PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Neuromental health aspects in adults with CHD after cardiopulmonary bypass intervention during childhood.

OBJECTIVE: It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients. METHODS: Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life. RESULTS: Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life. CONCLUSIONS: The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.

[Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives]. / Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern im Lichte der 2022-ESC-PAH-Leitlinien.

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart disease" is addressed only relatively superficial in these guidelines. Therefore, in the present article, this topic is commented in detail from the perspective of congenital cardiology.

Cognitive function in adults with Fontan palliation versus acyanotic CHD patients and association with health-related quality of life.

BACKGROUND: Impairments and developmental delay are often reported in infants and young children with CHD. However, currently, there is no data regarding cognitive abilities assessed by standardised intelligence tests in adults with CHD. This study assesses the cognitive function in Fontan patients compared with acyanotic CHD patients whether restrictions in cognitive function are present in adulthood and its association with health-related quality of life. METHODS: Forty-four adult CHD (female n = 21 (47.7%); mean age 34.7 ± 11.9 years), 22 with Fontan circulation and 22 with acyanotic CHD, underwent the Wechsler Intelligence Scale for adults as patients during routine follow-up in 2018. The Medical Outcomes Study Questionnaire Short-Form 36 Health Survey (SF-36) assessed health-related quality of life. RESULTS: Fontan patients showed significantly better results in the FSIQ (p = 0.020) and perceptual reasoning (p = 0.017) in comparison with patients with acyanotic CHD. All adult CHD patients showed normal IQ in subscales and full-scale IQ (FSIQ). In health-related quality of life, no association with cognitive function was found and no significant difference between both CHD groups, but trends to reduced values in acyanotic adult CHD. CONCLUSIONS: Interestingly, our study results in adult Fontan patients showed that it is possible to live an adult life with normal cognitive function and good health-related quality of life with a univentricular heart. Thus, this study could be a guidepost for more in-depth studies on cognitive function in Fontan survivors. In addition, the focus should be on health-related quality of life of adult CHD with simple CHD in particular, since a reduced health-related quality of life is not only medically based.

[Pulmonary arterial hypertension in congenital heart disease - Part I]. / Pulmonale arterielle Hypertonie bei Erwachsenen mit angeborenen Herzfehlern ­ Teil I.

The number of adults with congenital heart disease (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary arterial hypertension (PAH), which may develop early in untreated CHD. Despite timely treatment of CHD, PAH often persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart defects" is addressed only relatively superficially in these guidelines. Therefore, this article addresses the perspective of congenital cardiology in greater depth.

Bioelectrical Impedance Analysis as a Contemporary Biomarker of Obesity in Adults with Marfan- or Loeys-Dietz-Syndrome.

Background: It is clinically widely overlooked that many patients with Marfan- (MFS) or Loeys-Dietz-Syndrome (LDS) are obese. While anthropometric routine parameters are not very suitable, the modern Bioelectrical Impedance Analysis (BIA) seems superior for the acquisition of reliable noninvasive assessment of body composition of patients. The aim of the study was to assess the body composition of patients with MFS/LDS by BIA in order to detect occult obesity, which may be a risk marker for aortic or vascular complications. Methods: In this exploratory cross-sectional study, 50 patients (66% female; mean age: 37.7 ± 11.7 [range: 17-64] years) with a molecular genetic (n = 45; 90%) or clinical (n = 5; 10%) proven diagnosis of MFS or LDS were enrolled between June 2020 and February 2022. All BIA-measurements were performed with the Multifrequence-Impedance-Analyzer Nutriguard-MS (Data Input, Poecking, Germany). Results: The MFS/LDS collective was significantly different from an age-, sex-, and BMI-adjusted control in terms of body fat, percent cellularity, body cell mass, extra cellular mass/body cell mass index, and phase angle (all p < 0.05). The mean BIA-measured bodyfat was 31.7 ± 8.7% [range: 9.5-53.5%], while the mean calculated BMI of the included patients was 23.0 ± 4.8 kg/ m 2 [range: 15.2-41.9 kg/ m 2 ]. Therefore, using the obesity cut-off values for the body fat percentage of 25% in men and 35% in women, the BIA classifies as many as 28 patients (56.0%) as obese. In contrast only 12 patients (24.0%) were pre-obese, respectively 3 (6.0%) obese by BMI. The significant difference (p < 0.001) had an accordance of 42.7%. Overall, 15 patients (13 MFS; 2 LDS) had previous aortic surgery (n = 14) and/or interventional treatment (n = 2) for aortic complications (aneurysm, aortic dissection). 11 out of these 15 (73.3%) were currently classified as obese by BIA. Conclusions: The fact that many patients with MFS or LDS are obese is widely unknown, although obesity may be associated with impaired vascular endothelial function and an increased risk of cardiovascular complications. Also, in patients with MFS/LDS, BIA allows a reliable assessment of the body composition beyond the normal anthropometric parameters, such as BMI. In the future, BIA-data possibly may be of particular importance for the assessment of the vascular risk of MFS/LDS patients, besides the aortic diameters.

