed. Kaplan-Meier curves and proportional hazards models were estimated to assess the impact of pregnancy and multiparity on a composite long-term adverse outcome defined as death, heart failure, or unanticipated cardiac surgery. Peripartum cardiac complications were also assessed. RESULTS: Thirty-three parous and 20 nulliparous, nonpregnant controls with primary pulmonary valve replacement or repair were identified. Among the parous women, there were 95 pregnancies (median, 3.0; 1-10) resulting in 81 live births. Peripartum cardiac complications occurred in 28 (29.8%; 95% confidence interval, 20.4-39.2) of the pregnancies. A composite adverse long-term cardiac outcome occurred in 17 of 33 parous women, over 417 person-years (4 per 100 person-years) and 1 of 20 nulliparous women over 258 person-years (0.4 per 100 person-years); women with pregnancies were more likely at any point in time to have a composite long-term adverse cardiac outcome compared with nulliparous controls. Women with 2 or more pregnancies were more likely to have a composite adverse cardiac outcome than those with less than 2 pregnancies (hazard ratio, 8.8; 95% confidence interval, 1.5-50.3). CONCLUSION: Peripartum cardiac complications are common in women with prior pulmonary valve repair or replacement. Pregnancy appears to increase the risk of long-term adverse cardiac outcomes in these patients when compared with nulliparous controls.

Perinatal outcomes and long-term offspring cardiovascular morbidity of women with congenital heart disease.

OBJECTIVE: Due to advances in medical care available for patients with congenital heart disease, these patients now have a longer life span. As a result, the number of pregnancies of mothers with congenital heart disease has increased. The purpose of the current study was to evaluate perinatal outcomes of women with congenital heart disease and to examine long-term cardiovascular morbidity of offspring to mothers with congenital heart disease. MATERIALS AND METHODS: A population-based cohort analysis was performed including all singleton deliveries occurring between years 1991-2014 at Soroka University Medical Center. The primary exposure was defined as maternal congenital heart disease. Short-term perinatal outcomes as well as long-term cardiovascular related hospitalizations of the offspring up to the age of 18 years were compared between women with and without congenital heart disease. To compare perinatal outcomes between the two study groups, we conducted a Generalized Estimation Equation (GEE) model accounting for maternal age and multiple occurrence in the cohort, parity, fertility treatment, smoking and induction of labor. To compare cumulative long-term cardiovascular morbidity, we compared cardiovascular hospitalization incidence of offspring of mothers with and without congenital heart disease using Kaplan-Meier survival curve and Weibull regression analysis to control for maternal age, diabetes, smoking, hypertension, birth weight, and cesarean delivery. RESULTS: During the study period 243,313 deliveries met the inclusion criteria, of which 369 were of mothers with congenital heart disease. Maternal congenital heart disease was found to be an independent risk factor for low birth weight (adjusted OR = 1.5 CI 95 % 1.06-2.32, p=0.022) and cesarean delivery (adjusted OR = 1.4 CI 95 % 1.09-1.86, p = 0.008). Mothers with congenital heart disease also had higher risk for placental abruption, but it was not statistically significant (adjusted OR = 2.3 CI 95 % 0.87-6.16, p = 0.09). Long-term cardiovascular morbidity was comparable between the groups (Log rank p = 0.251; adjusted HR = 1.6 CI 95 % 0.62-4.42, p = 0.313). CONCLUSION: In conclusion, pregnancies of women with congenital heart disease are at an increased risk for adverse short-term perinatal outcomes, such as low birth weight and cesarean delivery. However, we did not find an increase in long-term cardiovascular morbidity among offspring of these women; still this association cannot be ruled out.