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Introduction: Patients with congenital heart disease (ConHD) are at increased risk for adverse cardiac events. Predicting long-term outcomes and guidance of patient management might benefit from a range of (new) biomarkers. This is a rapidly evolving field with potentially large consequences for clinical decision making. With a systematic review of available biomarkers in ConHD we identified the clinical role of these markers, knowledge gaps and future research directions., Methods: We systematically reviewed the literature on associations between blood biomarkers and outcome measures (mortality or composite adverse outcomes in patients with ConHD., Results: The inclusion criteria were met by 102 articles. Biomarkers assessed in more than 3 studies are discussed in the main text, those studied in 3 or less studies are summarized in the supplement. Thus, we discuss 15 biomarkers from 92 studies. These biomarkers were studied in 32,399 / 10,735 patients for the association with mortality and composite adverse outcomes, respectively. Biomarkers that were studied most and had statistically significant associations with mortality or composite adverse outcomes were (NT-pro)BNP, MELD-XI score, Hs-CRP, creatinine, albumin and sodium. Most of these biomarkers are involved in intracardiac processes associated with inflammation or are markers of renal function., Conclusion: For (NT-pro)BNP, clinical value for prediction of mortality and composite adverse outcomes in adult and paediatric ConHD has been shown. For MELD-XI, hs-CRP, albumin, creatinine, sodium, RDW, and GDF-15, correlations with mortality and composite adverse outcomes have been demonstrated in patient groups with mixed types of ConHD, but clinical utility needs additional exploration., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, long-term impact on neurodevelopment and executive functioning in adults with CHD are not completely understood., Purpose: To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood., Material and Methods: Longitudinal study of a cohort of patients (n = 194, median age: 49.9 [46.1-53.8]) who were operated in childhood (< 15 years old) between 1968 and 1980 (median follow-up time: 45 [40-53] years) for one of the following diagnoses: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA). Behavior Rating Inventory of Executive Function-Adult version (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning., Results: 40-53 years after surgery, the CHD group did show significantly better executive functioning compared to the norm data. No significant difference was found between mild CHD (ASD, VSD and PS) and moderate/severe CHD (ToF and TGA). Higher education, NYHA class 1 and better exercise capacity were associated with better self-reported executive functioning, whereas females or patients taking psychiatric or cardiac medications reported worse executive functioning., Conclusions: Our findings suggest favorable outcomes (comparable to normative data) regarding executive functioning in adults with CHD, both self- and informant-reported. However, further study is warranted to explore more in detail the different cognitive domains of executive functioning in these patients., (© 2023. The Author(s).)

Introduction: Due to the improved survival in individuals with congenital heart disease (CHD), considering their reproductive health has become more important. Currently, this topic is still underexplored., Areas Covered: We discuss fertility, sexuality, assisted reproductive technology (ART), and contraception in adults with CHD., Expert Opinion: Timely counseling regarding fertility, sexuality, pregnancy, and contraception is necessary, preferably during teenage years. Due to a lack of data, whether or not to perform ART in adults with CHD is almost always based on expert opinion and follow-up in an expert center is recommended. Future research is necessary to fill the gaps in knowledge on the risks and frequency of complications of ART in adults with CHD, but also to be able to differentiate the relative risks in the different types of CHD. Only then will we be able to counsel adults with CHD correctly and not unjustly deprive someone of a chance of pregnancy.

Introduction: Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, knowledge about their psychosocial functioning is limited., Methods: Longitudinal cohort study of patients (n = 204, mean age: 50 years, 46.1% female) who were operated during childhood (< 15 years) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot or transposition of the great arteries. Psychosocial functioning was measured every 10 years, using standardized and validated questionnaires. Results were compared with the general Dutch population and over time., Results: After a median follow-up of 45 [40-53] years adults with CHD had a significantly lower educational level, occupation level and employment rate, but better health-related quality of life and emotional functioning compared with normative data. Patients with moderate/severe defects reported significantly more self-perceived physical restrictions and lack of physical strength due to their CHD. Compared to 2011, in 2021 patients considered their CHD as more severe and they felt more often disadvantaged., Conclusions: Overall, despite a lower education, occupation level and employment rate, our sample of patients with CHD had a positive perception of their life and  their psychosocial functioning was even better than the norm. Although the quality of life was very good, their view on their disease was more pessimistic than 10 years ago, especially for patients with moderate/severe CHD., (© 2022. The Author(s).)