Improved Tricuspid Valve Function, Preload Recruitment and Ventricular Efficiency During Submaximal Exercise in Patients with Unoperated Ebstein's Anomaly: An MRI Study.

BACKGROUND: Adolescents and adults with native Ebstein's anomaly (EA) are at the benign part of the Ebstein spectrum, having survived infancy without surgery. In this population, surgical indication and timing remain objects of controversy and depend, among other factors, on exercise capacity. PURPOSE: To better understand the pathophysiology of exercise adaptation in native EA. STUDY TYPE: Retrospective. POPULATION: Ten patients with unoperated EA (age range 18-61 years) and 13 healthy subjects as controls. FIELD STRENGTH/SEQUENCE: Balanced steady-state free precession cine and phase contrast flow sequences at 1.5 T. ASSESSMENT: We measured volumes and flows at rest and during submaximal exercise. Hemodynamic parameters including stroke volume (SV), cardiac index (CI), ejection fraction (EF), and tricuspid regurgitation (TR) were calculated. STATISTICAL TESTS: We used nonparametric Mann-Whitney U-test and Wilcoxon signed-rank test. A P-value of <0.05 was considered statistically significant. RESULTS: Rest CI and SV were significantly higher in controls; rest heart rate (HR) was similar in the two groups (median 71 bpm by patients and 65 bpm by controls, P = 0.448). During exercise, CI increased significantly in both groups: from 2.40 to 3.35 L/min/m2 in the patient group and from 3.60 to 4.20 L/min/m2 in controls; HR increased significantly in both groups. SV increased significantly in the patient group, whereas it remained stable in controls (P = 0.5284). Patients' median TR decreased significantly: median 42% at rest and 30% during exercise; concomitantly, left ventricular (LV) preload increased significantly (+3% indexed LV end-diastolic volume) as did LVEF (median 59% at rest vs. 65% during exercise). DATA CONCLUSION: During submaximal exercise, patients with mild to moderate EA improved their cardiovascular system's total efficiency by increasing CI; this was obtained by an increase in HR and by the recruitment of volume, as shown by an increased LV end-diastolic volume and SV, with simultaneous decrease in TR. This was different from healthy subjects in which CI increased only due to HR increase. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY STAGE: 3.

Transcatheter implantation of covered stents serving as extravascular conduits-Proof of a CT-based approach in three cases.

BACKGROUND: Covered stents perform similar to surgically implanted conduits, although the stents work inside of vessels. We present a computed tomography (CT)-based workflow for the implantation of covered stents as extravascular conduits. METHODS: We selected three different use cases: 1. Connecting a left-sided partially anomalous drainage of a pulmonary vein to the left atrium. 2. Bypassing an outgrown Dacron conduit in aortic recoarctation. 3. Re-directing hepatic venous blood to the left lung in a Fontan patient with heterotaxy, connecting the innominate vein to the right pulmonary artery like a right-sided cavopulmonary connection. By postprocessing and analyzing CT scans for planning and by the use of long needles under biplane fluoroscopy for the realization of the procedure, we projected and performed the exit of a long needle out of a vessel, the re-entering of a target vessel, and the bridging of the extravascular distance by implantation of covered stents. RESULTS: In all three cases, the covered stents were placed successfully, connecting vessels of 15-50 mm distance from each other with very good hemodynamic results. In one case, two stents were placed consecutively, overlapping each other to accomplish an exact fitting at the connection sites to the native vessels.

