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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.

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. publisher: Elsevier articletitle: Contraceptive Practices of Women With Complex Congenital Heart Disease journaltitle: The American Journal of Cardiology articlelink: http://dx.doi.org/10.1016/j.amjcard.2016.11.047 content_type: article copyright: © 2016 Elsevier Inc. All rights reserved. ispartof: American Journal of Cardiology vol:119 issue:6 pages:911-915 ispartof: location:United States status: published

Background Women with single ventricle cardiac physiology who have undergone Fontan procedures are surviving well into reproductive age and have historically been discouraged from pregnancy despite the paucity of data regarding maternal and neonatal outcomes. Objective Our primary objective was to investigate the maternal and neonatal outcomes of pregnant women in a large cohort 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 gravidas with a Fontan palliation who delivered at UCLA Medical Center over a ten-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 substantially blunted in Fontan physiology. Third trimester cardiac index positively correlated to birthweight z-score (R2=0.48, p=0.038) but not to SGA (R2=0.13, p=0.339). The most common cardiac complications in pregnancies exceeding 24 weeks’ gestation were sustained arrhythmia (37.5%) and decompensated heart failure (21%). The 37 pregnancies comprised 25 (67.6%) live births, 1 (2.7%) fetal demise, 9 (24%) spontaneous abortions, and 2 (5.4%) pregnancy terminations. Of the live births, 60% were preterm at an average gestational age of 34.9±3.7 weeks. Newborns were delivered via cesarean in 53%, operative vaginal delivery in 28%, and spontaneous vaginal delivery in 20%. Forty percent of neonates were born small (less than the 10th centile) for gestational age, and 44.0% of all neonates were admitted to the NICU. Conclusions Women with a single ventricle and Fontan physiology can have successful pregnancy, although they are at increased risk for arrhythmias and heart failure. The decreased cardiac reserve in these gravidas blunts the normal increase in maternal cardiac output, which is associated 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 the onset of parturition.

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.

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. ispartof: European Journal of Cardiovascular Nursing vol:12 issue:1 pages:7-16 ispartof: location:England status: published

Many children born with complex childhood illnesses that historically caused early death are now surviving into adulthood with the expectation of leading meaningful and productive lives. They will ultimately need to transition their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now, patients are lost to appropriate follow-up. In fact, the number of adults with congenital heart disease (CHD) in the United States is rising exponentially and now exceeds 1 000 000.1,–,7 At least half of these patients may have complex CHD. Fewer than 30% of adults with CHD are seen by appropriate specialized providers. Fewer than 15% of these patients, who are seen in specialty adult CHD (ACHD) clinics, have CHD that is classified as severe.8 Thus, adolescents with CHD constitute a growing population of individuals for whom a well-planned and well-executed “transition process” is essential. The goals of a formal transition program are to prepare young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should include age-appropriate education about medical conditions and promote skills in communication, decision making, self-care, and self-advocacy.9,–,13 It should foster greater personal and medical independence and a greater sense of control over health, healthcare decisions, and psychosocial environment. The ultimate goal of a transition program is to optimize the quality of life (QOL), life expectancy, and future productivity of young patients.14 We acknowledge that the development of ideal transition programs is a …

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.

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.

Early diagnosis and surgical advances have ensured that infants born with congenital heart disease (CHD) will reach adolescence. As the population of adolescent female patients with CHD continues to grow health professionals must be prepared to advise not only their patients but also the patients parents and primary care providers about the contraceptive choices that may be safely prescribed. This article discusses contraceptive issues to consider as teens with CHD begin to transition into young adults and begin engaging in adult behaviors. (authors)

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.

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.

Objective: To evaluate pregnancy-related hospitalizations of women with congenital heart disease (CHD) in California Background: It is recommended that women with complex CHD receive specialty care during pregnancy. There is a perception that these women require cesarean section (CS) for cardiac reasons, but hemodynamic changes are often less with a vaginal delivery. There are no population based studies evaluating pregnancy in CHD. Methods: California hospital discharge data from the years 2000–2003 were analyzed. Subjects were chosen by age (12–44 years), diagnosis code for CHD and diagnosis or procedure code related to pregnancy. Among the hospitals, there were 7 self-identified specialized adult CHD centers. Descriptive statistics were used to characterize the population by age, CHD diagnosis (complex or non-complex), insurance, hospital (adult CHD center or not), and outcome (delivery, ante-partum, post-partum condition). Multivariate regression was used to determine predictors for CS. Results: There were 1032 hospitalizations among women age 14–44 years (mean 27.7 years). Hospitalizations occurred at 210 different hospitals, with only 14% at the 7 adult CHD centers. CHD diagnoses were complex in 52% and non-complex in 48%. Among the hospitalization outcomes, 77% were deliveries (66% vaginal, 34% CS), 2% abortive and 21% ante or post partum conditions. CS rates were not statistically different between CHD centers (32%) and non-centers (34%) but were higher than the general population (21%). Women with complex CHD were more likely to have a CS (p=0.005) or an abortive outcome (p Conclusion: Most data on pregnancy in CHD are from single center studies, however, the majority of women with CHD delivered at non-CHD centers. Overall pregnancy outcomes did not appear different by hospital type, but the rates of CS in women with complex CHD suggests specialty care may affect mode of delivery. Understanding of the outcomes of pregnancy in women with CHD would be enhanced by detailed population-based studies.

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.

