Frequency of Miscarriage/Stillbirth and Terminations of Pregnancy Among Women With Congenital Heart Disease in Germany, Hungary and Japan.

BACKGROUND: The 2011 guidelines of the European Society of Cardiology (ESC) on the management of cardiovascular diseases during pregnancy define the maternal predictors for neonatal complications. The aim of this study was to determine whether these are associated with an increased number of miscarriages/stillbirths and terminations of pregnancy (TOPs) also in patients with congenital heart defects (CHD). METHODS AND RESULTS: The 634 women from Germany, Hungary and Japan were surveyed concerning the issues of sexuality and reproductive health, as well as their general life situation and medical care. 25% of the recorded pregnancies in women with CHD resulted in miscarriage, stillbirth or TOP. Affecting 16.8% of all recorded pregnancies, miscarriages or stillbirths occurred more frequently than in the general population and more than previously recorded for patients with CHD. TOP occurred in 8% of the surveyed pregnancies. Underlying maternal predictors for neonatal events had an influence on the number of TOP; among those with underlying predictors, TOP was recorded 3-fold more than in those without such predictors (15.6% vs. 5.5%). Remarkably, a significant deficit regarding the level of information on potential pregnancy-associated risks was observed in all 3 participating countries. CONCLUSIONS: Pregnant women with CHD should always be treated and counseled individually by cardiologists, gynecologists, obstetricians and anesthetists with appropriate expert knowledge. (Circ J 2016; 80: 1846-1851).

A Biobank for Long-term and Sustainable Research in the Field of Congenital Heart Disease in Germany.

Congenital heart disease (CHD) is the most frequent birth defect (0.8%-1% of all live births). Due to the advance in prenatal and postnatal early diagnosis and treatment, more than 90% of these patients survive into adulthood today. However, several mid- and long-term morbidities are dominating the follow-up of these patients. Due to the rarity and heterogeneity of the phenotypes of CHD, multicenter registry-based studies are required. The CHD-Biobank was established in 2009 with the aim to collect DNA from patients and their parents (trios) or from affected families, as well as cardiovascular tissues from patients undergoing corrective heart surgery for cardiovascular malformations. Clinical/phenotype data are matched to the International Paediatric and Congenital Cardiac Code (IPCCC) and the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10). The DNA collection currently comprises samples from approximately 4200 participants with a wide range of CHD phenotypes. The collection covers about 430 trios and 120 families with more than one affected member. The cardiac tissue collection comprises 1143 tissue samples from 556 patients after open heart surgery. The CHD-Biobank provides a comprehensive basis for research in the field of CHD with high standards of data privacy, IT management, and sample logistics.

[Competence Network for Congenital Heart Defects: innovative research for a young patient group]. / Kompetenznetz Angeborene Herzfehler: Neue Forschungskonzepte für eine junge Patientengruppe.

BACKGROUND: Congenital heart disease is the most frequent congenital malformation in humans and affects almost 1 % of all newborns. Thanks to advances in diagnostics and treatment, over 90 % of those affected reach adulthood today. Patient numbers are therefore growing constantly. The majority of those affected, however, remain chronically ill throughout life and require continuous specialized care. The spectrum of different clinical pictures is vast. Since this is a relatively young patient group, both research and care are lacking relevant knowledge and experience. OBJECTIVE: The Competence Network for Congenital Heart Defects (CNCHD) was founded in 2003 to tackle the challenge of heterogeneity and accordingly small sample sizes by means of networked research. The following article offers an overview of the network and its mode of operation. MATERIAL AND METHODS: The Germany-wide research network involves all relevant levels of research, public health care, the patient community and the general public. On the basis of a complex and flexible database infrastructure, it facilitates multicenter and interdisciplinary research in the field of congenital heart disease. RESULTS AND DISCUSSION: The CNCHD succeeded in establishing research on congenital heart disease throughout Germany by providing a powerful research network and a suitable infrastructure. Scientists from across Germany and all over the world use this basis to jointly find answers to burning questions in the field of congenital heart disease.

The importance of socio-demographic factors for the quality of life of adults with congenital heart disease.