[Care of adults with congenital heart diseases in Germany-Leading role by internal medicine specialists and general practitioners]. / Versorgung von Erwachsenen mit angeborenen Herzfehlern in Deutschland ­ tragende Rolle von Internisten und Hausärzten.

BACKGROUND: The number of adults with congenital heart disease (ACHD) is steadily increasing. The present cross-sectional study was conceived to investigate ACHD care from the perspective of patients and family practitioners (specialists for general medicine and internal medicine, general practitioners). METHODS: Questionnaire-based cross-sectional study to analyze the real care situation of ACHD in Germany from the perspective of patients and primary care physicians (PCP). RESULTS: The questionnaire was completed by 4493 ACHD (53.7% female; 41.3 ± 16.9 years) and 1055 PCP. The majority of ACHD (79.8%) visited their PCP for noncardiac health problems but also for cardiac problems. Almost all ACHD had substantial needs for medical consultation (performance, employment etc.). Of the patients 2014 (44.8%) did not know of any certified ACHD specialists or specialized centers and 2816 (62.7%) respondents were not aware of any ACHD patient organization. Of the PCPs 87.5% had cared for ACHD of all severities due to defect-typical residual and resulting symptoms. Many were not aware of any certified ACHD specialists. Only 28.5% consulted an ACHD specialist. Only 23.5% were aware of ACHD patient organizations. CONCLUSION: General practitioners are a mainstay of ACHD care in Germany. The present study shows that ACHD and their general practitioners are largely uninformed about the specialized care structures available nationwide, despite the high level of need for specialist care. In order to keep the morbidity and mortality of affected patients low, solutions must be elaborated in future to involve and integrate primary care physicians more intensively into the already existing dedicated ACHD care structures, in cooperation with specialized pediatric cardiologists, cardiologists and centers.

Predictors of health-related quality of life in children with chronic heart disease.

OBJECTIVE: Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease. Patients and methods The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers. RESULTS: Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart-lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child's health-related quality of life. CONCLUSIONS: Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.

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).

Prognostic Value of Plasma B-Type Natriuretic Peptide in the Long-Term Follow-up of Patients With Transposition of the Great Arteries With Morphologic Right Systemic Ventricle After Atrial Switch Operation.

BACKGROUND: B-type natriuretic peptide (BNP) is an established marker for heart failure assessment, but the prognostic quality of BNP after atrial switch operation (ASO) has not yet been elucidated. METHODS AND RESULTS: In 89 patients (median age, 24 years; range, 15-35 years) after ASO, BNP was measured. During a 48-months follow-up we focused on critical cardiac events, defined as decompensation, sudden cardiac death or need for heart transplantation. BNP was considerably lower in 81 patients in functional class (FC) I/II (median, 35 pg/ml; range, 3-586 pg/ml) than in 6 patients in FC III/IV (median, 246 pg/ml; range, 14-1,150 pg/ml, P≤0.073). BNP was significantly higher after Mustard than after Senning procedure (P≤0.030). There was no significant difference in BNP between simple or complex transposition of the great arteries (TGA) (P≤0.44). Eleven subjects (13%, 95% CI: 7-22%) had a critical cardiac event within 48 months. On ROC analysis BNP had a high predictive value regarding discrimination of patients with and without critical events (area under the ROC curve, 0.90; 95% CI: 0.76 to >0.99, P<0.001). The cut-off was 85 pg/ml (sensitivity, 88%; specificity, 85%). Additionally, estimated event-free-survival was longer after Senning than after Mustard procedure (P≤0.017). There was no significant difference in outcome between patients with simple or complex TGA with regard to occurrence of critical events. CONCLUSIONS: BNP is a sensitive and specific prognostic marker for critical cardiac events after ASO.

Intraoperative renal near-infrared spectroscopy indicates developing acute kidney injury in infants undergoing cardiac surgery with cardiopulmonary bypass: a case-control study.