Background: Growth-differentiation factor-15 (GDF-15), a cytokine with broad cardiac and non-cardiac activity, has diagnostic and prognostic value in various diseases, including heart failure. We aimed to investigate the release of GDF-15 in adults with congenital heart disease (ConHD), and assess the association with cardiac function and functional capacity., Methods: A total of 587 consecutive adults with ConHD (median age 33 [IQR 25-41] years, 59% men, and 90% NYHA I) underwent electrocardiography, echocardiography, venepuncture and were seen by a cardiologist. A subset of 143 patients underwent bicycle ergometry on the same day., Results: Median plasma GDF-15 was 618 [IQR 487-867] ng/L. In 87 patients (15%), GDF-15 was above the reference value of normal (1109 ng/L). GDF-15 levels were higher in older patients (r=0.367, p<0.001). GDF-15 was higher in patients with elevated pulmonary pressure (median 1114 [IQR 796-2320 ng/L) than in patients with normal pulmonary pressure (median 606 [IQR 481-826] ng/L, p<0.001). GDF-15 correlated positively with NT-proBNP (r=0.445, p<0.001). In multivariate analysis adjusting for age, sex, and NT-proBNP, hs-TnT and hs-CRP, GDF-15 above the reference value was associated with NYHA class (odds ratio for NYHA≥II: 3.5 [95% CI 1.8-6.8], p<0.001), and decreased exercise capacity (odds ratio for workload >85%:0.2 [95% CI 0.06-0.8], p=0.018), but not with systolic ventricular function or ECG rhythm., Conclusions: GDF-15 is elevated in a substantial number of patients and higher in those with elevated pulmonary pressures, regardless of underlying congenital diagnosis. GDF-15 is associated with NYHA class, NT-proBNP and exercise capacity, suggesting the marker has diagnostic and potential prognostic value in adults with ConHD., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Cardiac disease in pregnancy is a challenging health care problem. The number of cases and their complexity is increasing, such that heart disease is now the leading cause of maternal mortality in developed countries. Numerically, women with congenital heart disease (CHD) make up the majority of cases and although maternal mortality is infrequent, a good outcome is only achieved though meticulous care, which starts pre-pregnancy and continues for months after the pregnancy has ended. All women with CHD should be assessed and counseled before pregnancy and carefully monitored during pregnancy, the delivery and in the puerperium. In most cases, pregnancy is well tolerated but in some conditions, such as pulmonary hypertension or severe dilatation of the aorta, pregnancy is extremely high risk and should be advised against.

Objectives: To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders., Design and Patients: Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18-45 years): atriopulmonary anastomosis (n = 23), atrioventricular connection (n = 5) and total cavopulmonary connection (n = 10)., Results: Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n = 2), uterus bicornis (n = 1) and related to endometriosis (n = 1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n = 15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population)., Conclusion: Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.

Aims: To test the predictive value of medical variables, covering the complete medical course from birth until the present, for long-term behavioural and emotional problems in adulthood, in patients operated for congenital heart disease in childhood., Methods and Results: This study concerns the second psychological and medical follow-up of a cohort of patients operated for congenital heart disease (n=362; age 20-46 years). Behavioural and emotional problems were assessed with the Young Adult Self-Report and the Young Adult Behavior Checklist. Medical prediction variables were derived from medical examination and file search. Being female, having low exercise capacity and restrictions imposed by physicians are significant predictors for behavioural and emotional problems as reported by patients themselves. Regarding the scar, personal experiences of patients form a better predictor for later problems than judgement of aesthetical aspects by physicians. Early hospitalisations with reoperations are predictive for behavioural and emotional problems as reported by other informants. The cardiac diagnoses of ventricular septal defect and transposition of the great arteries are associated with higher levels of behavioural and emotional problems., Conclusion: Recent experiences concerning the scar, physical condition and imposed restrictions are the strongest predictors for behavioural and emotional problems as reported by patients themselves.

During pregnancy cardiac output rises by 40-50%. At first stroke volume in particular increases, with the heart rate rising mainly in the second part of the pregnancy. More and more women with congenital heart disease are reaching adulthood and want to become pregnant. The haemodynamic changes during pregnancy are an additional burden for women with heart disease. Risk factors for occurrence of important cardiac problems during pregnancy are: previous cardiac events, lower functional class, cyanosis, left-sided heart obstruction or a diminished systolic function of the systemic ventricle. Pregnancy is contraindicated in pulmonary hypertension, in Marfan syndrome with an ascending aorta diameter of > 50 mm and in severe mitral or aortic valve stenosis. The recurrence risk for children varies between 3 and 50%, depending on the nature of the congenital heart disease. Foetal outcome is depending on the haemodynamic situation and medication use of the mother. ACE inhibitors are contraindicated during pregnancy.

Background: Contrast echocardiography improves endocardial border detection of the left ventricle. Whether this is also true for the right ventricle (RV) is unknown. The aim of this study is to assess whether the use of contrast (Sonovue) echocardiography has additional value in RV endocardial border visualisation (EBV), and whether it has impact on the echocardiographic judgement of RV function., Methods: Twenty adult patients with congenital heart disease were imaged using second harmonic two-dimensional echocardiography with and without contrast. Two independent observers analysed EBV of 13 RV wall segments in each patient. EBV was graded for each wall segment from 0 to 3 ( 0 = not visible, 3 = optimal visible)., Results: EBV improved in all patients with contrast echocardiography compared to second harmonic imaging (mean EBV 1.00 +/- 0.77 with second harmonics, 2.13 +/- 0.75 with contrast, P < 0.0001). The benefit was most evident in the near-field images. In 55% of the patients visual estimation of RV function changed with contrast echocardiography., Conclusion: The use of contrast echocardiography is superior to second harmonic imaging for RV EBV. Improved EBV may allow more accurate assessment of RV dimensions and function.

Aims: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups., Methods and Results: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results., Conclusions: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.