Objectives. This study sought to determine risks and outcome of pregnancy and delivery after the modified Fontan operation. Background. Increasingly, female Fontan patients reaching child-bearing years are interested in having children. To date, the number of reported pregnancies is small, and pregnancy has therefore been discouraged. Methods. One hundred ten of 126 female patients from the Fontan registries of the Mayo Clinic and University of California Los Angeles Medical Center responded to a mailed questionnaire. An additional six patients with a reported pregnancy from other centers were identified and reviewed to assess pregnancy outcomes. Results. Among the participating centers, a total of 33 pregnancies after Fontan operation for various types of univentricular heart disease were reported. There were 15 (45%) live births from 14 mothers, with 13 spontaneous abortions and 5 elective terminations. In the 14 women with live births, the median number of years between operation and pregnancy was 4 (range 2 to 14). Reported prepregnancy problems in these gravidas included atrial flutter in one patient and ventricular dysfunction, aortic regurgitation and atrioventricular valve regurgitation in another. One patient developed supraventricular tachycardia during pregnancy and had conversion to sinus rhythm. No maternal cardiac complications were reported during labor, delivery or the immediate puerperium. There were six female and nine male infants (mean gestational age 36.5 weeks; median weight 2,344 g). One infant had an atrial septal defect. At follow-up, mothers and infants were alive and well. Conclusions. Pregnancy after the Fontan operation appears to have been well tolerated in 13 of 14 gravidas. There does appear to be an increased risk of miscarriage. The tendency to routinely discourage pregnancy may need to be reconsidered.

To examine the extent to which congenital heart disease (CHD) influences ovarian function, 98 women (mean age 32.7 years) were asked to complete a mailed questionnaire regarding their menstrual patterns. Patients were divided into acyanotic (60%) and cyanotic (38.8%) groups. Cycle lengths, duration of menstrual cycle, regularity of menses, amount of flow, and breakthrough bleeding were compared in these two groups and against agematched controls. The mean age of menarche for the sample (13.4 years) was significantly different (p

Since the initial description by Fontan and Baudet 1 of a surgical procedure for tricuspid atresia, a number of modifications have evolved permitting application of this operative technique to a number of complex cyanotic congenital heart defects. 2 For the postoperative female patient, questions regarding fertility and pregnancy are being raised. Whether and how an atrial-dependent pulmonary circulation and the elevated systemic venous pressure commonly seen after Fontan might influence ovarian function and menstrual patterns has not been an area of inquiry. The specific aim of the study, therefore, was to characterize the menstrual patterns of females after Fontan or modified Fontan repair.

Diminished glomerular filtration rate, proteinuria, and large hypercellular congested glomeruli with segmental sclerosis are found in late survivors with cyanotic congenital heart disease (CCHD). Hyperuricemia is common, acute gouty arthritis is less common than uric acid levels would predict, and overt tophaceous deposits of uric acid are exceptional. The role of the kidney in causing the basic biochemical disturbances, and the relative importance of impaired urate excretion vs urate overproduction have not been established. Accordingly, we reviewed the courses of two index patients and prospectively studied eight additional CCHD patients from 28 years to 46 years old with mean hematocrits of (62 +/- 10%). Plasma creatinine concentration was normal (0.9 +/- 0.1 mg/dl) yet glomerular filtration rate was mildly reduced to 93 +/- 14 ml/min as measured by creatinine clearance and to 81 +/- 6 ml/min as measured by 111In DTPA. Three patients had significant proteinuria and one was nephrotic. Plasma uric acid concentration was high in all but one (8.2 +/- 2.1 mg/dl), mean 24 hr uric acid excretion was normal (564 +/- 221 mg), and fractional uric acid excretion was relatively low (6.3 +/- 2.6%). The two patients with highest plasma uric acid levels (12.0 and 10.2 mg/dl) had the lowest fractional excretions (2.8% and 4.0%). Both of these patients had diminished capacity to excrete a water load (38% and 27%/4 hr) and to maximally concentrate urine (520 and 635 mOsm/kg after water deprivation and vasopressin). In conclusion, high plasma uric acid levels in late survivors with CCHD are secondary to inappropriately low fractional uric acid excretion, not to urate overproduction.(ABSTRACT TRUNCATED AT 250 WORDS)

The dynamic increase in oxygen uptake (VO2) at the start of exercise reflects the circulatory adjustments to metabolic changes induced by the exercise. Because VO2 measured at the lungs is the product of pulmonary blood flow and arteriovenous oxygen difference, pathologic conditions affecting the capacity of these factors to change would be expected to alter VO2 kinetics. To determine whether measurement of VO2 kinetics can detect conditions in which the pulmonary blood flow response to exercise is abnormal, VO2 was measured, breath-by-breath, during the transition from rest to exercise in 13 adults with cyanotic congenital heart disease (central venoarterial shunting) and in nine normal subjects. The increase in VO2 above baseline during the first 20 sec of exercise (phase I), reflecting the immediate increase in pulmonary blood flow, was diminished in the patients compared with that in normal subjects (14.8 +/- 10.9 vs. 49.8 +/- 19.2 ml of oxygen) (p less than .001). The patients' phase I responses correlated with their reported physical activity tolerance (p less than .01). In addition, the second phase of the VO2 response kinetics was prolonged in patients compared with normal subjects (half-time = 63 +/- 13 vs 15 +/- 13 sec) (p less than .001). We conclude that striking disturbances in VO2 kinetics occur in patients with cyanotic congenital heart disease and that these measurements provide a useful noninvasive means of evaluating the degree to which the increase in pulmonary blood flow is constrained in response to exercise.

The Fontan procedure is the definitive operation for patients with single ventricle physiology. As more women with Fontan operations reach childbearing age, it is important to determine the impact pregnancy has on short and long-term outcomes. The purpose of this multicenter study was to determine