INTRODUCTION: Acute kidney injury (AKI) is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Renal near-infrared spectroscopy (NIRS) is used to evaluate regional oximetry in a non-invasive continuous real-time fashion, and reflects tissue perfusion. The aim of this study was to evaluate the relationship between renal oximetry and development of AKI in the operative and post-operative setting in infants undergoing cardiopulmonary bypass surgery. METHODS: In this prospective study, we enrolled 59 infants undergoing cardiopulmonary bypass surgery for congenital heart disease for univentricular (n = 26) or biventricular (n = 33) repair. Renal NIRS was continuously measured intraoperatively and for at least 24 hours postoperatively and analysed for the intraoperative and first 12 hours, first 24 hours and first 48 hours postoperatively. The renal oximetry values were correlated with the paediatric risk, injury, failure, loss, end (pRIFLE) classification for AKI, renal biomarkers and the postoperative course. RESULTS: Twenty-eight (48%) infants developed AKI based on pRIFLE classification. Already during intraoperative renal oximetry and further in the first 12 hours, 24 hours and 48 hours postoperatively, significantly lower renal oximetry values in AKI patients compared with patients with normal renal function were recorded (P < 0.05). Of the 28 patients who developed AKI, 3 (11%) needed renal replacement therapy and 2 (7%) died. In the non-AKI group, no deaths occurred. Infants with decreased renal oximetry values developed significantly higher lactate levels 24 hours after surgery. Cystatin C was a late parameter of AKI, and neutrophil gelatinase-associated lipocalin values were not correlated with AKI occurrence. CONCLUSION: Our results suggest that prolonged low renal oximetry values during cardiac surgery correlate with the development of AKI and may be superior to conventional biochemical markers. Renal NIRS might be a promising non-invasive tool of multimodal monitoring of kidney function and developing AKI in infants undergoing cardiac surgery with cardiopulmonary bypass.

Propofol effect on cerebral oxygenation in children with congenital heart disease.

Propofol is a short-acting, intravenously administered hypnotic agent which is used in procedural sedation in children. Propofol is known to decrease systemic vascular resistance, arterial blood pressure and can lead to desaturations and decreased systemic perfusion in children with cardiac shunting. This may result in a reduction in cerebral blood flow and oxygenation. Near-infrared spectroscopy (NIRS) can monitor cerebral tissue oxygenation in the frontal neocortex. The objective of our study was to measure the changes in cerebral oxygen and blood supply after Propofol infusion in children with congenital heart disease. Propofol infusion may reduce cerebral oxygenation in children with congenital heart disease. The study group consisted of 32 children (f:m = 18:14), with median age of 49 (5-112) months and median weight of 15 (5-34) kg. We performed NIRS derived continuous measurement of cerebral oxygenation and cardiac output using Electrical velocimetry for 5 min before and after sedation with Propofol (1-2 mg/kg i.v.) for cardiac catheterization. Simultaneously, non-invasive arterial blood pressure and transcutaneous oxygen saturation were measured. Propofol sedation led to a significant decrease in mean arterial pressure (79 ± 16 vs. 67 ± 12 mmHg) (p = 0.01) and cardiac index (3.2 ± 0.8 vs. 2.9 ± 0.6 ml/min/m(2)) (p = 0.03). In contrast, cerebral tissue oxygenation index, increased significantly from 57 ± 11 to 59 ± 10 % (p < 0.05). Sedation with Propofol increased cerebral tissue oxygenation despite a decrease in cardiac index and arterial blood pressure. This may be caused by a decreased oxygen consumption of the sedated brain with intact cerebral auto-regulation.

Pregnancy-related obstetric and cardiologic problems in women after atrial switch operation for transposition of the great arteries.

