Lack of sexual orientation and gender identity (SOGI) data creates barriers for lesbian, gay, bisexual, transgender, and queer (LGBTQ+) people in health care. Barriers to SOGI data collection include physician misperception that patients do not want to answer these questions and discomfort asking SOGI questions. This study aimed to assess patient comfort towards SOGI questions across five quaternary care adult congenital heart disease (ACHD) centres.

A survey administered to ACHD patients (≥18 years) asked (1) two-step gender identity and birth sex, (2) acceptance of SOGI data, and (3) the importance for ACHD physicians to know SOGI data. Chi-square tests were used to analyse differences among demographic groups and logistic regression modelled agreement with statement of patient disclosure of SOGI improving patient–physician communication.

Among 322 ACHD patients, 82% identified as heterosexual and 16% identified as LGBTQ+, across the age ranges 18–29 years (39.4%), 30–49 years (47.8%), 50–64 years (8.7%), and > 65 years (4.0%). Respondents (90.4%) felt comfortable answering SOGI questions. Respondents with bachelor’s/higher education were more likely to “agree” that disclosure of SOGI improves patient–physician communication compared to those with less than bachelor’s education (OR = 2.45; 95% CI 1.41, 4.25; p = .0015).

These findings suggest that in this largely heterosexual population, SOGI data collection is unlikely to cause patient discomfort. Respondents with higher education were twice as likely to agree that SOGI disclosure improves patient–physician communication. The inclusion of SOGI data in future studies will provide larger samples of underrepresented minorities (e.g. LGBTQ+ population), thereby reducing healthcare disparities within the field of cardiovascular research.

National standards to ensure effective transition and smooth transfer of adolescents from paediatric to adult services are available but data on successful transition in CHD are limited. The aim of this study is to assess the effectiveness of our transition pathway.

Adolescents with CHD, aged 15–19 years, who attended the joint cardiac transition clinic between 2009 and 2018 were identified from the Patient Administration Systems. Patient attendance at their first adult CHD service appointment at Royal Papworth Hospital was recorded.

179 adolescents were seen in the joint cardiac transition clinic in the 9-year study period. The median age of the patients when seen was 16 (range 15–19) years. 145 patients were initially planned for transfer to the Royal Papworth Hospital adult CHD service. Three patients were subsequently excluded and the success of the transfer of care in 142 patients were analysed. 112 (78%) attended their first follow-up in the adult CHD clinic as planned, 28 (20%) attended after reminders were sent out with 5/28 requiring multiple reminders, and only 2 (1.4%) failed to attend. Overall, transfer of care was achieved in 140 (98.6%) patients.

A dedicated joint cardiac transition clinic involving multi-professional medical and nursing teams from paediatric and adult cardiology services appears to achieve high engagement rates with the adult services. This approach allows a ‘face’ to be put on a named clinician delivering the adult service and should be encouraged.

The aim of this cross-sectional study was to examine the prevalence of the multidimensional phenomenon of fatigue in adults with congenital heart disease.

Adults with congenital heart disease are a growing population, and patient-reported outcomes can provide valuable information about the patient’s experience of living with CHD. Fatigue is a multidimensional phenomenon that can be described as an overwhelming feeling of exhaustion with a reduced capacity of mental and physical work. Fatigue can be observed clinically in adults with congenital heart disease, but the actual prevalence is unknown.

Fatigue was assessed by the Multidimensional Fatigue Inventory which enables the respondent to report the presence of fatigue according to five dimensions: “general fatigue,” “physical fatigue,” “mental fatigue,” “reduced motivation,” and “reduced activity.” The questionnaire was sent to 463 patients in Lund and Umeå. Four groups with complex CHD and two groups with moderately complex CHD were included. The reliability (internal consistency) of the Multidimensional Fatigue Inventory was tested for all dimensions and groups of diagnosis.