PURPOSE: We conducted a study to measure the associations of socio-demographic factors with quality of life outcomes among adults with congenital heart disease (CHD). METHODS: Results are drawn from a questionnaire-based survey in 676 adults with CHD and compared to age and sex-matched controls of a representative national survey. Subjective outcomes were measured as health-related quality of life (hrQoL), health and life satisfaction. The associations of the subjective well-being with the degree of severity of the underlying heart defect and socio-demographic factors such as educational and employment status were quantified in multiple linear regression models. RESULTS: A significant correlation of the degree of severity of the heart defect was limited to the physical scale of the hrQoL, whereas for the mental scale of the hrQoL and the satisfaction scales, socio-demographic factors showed a stronger association. Furthermore, the associations of socio-demographic factors and subjective well-being were stronger in the patient group than in the control group. CONCLUSIONS: Socio-demographic factors can be significantly associated with the subjective well-being of adults with CHD. In order to assist the surgical successes of the past decades, which have ensured the survival of most of these patients into adulthood, increased attention should be paid to these domains in the care of adults with CHD.

Contraception in women with congenital heart disease.

The present study reports on contraceptive use, methods used, and counseling received on contraceptive issues for women with congenital heart disease and provides a brief review of current knowledge of the risks in relation to the different cardiac situations encountered with these specific patients. A total of 536 consecutive adult women with congenital heart disease (median age 29 years) were recruited from 2 tertiary care centers. They underwent a clinical assessment and completed a questionnaire regarding their contraceptive use. Oral contraceptives, condoms, and intrauterine devices were the most commonly used methods. Pregnancy occurred in almost every tenth woman despite the use of contraception. We identified a substantial number of women (20%) who were presently using contraceptive methods that were contraindicated for their specific cardiac condition. Additionally, a high proportion of patients (28%), in the group with high pregnancy-associated risks, were not using contraception despite having a sexual relationship. In our study, 43% of the women had not been counseled about contraception, and 48% had not been informed of the pregnancy-related risks by their treating physician. In conclusion, timely and competent counseling about contraception is important for women with congenital heart disease. Collaboration between cardiologists and gynecologists should be strengthened. Failure to give adequate family planning advice to this patient group could have hazardous consequences, causing an unnecessary risk to mother and child.

Sexuality and reproductive health in women with congenital heart disease.

The different biopsychosocial periods in a woman's life are all interactively associated with the cardiovascular system. The present study was designed to address questions related to sexuality and reproductive health in a large cohort of women with congenital heart disease. Overall, 536 women (median age 29 years, range 18 to 75) completed a questionnaire during their visit at 2 tertiary care centers for congenital heart disease. Patients were categorized according to their functional class and according to the degree of severity of the underlying heart defect. The median age at menarche was significantly delayed in patients with functional class III-IV and in women with complex or cyanotic anomalies. More than 1/4 of the women (29%) had at least once sought medical advice for menstrual discomforts, and the proportion was significantly increased for those in the worst functional class (49%, p <0.001) and for patients with a cyanotic heart defect (43%, p = 0.03). Overall, 9% reported increased or altered symptoms related to their heart defect during sexual activity. This proportion increased significantly with worsening functional class (6%, 11%, and 26% in functional class I, II, and III-IV, respectively; p = 0.001), increased severity (5%, 8%, and 17% for simple, moderate, and severe heart defects, respectively; p = 0.005), and in women with cyanosis (8% and 28% in acyanotic and cyanotic patients, respectively; p <0.001). In conclusion, to ensure high-quality care for this demanding and growing patient population, physicians must be aware that issues related to the entire reproductive cycle should be considered when counseling these patients.

[The competence network for congenital heart defects. Networking instead of isolated efforts for optimized research and care]. / Das Kompetenznetz Angeborene Herzfehler (KN AHF): Vernetzung statt Insellösungen für optimierte Forschung und Versorgung.

Congenital heart defects (CHD) are the most common congenital malformation; there is no clearly defined clinical picture. Although, thanks to medical progress, most of those affected survive into adulthood, they remain chronically ill throughout their lives and require specific care. By enabling nationwide cooperation and interdisciplinary research, the Competence Network for Congenital Heart Defects can provide and optimize this care. Clinical studies concerning clinical manifestations and recent diagnostic and treatment options can make use of the collected data. Being the core project of the Network, the National Register for congenital heart defects (registered association) registers all patients with a CHD throughout Germany. This data provides a basis for epidemiologic studies concerning quality of life and psychosocial aspects. Thus, integral care and swift transfer of knowledge between science and practice can be achieved. Meanwhile, pediatric guidelines could be developed and raised to S2 level. Additionally, the Register's results allow an assessment of the present situation of cardiac malformations, which is to be further evaluated in current studies. Particular attention is paid to the prevalence of CHD and adult patients' needs. In the long term, the concept of integrated care, and especially the idea of multidisciplinarity, is to be realized within the scope of a "health care database". All in all, the Competence Network for Congenital Heart Defects plays a substantial part in detecting needs and deficits of the current care for patients with CHD and helps developing strategies for an adequate and efficient care, while guaranteeing a high degree of transparency for those concerned.