BACKGROUND: The number of women with congenital heart disease reaching reproductive age has increased due to therapeutic advances. The aim of this study was to examine obstetric and cardiac problems during pregnancy after Mustard/Senning repair for transposition of the great arteries. METHODS AND RESULTS: Sixty pregnancies in 34 women from 3 centers were studied. The women were interviewed, and their records reviewed for clinical status and diagnostic evaluation. Age range was 16-34 years during first pregnancy, and all were in a low functional class. There were 11 miscarriages and 5 abortions. Of 44 successful pregnancies, 20 were vaginal deliveries and 24, cesarean sections. A total of 25% were delivered prematurely. Thirteen babies had birth weight <2,500g. Deterioration in functional class occurred in 7 pregnancies, without recovery in 5. Deterioration in systolic function occurred in 4 of 44 echocardiographically documented pregnancies, without recovery in 75%. In 2 women resuscitation was necessary during delivery, in 1, supraventricular tachycardia occurred during labor. CONCLUSIONS: Pregnancy is usually well-tolerated, but outcome is unforeseeable and life-threatening problems can occur. These women belong in cardiac care conducted by experienced congenital cardiologists, who systematically check for typical residua. The pregnancy should be planned and gynecologists/obstetricians with special expertise integrated into the consultations. During delivery a congenital cardiologist, and an anesthetist experienced in congenital cardiology, should be present for possible severe cardiac events.

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Clinical aspects and targeted inspiratory muscle training in children and adolescents with Fontan circulation: a randomized controlled trial.

Background: Since 1968, thousands of patients with a morphologically or functionally univentricular heart have been treated with a total cavopulmonary connection/Fontan operation. Because of the resulting passive pulmonary perfusion, blood flow is assisted by the pressure shift during respiration. Respiratory training is known to improve exercise capacity and cardiopulmonary function. However, there are limited data on whether respiratory training can also improve physical performance after Fontan surgery. The aim of the present study was to clarify the effects of six months of daily home-based inspiratory muscle training (IMT) aimed at increasing physical performance by strengthening respiratory muscles, improving lung function and peripheral oxygenation. Methods: In this non-blinded randomized controlled trial, the effects of IMT on lung capacity and exercise capacity were measured in a large cohort of 40 Fontan patients (25% female; 12.3±2.2 years) who were under regular follow-up by the outpatient clinic of the Department of Congenital Heart Defects and Pediatric Cardiology of the German Heart Center Munich. After a lung function test and a cardiopulmonary exercise test, patients were randomly assigned in a parallel arm design to either an intervention group (IG) or a control group (CG) via stratified and computer-generated letter randomization from May 2014 to May 2015. The IG completed a daily, telephone-monitored IMT of three sets of 30 repetitions for six months with an inspiratory resistive training device (POWERbreathe medic®), the CG continued their usual daily activities without an IMT until the second examination within the timeframe of November 2014 until November 2015. Results: After six months of IMT, lung capacity values in the IG (n=18) did not increase significantly compared to the CG [n=19; ΔFVC: IG: 0.21±0.16 l vs. CG 0.22±0.31 l; P=0.946 (CI: -0.16, 0.17); ΔFEV1: CG: 0.14±0.30 vs. IG: 0.17±0.20 P=0.707 (CI: -0.20, 0.14)]. Exercise capacity did not improve significantly, yet the maximum workload achieved trended to improve with an increase of 14% in the IG vs. 6.5% in the CG [P=0.113 (CI: -15.8, 1.76)]. There was a significant increase of oxygen saturation at rest in the IG compared to the CG [IG: 3.31%±4.09% vs. CG: 0.17%±2.92%, P=0.014 (CI: -5.60, -0.68)]. Compared to the CG, the mean oxygen saturation at peak exercise no longer dropped below 90% in the IG. This observation is thus not statistically significant, yet of clinical relevance. Conclusions: The results of this study show benefits of an IMT in young Fontan patients. Even if some data are not statistically significant, they may still be clinically relevant and may contribute to a multidisciplinary approach in patient care. IMT should therefore be an additional target and integrated into the training program to improve the prognosis of Fontan patients. Trial Registration: German Clinical Trials Register; DRKS.de; registration ID: DRKS00030340.

Detection of early signs of right ventricular systolic impairment in unoperated Ebstein's anomaly by cardiac magnetic resonance feature tracking.