The response rate was 56.6% (n= 262). In patients with complex CHD, 40.0–59.4% reported severe to very severe general fatigue, and patients with a single ventricle reported the highest prevalence (59.4%). Among patients with complex CHD, 29.2–40.0% reported severe to very severe mental fatigue. The Multidimensional Fatigue Inventory had a high reliability measured with Cronbach’s alpha.

The study findings show a relatively high prevalence of fatigue in adults with congenital heart disease, and general fatigue was the most prevalent. Further studies are needed regarding fatigue and its causes and consequences in adults with congenital heart disease.

The Multidimensional Fatigue Inventory proved to be an instrument with high reliability and low internal loss, which suggests that the instrument may be suitable to use as a patient-reported outcome in the care of adults with congenital heart disease, preferably at repeated occasions.

Amongst patients with CHD, the time of transition to adulthood is associated with lapses in care leading to significant morbidity. The purpose of this study was to identify differences in perceptions between parents and teens in regard to transition readiness.

Responses were collected from 175 teen–parent pairs via the validated CHD Transition Readiness survey and an information request checklist. The survey was distributed via an electronic tablet at a routine clinic visit.

Parents reported a perceived knowledge gap of 29.2% (the percentage of survey items in which a parent believes their teen does not know), compared to teens self-reporting an average of 25.9% of survey items in which they feel deficient (p = 0.01). Agreement was lowest for long-term medical needs, physical activities allowed, insurance, and education. In regard to self-management behaviours, agreement between parent and teen was slight to moderate (weighted κ statistic = 0.18 to 0.51). For self-efficacy, agreement ranged from slight to fair (weighted κ = 0.16 to 0.28). Teens were more likely to request information than their parents (79% versus 65% requesting at least one item) particularly in regard to pregnancy/contraception and insurance.

Parents and teens differ in several key perceptions regarding knowledge, behaviours, and feelings related to the management of heart disease. Specifically, parents perceive a higher knowledge deficit, teens perceive higher self-efficacy, and parents and teens agree that self-management is low.

COVD-19 pandemic has overwhelmed many healthcare systems worldwide. Underlying cardiovascular disease predisposes to greater disease susceptibility and more complications including mortality. Such data is unverified in adults with congenital heart disease (ACHD). The aim of the study is to report the Tehran experience with respect to preventative self-care measures, disease exposure, susceptibility, and outcomes after COVD-19 infection in ACHD patients.

A telephone-based survey was conducted in ACHD patients, focusing on new-onset symptoms that might indicate COVID-19 infection, prevention measures, confirmed infection rates, and outcomes.

Three-hundred and nine ACHD patients, with a mean age of 29.13 years (range from 14 to 72 years, SD = 10.64), and 170 (55%) women were assessed. The majority (86.7%) had moderate or complex ACHD. Two-thirds (67.3%) of the patients practiced high-level preventative self-care measures. After community exposure, 33.3% developed COVID-19, and after household exposure, 43.7% developed COVID-19. There was only one mortality in a post-operative patient. Thirty-seven patients (12%) reported new symptoms including cough (10%), fatigue (8%), fever (7%), and new dyspnoea (6.5%). Amongst 18 (6%) with confirmed COVID-19, there was only 1 mortality in a post-operative patient. Age (adjusted OR = 1.19, 95% CI: 1.07–1.31, p = 0.001), contact with confirmed COVID-19 cases (adjusted OR = 59.34, 95% CI: 3.68–955.10, p = 0.004) were independently associated with COVID-19 infection.

Mortality risk associated with COVID-19 infection in ACHD patients with moderate or severe disease appears to be relatively low, similar to the general population. Such risk appears to act through conventional risk factors, and in this cohort, we demonstrated age as a significant risk factor in addition to exposure to the development of COVID-19 infection. Preventative self-care measures are a potentially significant and impactful intervention target for intervention and for improving outcomes.

