Optimizing Prepregnancy Cardiovascular Health to Improve Outcomes in Pregnant and Postpartum Individuals and Offspring: A Scientific Statement From the American Heart Association.

This scientific statement summarizes the available preclinical, epidemiological, and clinical trial evidence that supports the contributions of prepregnancy (and interpregnancy) cardiovascular health to risk of adverse pregnancy outcomes and cardiovascular disease in birthing individuals and offspring. Unfavorable cardiovascular health, as originally defined by the American Heart Association in 2010 and revised in 2022, is prevalent in reproductive-aged individuals. Significant disparities exist in ideal cardiovascular health by race and ethnicity, socioeconomic status, and geography. Because the biological processes leading to adverse pregnancy outcomes begin before conception, interventions focused only during pregnancy may have limited impact on both the pregnant individual and offspring. Therefore, focused attention on the prepregnancy period as a critical life period for optimization of cardiovascular health is needed. This scientific statement applies a life course and intergenerational framework to measure, modify, and monitor prepregnancy cardiovascular health. All clinicians who interact with pregnancy-capable individuals can emphasize optimization of cardiovascular health beginning early in childhood. Clinical trials are needed to investigate prepregnancy interventions to comprehensively target cardiovascular health. Beyond individual-level interventions, community-level interventions must include and engage key stakeholders (eg, community leaders, birthing individuals, families) and target a broad range of antecedent psychosocial and social determinants. In addition, policy-level changes are needed to dismantle structural racism and to improve equitable and high-quality health care delivery because many reproductive-aged individuals have inadequate, fragmented health care before and after pregnancy and between pregnancies (interpregnancy). Leveraging these opportunities to target cardiovascular health has the potential to improve health across the life course and for subsequent generations.

Delivery Timing and Associated Outcomes in Pregnancies With Maternal Congenital Heart Disease at Term.

Background Current recommendations for delivery timing of pregnant persons with congenital heart disease (CHD) are based on expert opinion. Justification for early-term birth is based on the theoretical concern of increased cardiovascular stress. The objective was to evaluate whether early-term birth with maternal CHD is associated with lower adverse maternal or neonatal outcomes. Methods and Results This is a retrospective cohort study of pregnant persons with CHD who delivered a singleton after 37 0/7 weeks gestation at a quaternary care center with a multidisciplinary cardio-obstetrics care team between 2013 and 2021. Patients were categorized as early-term (37 0/7 to 38 6/7 weeks) or full-term (≥39 0/7) births and compared. Multivariable logistic regression was conducted to calculate the adjusted odds ratio for the primary outcomes. The primary outcomes were composite adverse cardiovascular, maternal obstetric, and adverse neonatal outcome. Of 110 pregnancies delivering at term, 55 delivered early-term and 55 delivered full-term. Development of adverse cardiovascular and maternal obstetric outcome was not significantly different by delivery timing. The rate of composite adverse neonatal outcomes was significantly higher in early-term births (36% versus 5%, P<0.01). After adjusting for confounding variables, early-term birth remained associated with a significantly increased risk of adverse neonatal outcomes (adjusted odds ratio 11.55 [95% CI, 2.59-51.58]). Conclusions Early-term birth for pregnancies with maternal CHD was associated with an increased risk of adverse neonatal outcomes, without an accompanying decreased rate in adverse cardiovascular or obstetric outcomes. In the absence of maternal or fetal indications for early birth, induction of labor before 39 weeks for pregnancies with maternal CHD should be reserved for routine obstetrical indications.

Bleeding and thrombotic risk in pregnant women with Fontan physiology.

BACKGROUND/OBJECTIVES: Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan. METHODS: We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors. RESULTS: We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33±5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096). CONCLUSIONS: Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy.

The landscape of cardiovascular care in pediatric cancer patients and survivors: a survey by the ACC Pediatric Cardio-Oncology Work Group.

OBJECTIVE: To enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors. STUDY DESIGN: A 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC. RESULTS: Sixty-five individuals, all self-identified as physicians, responded to the survey. Most respondents (n = 58,89%) indicated childhood cancer patients are regularly screened prior to and during cancer therapy at their centers, predominantly by electrocardiogram (75%), standard echocardiogram (58%) and advanced echocardiogram (50%) (i.e. strain, stress echo). Evaluation by a cardiologist prior to/during therapy was reported by only 8(12%) respondents, as compared to post-therapy which was reported by 28 (43%, p < 0.01). The most common indications for referral to cardiology at pediatric centers were abnormal test results (n = 31,48%) and history of chemotherapy exposure (n = 27,42%). Of note, during post-treatment counseling, common cardiovascular risk-factors like blood pressure (31,48%), lipid control (22,34%), obesity & smoking (30,46%) and diet/exercise/weight loss (30,46%) were addressed by fewer respondents than was LV function (72%). CONCLUSIONS: The survey data demonstrates that pediatric cancer patients are being screened by EKG and/or imaging prior to/during therapy at most centers. Our data, however, highlight the potential for greater involvement of a cardiovascular specialist for pre-treatment evaluation process, and for more systematic cardiac risk factor counseling in posttreatment cancer survivors.

