Exercise testing in adult congenital heart disease: At center stage for many reasons

Adult survivors with congenital heart disease represent rapidly evolving cohorts of complex patients that pose unique challenges to our adult cardiology clinics. These patients are at high risk of cardiovascular complications and premature death as young adults. Regular assessment at specialist centers by multidisciplinary expert teams is key to optimal management. Serial exercise testing has proven to be a powerful tool for optimal management of these patients. In this overview, the major tasks of exercise testing are reviewed. This includes prognostication and risk stratification as well, as the evaluation of specific causes of impaired exercise capacity based on analysis of the different components of cardio-pulmonary exercise testing. While peak oxygen consumption has proven to be a robust marker of outcome in adult cardiology, in patients with congenital heart disease, reference values may need to be adapted to specific congenital heart disease entities and the prognostic validity for specific patient cohorts requires further elucidation. Careful analysis of results from exercise testing often reveal important insights into the individual patient's cardiac physiology and may allow targeted interventions that improve exercise capacity and potentially outcome. This requires integration of clinical findings and findings from cardiac imaging. Occasionally, exercise testing during cardiac catheterization may reveal insights into exercise hemodynamics and may be important for decision-making, particularly for decision-making about occlusion of intra-cardiac shunts. From a research perspective, the analysis of exercise capacity and its components will enable us to make more evidence-based decisions for cardiac interventions and will allow a better understanding of prognosis

Pregnancy in women with congenital heart disease

Congenital heart defects are the most common birth defects. Major advances in open-heart surgery have led to rapidly evolving cohorts of adult survivors and the majority of affected women now survive to childbearing age. The risk of cardiovascular complications during pregnancy and peripartum depends on the type of the underlying defect, the extent and severity of residual haemodynamic lesions and comorbidities. Careful individualized, multi-disciplinary pre-pregnancy risk assessment and counselling, including assessment of risks in the offspring and estimation on long-term outcomes of the underlying heart defect, will enable informed decision making. Depending on the estimated risks, a careful follow-up plan during pregnancy as well as a detailed plan for delivery and postpartum care can reduce the risks and should be made by the multi-disciplinary tea

Impact of the COVID-19 Pandemic on Health-Related Concerns, Quality of Lifeand Psychological Adjustment in Young Adults with Congenital Heart Disease

Background: The risk for a severe disease course in case of infection with SARS-CoV-2 in young adults with congenital heart disease is largely unknown, potentially leading to uncertainty and anxiety among affected patients. This study aims to investigate health-related concerns, health-related quality of life and psychological adjustment in patients with congenital heart disease compared to healthy peers during the COVID-19 pandemic. Methods: One-hundred patients with congenital heart disease and 50 controls (M = 29.7, SD = 3.8 years) were recruited. They completed an online survey including the assessment of health-related concerns regarding COVID-19, the 12-item Short Form Health Survey and the Brief Symptom Inventory. Results: Patients considered COVID-19 to be a more serious issue (Generalized odds ratio [GenOR] = 1.67, p = 0.04), were more concerned about becoming infected (GenOR = 2.93, p < 0.001) and expressed more fear about leaving their homes (GenOR = 1.81, p = 0.004) while general anxiety symptoms were not different between groups (p = 0.23). Patients relied more on family and friends for support (30% vs. 2% in controls, p < 0.001) and reported better compliance with protective measures (p = 0.03). Mental health-related quality of life and psychological adjustment were not different between groups (p = 0.17 and p = 0.68, respectively). Physical health-related quality of life was lower in patients compared to controls (p = 0.03). Conclusions: Young adults with congenital heart disease in Switzerland are more concerned about their health during the COVID-19 pandemic compared to healthy peers. These concerns, however, do not translate into generally impaired mental wellbeing. The impact of the easing of lockdown measures on long-term anxiety levels and quality of life requires further stud

Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts

Coronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Congènit; Defectes cardíacsCoronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Congénito; Defectos cardiacosCoronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Congenital; Heart defectsObjective Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. Methods This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. Results 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. Conclusions Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.EPOCH-ASO is funded by internal grants without support from the pharmaceutical industry

Brain volumes in adults with congenital heart disease correlate with executive function abilities

Congenital heart disease is the most common birth defect, and patients are at risk for neurodevelopmental impairment and brain abnormalities. Yet, little is known about the link between brain volumes and cognitive function in adults with congenital heart disease. Forty-four patients and 53 controls between 18 and 32 years underwent brain magnetic resonance imaging and cognitive testing, assessed with an intelligence quotient and executive function global score. Associations between brain volumes and cognitive function were calculated using linear models. Cognitive function in patients was within the normal range (intelligence quotient: 97.74 (10.76)). Total brain volume was significantly smaller in patients compared to controls (1067.26 (113.53) vs 1113.04 (97.88) cm3, P 0.4). After adjusting for total brain volume, only corpus callosum volume remained significantly smaller (P = 0.03). Smaller total brain volume was associated with poorer overall executive functioning (P = 0.02) and inhibition (P < 0.01), in both patients and controls. The association between total brain volume and overall executive functioning was moderated by parental socioeconomic status (lower socioeconomic status was associated with a stronger association between brain volume and EF; interaction P = 0.03). In adults with congenital heart disease, despite normal intelligence quotient, brain volume alterations persist into adulthood and are related to executive functioning, in particular inhibitory control. Adults coming from low socioeconomic background and with altered brain volumes are especially vulnerable and should thus be followed-up during adulthood to ensure optimal social and educational support. Keywords: ACHD; Brain imaging; Brain volume; Congenital heart disease; Executive function

B-Type Natriuretic Peptide in Pregnant Women With Heart Disease

ObjectivesThe objectives of this study were to examine: 1) B-type natriuretic peptide (BNP) response to pregnancy in women with heart disease; and 2) the relationship between BNP levels and adverse maternal cardiac events during pregnancy.BackgroundPregnancy imposes a hemodynamic stress on the heart. BNP might be a useful biomarker to assess the ability of the heart to adapt to the hemodynamic load of pregnancy.MethodsThis was a prospective study of women with structural heart disease seen at our center. Serial clinical data and plasma BNP measurements were obtained during the first trimester, third trimester, and after delivery (>6 weeks).ResultsSeventy-eight pregnant women were studied; 66 women with heart disease (age 31 ± 5 years), and 12 healthy women (age 33 ± 5 years). During pregnancy, the median peak BNP level was higher in women with heart disease compared with control subjects (median 79, interquartile range 51 to 152 pg/ml vs. median 35, interquartile range 21 to 43 pg/ml, p < 0.001). In women with heart disease, those with subaortic ventricular dysfunction had higher BNP levels (p = 0.03). A BNP >100 pg/ml was measured in all women with events during pregnancy (n = 8). Sixteen women had increased BNP levels during pregnancy but did not have clinical events. None of the women with BNP ≤100 pg/ml had events. BNP ≤100 pg/ml had a negative predictive value of 100% for identifying events during pregnancy.ConclusionsMany pregnant women with heart disease have increased BNP levels during pregnancy. Incorporating serial BNP levels in into clinical practice can be helpful, specifically in adjudicating suspected adverse cardiac events during pregnancy