A 10% prevalence of intracranial aneurysms in patients with coarctation of the aorta has been described in a few studies. Our objective is to describe the rate of intracranial aneurysm detection in patients with coarctation of the aorta in the current era. We hypothesise that, with earlier detection and coarctation of the aorta intervention, the rate of intracranial aneurysm is lower than previously reported and screening imaging may only be warranted in older patients or patients with certain risk factors.

This is a retrospective study of 102 patients aged 13 years and older with coarctation who underwent brain computed tomography angiography, magnetic resonance imaging (MRI), or magnetic resonance angiography between January, 2000 and February, 2018.

The median age of coarctation repair was 4.4 months (2 days–47 years) and the initial repair was primarily surgical (90.2%). There were 11 former smokers, 4 current smokers, and 13 patients with ongoing hypertension. Imaging modalities included computed tomography angiography (13.7%), MRI (41.2%), and magnetic resonance angiography (46.1%), performed at a median age of 33.3 years, 22.4 years, and 25 years, respectively. There were 42 studies performed for screening, 48 studies performed for neurologic symptoms, and 12 studies performed for both screening and symptoms. There were no intracranial aneurysms detected in this study.

These results suggest that the rate of intracranial aneurysms may be lower than previously reported and larger studies should explore the risk of intracranial aneurysms in coarctation of the aorta in the current era.

Adults with CHD are known to greatly benefit from a prompt access to continuous expert care. On the other hand, coronavirus disease 2019 pandemic has determined a dramatic worldwide reconfiguration of the healthcare systems, with rapid redeployment of resources towards this emergency. Italy was the first Western country affected by a large-scale spread of coronavirus disease 2019. The aim of our study is to analyse the impact of the coronavirus disease 2019 outbreak on in-hospital care of patients with CHD in an Italian tertiary centre.

We retrospectively reviewed data on CHD hospital admissions in our centre since 1 March, 2020, when the adoption of a strict infection containment policy throughout the country resulted in limited access of patients to routine hospital care and resources reallocation to the care of infected patients. Comparison with data from the previous year was performed in order to identify any relevant differences attributable to the outbreak. Despite cancellation of all elective procedures, the overall number of urgent hospital admission remained stable throughout the period of study. Patients admitted during the pandemic had greater disease complexity (p = 0.001) with longer length of in-hospital stay (p = 0.01). No adverse events or positive swabs were reported among CHD patients who were admitted to hospital or medical personnel caring for these patients.

Data from our early experience suggest that coronavirus disease 2019 pandemic did not impact significantly on the provision of urgent care to adult patients with CHD.

Clinical studies have revealed decreased exercise capacity in adults with small, unrepaired ventricular septal defects. Increasing shunt ratio and growing incompetence of the aortic and pulmonary valve with retrograde flow during exercise have been proposed as reasons for the previously found reduced exercise parameters. With MRI, haemodynamic shunt properties were measured during exercise in ventricular septal defects.

Patients with small, unrepaired ventricular septal defects and healthy peers were examined with MRI during exercise. Quantitative flow scans measured blood flow through ascending aorta and pulmonary artery. Scans were analysed post hoc where cardiac index, retrograde flows, and shunt ratio were determined.

In total, 32 patients (26 ± 6 years) and 28 controls (27 ± 5 years) were included. The shunt ratio was 1.2 ± 0.2 at rest and decreased to 1.0 ± 0.2 at peak exercise, p < 0.01. Aortic cardiac index was lower at peak exercise in patients (7.5 ± 2 L/minute/m2) compared with controls (9.0±2L l/minute/m2), p<0.01. Aortic and pulmonary retrograde flow was larger in patients during exercise, p < 0.01. Positive correlation was demonstrated between aortic cardiac index at peak exercise and previously established exercise capacity for all patients (r = 0.5, p < 0.01).