Maternal Fontan procedure is a predictor of a small-for-gestational-age neonate: a 10-year retrospective study.

BACKGROUND: Women with single ventricle cardiac physiologic condition who have undergone Fontan procedures are surviving well into reproductive age and historically have been discouraged from pregnancy, despite the paucity of data regarding maternal and neonatal outcomes. OBJECTIVE: Our primary objective was to investigate, in a large cohort, the maternal and neonatal outcomes of pregnant women who have undergone the Fontan procedure and to understand maternal and neonatal sequelae of their pregnancies. STUDY DESIGN: This single-center retrospective cohort study involves pregnant women with a Fontan palliation who delivered at UCLA Medical Center over a 10-year period (2007-2017). All pregnancies were evaluated for differences in maternal and neonatal characteristics. RESULTS: We identified 37 distinct pregnancies in 24 women with a Fontan procedure. The physiologic pregnancy-related increase in cardiac output is blunted substantially in Fontan circulation. Third-trimester cardiac index positively correlated to birthweight z-score (R2=0.48; P=.038) but not to small for gestational age (R2=0.13; P=.339). The most common cardiac complications in pregnancies of >24 weeks gestation were sustained arrhythmia (37.5%) and decompensated heart failure (21%). The 37 pregnancies comprised 25 live births (67.6%), 1 fetal death (2.7%), 9 spontaneous abortions (24%), and 2 pregnancy terminations (5.4%). Of the live births, 60% were preterm at an average gestational age of 34.9±3.7 weeks. Newborn infants were delivered via cesarean in 53%, operative vaginal delivery in 28%, and spontaneous vaginal delivery in 20%. Forty percent of neonates were born small (<10th percentile) for gestational age; 44.0% of all neonates were admitted to the neonatal intensive care unit. CONCLUSION: Women with a single ventricle and Fontan circulation can have a successful pregnancy, although they are at increased risk for arrhythmias and heart failure. The decreased cardiac reserve in these pregnancies blunts the normal increase in maternal cardiac output, which is associated with preterm delivery and small-for-gestational-age neonates. Further studies are needed to determine to what extent the impaired rise in maternal cardiac output reduces uteroplacental perfusion, placental exchange, fetal growth, and onset of parturition.

Family Planning and Pregnancy Management in Adults with Congenital Heart Disease.

With advances in congenital heart disease management, there are an increasing number of women reaching reproductive age. Pregnancy results in a surge of hormones and increased demands on both the cardiovascular (CV) and respiratory systems. Depending on the heart defect and the treatments the mother has undergone, these hemodynamic changes can result in an increased risk of maternal CV events and an increased risk of fetal morbidity and mortality. Thus, it is important to have a comprehensive approach to adult congenital heart disease patients involving pre-pregnancy planning in addition to diligent peri- and post-partum care.

Contraceptive Practices of Women With Complex Congenital Heart Disease.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking.

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association.

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.

Cardiovascular and Neonatal Outcomes in Pregnant Women With High-Risk Congenital Heart Disease.

Congenital heart disease (CHD) increases the risk of adverse maternal and neonatal outcomes. However, previous studies have included mainly women with low-risk features. A single-center, retrospective analysis of pregnant women with CHD was performed. Inclusion criteria were the following high-risk congenital lesions and co-morbidities: maternal cyanosis; New York Heart Association (NHYA) functional class >II; severe ventricular dysfunction; maternal arrhythmia, single ventricle (SV) physiology, severe left-sided heart obstruction and severe pulmonary arterial hypertension. Multivariate analyses for predictors of adverse maternal cardiovascular and neonatal outcomes were performed. Forty-three women reported 61 pregnancies. There were no maternal or neonatal deaths. Maternal cardiac (31%) and neonatal (54%) complications were frequent. The most frequent cardiac events were pulmonary edema, arrhythmia, and reduced NYHA class. Previous arrhythmia conferred a 12-fold increase in the odds of experiencing at least one major cardiac complication. Maternal SV physiology was an independent risk factor for low birth weight, risk of neonatal intensive care unit admission and lower gestational age. Maternal cyanosis and severe pulmonary arterial hypertension also predicted adverse neonatal outcomes. In conclusion, mothers without antepartum arrhythmia or functional incapacity are unlikely to experience arrhythmias or a decrease in NYHA class during pregnancy. In addition, SV physiology is a robust predictor of neonatal complications. Antepartum counseling and assessment of maternal fitness are crucial for the woman with CHD.

Contraception Practices and Pregnancy Outcome in Patients after Fontan Operation.

OBJECTIVE: The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation. DESIGN: We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study. RESULTS: Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect). CONCLUSIONS: Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population.

Development of an international research agenda for adult congenital heart disease nursing.