Background: Cardiovascular magnetic resonance feature-tracking analysis (CMR-FT) provides a quantitative assessment of myocardial contraction with potential for diagnostic and prognostic ability in a wide spectrum of diseases. Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve. However, it is also considered a disorder of development affecting the global right ventricular myocardium. Aim of our study is to describe the complex contractile mechanics of the functional right ventricle (RV) in patients affected by EA through CMR-FT. Methods: Fifty surgery-free EA patients who had undergone a complete CMR protocol at our institution between January 2017 and December 2020 were selected for the retrospective study. A historical control group of twenty-five healthy subjects was also included. CMR-FT analysis was performed at a dedicated workstation by manually tracing RV endo- end epicardial borders on steady-state-free-precession (SSFP) cine images. Strain values were calculated. Apical displacement of the tricuspid valve (TV) was measured on a 4-chamber cine image from the right atrio-ventricular junction to the functional annulus of the TV. Results: EA patients presented significantly impaired RV global radial strain (GRS) and global circumferential strain (GCS) compared to controls (P<0.0001 and P=0.0008, respectively). In a subgroup analysis, GRS was significantly compromised in patients with a severely displaced TV (>16 mm/m2) compared to milder forms (P=0.03) and to controls (P<0.0001). Among EA patients with a preserved ejection fraction, 12 (48%) vs. 6 (24%) controls had reduced both GRS and GCS. Conclusions: The contractile pattern of the functional RV in EA is characterised by prevalent alterations in the short-axis direction as indicated by reduced GRS and GCS. Strain values might be reduced prior to routine used functional parameters like RV ejection fraction (RVEF) and can possibly serve as an early predictor of myocardial dysfunction in EA patients.

Psychosocial well-being in postpartum women with congenital heart disease.

Background: Improved treatment options for congenital heart disease (CHD) lead to a growing number of women with CHD at reproductive age. Due to physical and psychological burden, pregnancies in women with CHD often count for high-risk. Resulting emotional distress can adversely impact pregnancy, motherhood and fetal health. The present study aims to retrospectively investigate mental outcomes and indices of adjustment in women with CHD before, during and after pregnancy. The novel concept of illness identity is applied to explain how patients experience and integrate their CHD into their identities. Methods: Patient-reported outcome measures on mental functioning and illness identity were assessed in a sample of 121 postpartum women with CHD [mean age: 42.7±9.2 (range, 27-81) years] at the German Heart Centre Munich between August and November 2021 in a cross-sectional design. Descriptive analyses, correlations and linear regression models were calculated. Results: Retrospectively assessed prevalence of emotional distress before giving birth was high (47.0%) and peaked shortly after childbirth in terms of elevated symptoms of postpartum depression and trauma. During the course of maternity, emotional distress decreased significantly (24.1%, P<0.001). Overall, postpartum women demonstrated high scores in functional illness identity states (i.e., acceptance and enrichment) and low scores in dysfunctional states (i.e., rejection and engulfment). CHD severity was not directly associated with mental outcomes (P>0.05), whereas maternal cardiovascular risk, according to the WHO classification, was significantly associated with a higher prevalence of postpartum trauma (t=2.485, P=0.015). Conclusions: Postpartum mental health problems, such as (postpartum) depression, anxiety, and posttraumatic stress can become a serious burden which might be detrimental to the mother's well-being and her infant's development. Present findings emphasise the urgent need for a holistic approach focusing on pregnant women with CHD starting at the prepartum stage to prevent adverse consequences and promote maternal well-being. Illness identity might become an important target construct for clinical practice as it may positively and enduringly influence mental well-being of pregnant women with CHD.

COVID-19 Infections in Adults with Congenital Heart Disease-A Prospective Single-Center Study in an Outpatient Setting.

BACKGROUND: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. METHODS: During the study period from January to June 2021, all consecutive outpatients from our ACHD clinic were eligible to participate. Clinical data were collected. An antibody test for COVID-19 was performed in all patients. Cardiovascular magnetic resonance imaging (CMR) was offered to those with a positive antibody test. RESULTS: Overall, 420 patients (44.8% female, mean age 36.4 ± 11.6 years) participated. Congenital heart defect (CHD) complexity was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. Altogether, 28 (6.7%) patients had a positive antibody test. Out of these, 14 had an asymptomatic course. The others had mainly mild symptoms and were managed as outpatients. Furthermore, 11 patients (39.3%) had even not been aware of their infection. Fourteen patients underwent a CMR without signs of myocardial involvement in any of them. CONCLUSIONS: We observed a number of undetected cases of COVID-19 infections in our ACHD population. Reassuringly, in all cases, the infection had a mild clinical course.