Small, unrepaired ventricular septal defects revealed declining shunt ratio with increasing exercise and lower aortic cardiac index. Patients demonstrated larger retrograde flow both through the pulmonary artery and the aorta during exercise compared with controls. In conclusion, adults with unrepaired ventricular septal defects redistribute blood flow during exercise probably secondary to a more fixed pulmonary vascular resistance compared with age-matched peers.

Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.

Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.

The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).

Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

The growing adult congenital heart disease (CHD) population requires efficient healthcare organisation. It has been suggested that clinically appropriate care be provided for individual patients on the least complex level possible, in order to alleviate saturation of special care programmes.

Semi-structured interviews with 10 general and 10 adult CHD cardiologists were conducted to elucidate opinions on healthcare organisation in Belgium. A particular focus was placed on the potential role of general cardiologists. The software program NVivo 12 facilitated thematic analysis.

A discrepancy existed between how general cardiologists thought about congenital care and what adult CHD cardiologists considered the minimum knowledge required to adequately treat patients. Qualitative data were categorised under the following themes: knowledge dissemination, certification, (de)centralisation of care, the role of adult CHD cardiologists, the role of dedicated nurse specialists, and patient referral. It appeared to be pivotal to organise care in such a way that providing basic care locally does not impede the generation of sufficient patient volume, and to continue improving communications between different care levels when there is no referral back. Moreover, practical knowledge is best disseminated locally. Cardiologists’ opinions on certification and on the role of dedicated nurse specialists were mixed.

On the basis of the results, we propose five recommendations for improving the provision of care to adults with CHD. A multidimensional approach to defining the role of different healthcare professionals, to improving communication channels, and to effectively sensitising healthcare professionals is needed to improve the organisation of care.

As a result of medical advances, the adult congenital heart disease population is rapidly expanding. Nonetheless, most patients remain prone to increased morbidity and mortality. Therefore, long-term medical resource use is required. This systematic review aims to present the trends over the past decades of medical resource utilization in adult congenital heart disease as well as its current status, with a focus on hospitalizations, emergency department visits, outpatient cardiology visits, and visits to other healthcare professionals.

MEDLINE (Pubmed), Embase, and Web of Science were searched for retrospective database research publications. The ISPOR checklist for retrospective database research was used for quality appraisal. Trends over time are explored.

Twenty-one articles met the inclusion criteria. All but one of the studies was conducted in Western Europe and North America. The absolute number of hospitalizations has been increasing over the last several decades. This increase is highest in patients with mild lesions, although these numbers are largely driven by hospitalizations of patients with an atrial septal defect or a patent foramen ovale. Meanwhile, outpatient cardiology visits are increasing at an even higher pace, and occur most often in geriatric patients and patients with severe lesions. Conversely, the number of hospitalizations per 100 patients is decreasing over time. Literature is scarce on other types of healthcare use.

A strong rise in healthcare utilization is noticed, despite the mitigating effect of improved efficiency levels. As the population continues to grow, innovative medical management strategies will be required to accommodate its increasing healthcare utilization.

Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.

In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnea using a type-3 portable overnight polygraph in adult patients with congenital heart disease were evaluated.

There were 104 patients [median age: 36 (interquartile range: 28–48) years] who were admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37%, 16%, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15≤ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnea, respectively. A large majority of the sleep apnea cases were categorised as obstructive sleep apnea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnea (respiratory disturbance index <5). Multivariable analysis showed that New York Heart Association class ≥II (OR, 4.36; 95% CI, 1.09–20.87) and body mass index ≥25 (OR, 4.29; 95% CI, 1.32–15.23) were independent risk factors for a respiratory disturbance index ≥15.

Our results showed a high prevalence of sleep apnea in adult patients with congenital heart disease. Its unique haemodynamics may be associated with a high prevalence of sleep apnea. Congestive heart failure and being overweight are important risk factors for sleep apnea. Management of heart failure and general lifestyle improvements will be important for controlling sleep apnea symptoms in these patients.

Adults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life.

Adults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review.

Of 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001).

Among adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.