BACKGROUND: Since the population of adults with congenital heart disease (CHD) is growing, the role of nurse specialists is expanding. In order to advance ACHD nursing, the establishment of an international nursing research agenda is recommended. We aimed to investigate research priorities as perceived by nurse specialists and researchers in ACHD. METHODS: We applied a sequential quan-qual design. In the quantitative phase, a two-round Delphi study was conducted, in which 37 nurse specialists and nurse researchers in ACHD care participated. Respondents assessed the level of priority of 21 research topics using a 9-point rating scale (1 = no priority at all; 9 = very high priority). In the qualitative phase, semi-structured interviews were performed with six selected Delphi panelists, to scrutinize pending research questions. RESULTS: This study revealed that priority should be given to studies investigating knowledge and education of patients, outcomes of Advanced Practice Nursing, quality of life, transfer and transition, and illness experiences and psychosocial issues in adults with CHD. A low priority was given to post-operative pain, sexual functioning, transplantation in ACHD, and health care costs and utilization. Agreement about the level of priority was obtained for 14 out of 21 research topics. CONCLUSION: Based on this study, we could develop an international research agenda for ACHD. Researchers ought to focus on these areas of highest priority, in order to expand and strengthen the body of knowledge in ACHD nursing.

Eisenmenger syndrome: A clinical review.

Eisenmenger syndrome is a medical condition, characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Patients with Eisenmenger syndrome are not very prevalent in cardiovascular nurses' practice. However, nurses need to have some basic knowledge about the syndrome, in case they have to care for such a patient in their clinical practice. In this article, we describe the epidemiology, outcome, pathophysiology, clinical presentation, medical management and counseling issues of this condition.

Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of America and Europe.

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

Menstrual cycle and its disorders in women with congenital heart disease.

OBJECTIVES: To investigate the age at menarche, the prevalence of menstrual cycle (interval) disorders, and determinants in women with congenital heart disease (CHD). DESIGN: Using two CHD registries, 1802 (82%) of the 2196 women with CHD contacted (aged 18-58 years) provided written informed consent. After exclusion of patients with genetic disorders known to be associated with menstrual cycle disorders, 1593 eligible patients remained. Interviews by telephone and reviews of medical records were conducted. RESULTS: Overall, the age at menarche was slightly increased in women with CHD (13.3 vs. 13.1 years in the general population), mainly attributable to an increased prevalence of primary amenorrhea (n = 147; 9.2%). Other menstrual cycle disorders were documented: secondary amenorrhea (n = 181, 11.4%), polymenorrhea (n = 103, 6.5%), oligomenorrhea (n = 90, 5.6%), and menorrhagia (n = 117, 6.5%). The occurrence of these disorders also depended on the presence of cyanotic heart disease, surgical status, the number of surgical interventions, and the severity of CHD. DISCUSSION: Menstrual cycle disturbances, in particular primary amenorrhea, were frequently observed in this population. Patients with complex (cyanotic) heart disease needing repeated surgical interventions prior to menarche are especially at risk.

Pregnancy outcomes after atrial repair for transposition of the great arteries.

Increasingly, women born with complete transposition of the great arteries who have undergone atrial repair by either the Senning or the Mustard procedure are reaching childbearing age. This study reports on pregnancy outcomes after the atrial repair of transposition of the great arteries. Record review and standardized questionnaires were used to ascertain the outcomes of 70 pregnancies reported in 40 women (36 Mustard procedures, 4 Senning procedures). Of the 70 pregnancies, 54 resulted in 56 live births, 10 in miscarriages, and 6 in therapeutic abortions. At pregnancy, 31 women were in New York Heart Association class I, 8 were in class II, and 1 was in class III. Thirty-nine percent of the infants were delivered prematurely and weighed 2,714 +/- 709 g; 28% were delivered by cesarean section, 8 for cardiac indications. Maternal complications included arrhythmias in 5 women and hemoptysis in 2 women. Heart failure occurred in 6 women, developing during the second and third trimesters. Postpartum cardiac events developed 2 to 9 days postpartum: heart failure in 5 women, atrial fibrillation in 1 woman, and decreased oxygen saturation due to a new atrial baffle leak in 1 woman. Severe right ventricular (RV) failure led to cardiac transplantation after delivery in 1 woman; another developed heart failure and then died suddenly 1 month after delivery. There was 1 late death, 4 years after the patient's last pregnancy. In conclusion, pregnancy after atrial repair carries a moderate degree of risk and should be undertaken with caution.

Gynecological health of females with congenital heart disease.

Because of major advances in diagnostic and surgical methods, females with congenital heart disease (CHD) now survive into and beyond their reproductive years. Management of pregnancy in this patient population is well described, but gynecologic management such as menstruation, contraception and menopause have received scanty attention. Accordingly, the gynecologic health issues confronting these patients are described. Menstrual patterns in acyanotic females with CHD are similar to the general population, but cyanotic females have menstrual irregularities including amenorrhea, which implies anovulation and an increased risk of uterine carcinoma. Anticoagulants predispose to heavy vaginal bleeding and corpus luteum rupture. Contraceptives must be selected according to individual patient profiles. Hormone replacement therapy is warranted for relief of menopausal symptoms as in the general population and should be relatively safe because estrogen dose is low.

Contraception for the adolescent and young adult with congenital heart disease.

Increasing numbers of females born with congenital heart disease are reaching childbearing age. Practitioners involved in the management of the adolescents or adults must be aware not only of the risks of pregnancy associated with varying types of CHD but with other issues involving the reproductive cycle to provide appropriate care, counseling, and education.