Abstract Background Tetralogy of Fallot (ToF) accounts for approximately 7 to 10 % of all CHD and is commonly associated with failure of the right ventricle (RV). Parameters providing more insight into the mechanics of RV failure are needed to guide clinical decision making. As advancements in Three-Dimensional (3D) echocardiography (3DE) have enabled more detailed analysis of RV volume and function, the way is paved for new 3D derived parameters. We developed a custom software to assess 3D RV shape and function. Purpose The aim of this study was to evaluate the differences of 3DE derived RV geometry (curvature) and deformation parameters in adults with ToF and controls. Methods In 50 patients with ToF and 50 controls 3DE RV images were acquired. RV geometries from 3D datasets were segmented using commercially available RV analysis software (TomTec 4D-RV function 2.0) and processed using custom software (RV-dynamics) to calculate Curvature, Area Strain (AS) and Longitudinal Strain (LS). Parameters were measured globally and over different segments of the RV (i.e. septal body (SB), inferior free wall (IFW), anterior free wall (AFW), lateral free wall (LFW), apex (Ax), RV inflow tract (RVIT), RV outflow tract (RVOT), anterior boundary (AB) and posterior boundary (PB)). Results Globally and for all independent regions of the RV, AS was significantly reduced in patients with ToF as compared with healthy controls. Except for AFW, global and all regional values of LS were significantly reduced in patients with ToF as compared with healthy controls. Global AS and LS were strongly correlated with RVEF with values for r of -0.9 and -0.6 respectively. RV curvature differed over multiple segments, predominantly in the end-diastolic Ax and end-systolic PB and Ax regions. Conclusion The RV-dynamics software gives the opportunity to analyze 3D RV morphology and RV deformation parameters in the diseased RV in more detail. AS and LS may provide more detailed assessment of the functional condition of the diseased RV in ToF whereas curvature provides an interesting method for the quantification of RV remodeling. Further insight in the different mechanisms of dysfunction of the RV may improve clinical decision making.

Aims:Tetralogy of Fallot (ToF) is a congenital heart disease requiring surgical repair in early childhood. Affected patients cope with severe right ventricle (RV)-related morbidity during adulthood, which is mostly attributed to RV remodelling. Improved understanding of RV remodelling patterns is essential for advancements in clinical decision-making and treatment planning. Consequently, a method to enable detailed quantification of directional components of RV function was developed using 3D echocardiography (3DE). Methods and results:From RV-focused 3DE studies of 50 ToF patients and 50 healthy controls, 3D dynamic RV meshes were obtained using commercially available software (TomTec 4D RV-Function). A software tool was developed in-house to decompose RV contraction in longitudinal (LT), radial, and anteroposterior (AP) motion directions. Their respective contributions to RV ejection fraction (EF) were calculated. The AP contraction component was identified as the main contributor in RV function deterioration, showing significant relative reduction in ToF patients compared with healthy controls [38% (31-42) vs. 40% (37-44), P = 0.004]. Conversely, the LT direction showed significant relative increase in ToF compared with healthy controls [28% (26-33) vs. 26% (23-31), P = 0.009]. Conclusion:Our custom software allowed for effective directional decomposition of RV EF, enabling detailed quantification of RV function in patients with ToF. RV function deterioration was primarily due to a decreased contribution of AP wall motion to RV EF, whereas LT contribution showed relative increase. These findings highlight the need for more detailed RV function assessment for improved understanding of RV remodelling.

OBJECTIVES:Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood. METHODS:Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968-1980 and were evaluated every decade since 1990. RESULTS:Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up. Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40-52) years. Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients. Reduced right and left ventricular ejection fraction (EF) was found in 33% and 13%, respectively. Exercise capacity and maximum rate of oxygen consumption were mildly impaired in 14% and 32% of patients. Patients who underwent an infundibulectomy during initial surgery were significantly more likely to undergo reintervention (HR=8.32, p=0.003). Patient-reported QoL scores remained stable over time and consistently exceeded those of the age-matched GDP. CONCLUSION:Fifty-year survival after surgery for pulmonary valve stenosis was excellent and comparable to the GDP. Most patients maintained preserved ventricular function, functional capacity and excellent QoL. Routine lifelong follow-up may not be necessary for all patients, but should be considered for those who underwent an infundibulectomy or have residual lesions.

Tetralogy of Fallot (ToF) accounts for approximately 7–10

BACKGROUND:Cardiac resynchronisation therapy (CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV. METHODS:This is an international, retrospective study including patients >18 years from 33 centres with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA. The primary endpoint included overall survival and survival free from HF. The secondary endpoint was a composite of death, hospitalisation for HF, heart transplant, mechanical support and ventricular tachycardia/implantable cardioverter-defibrillator therapies. RESULTS:We identified 105 out of 1721 patients (3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 (1.6-8) years. QRS improvement was limited to those with previous pacing (167±35 vs 154±28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peak VO2 and tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though they were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, anatomy, presence of complex disease, previous HF and sRV dysfunction at baseline. At univariable analysis, CRT (HR 4.39-95%, CI 1.6 to 11.9; p=0.003), older age and moderate-to-severe sRV dysfunction at baseline were predictive of death, while CRT (HR 3-95%, CI 1.3 to 7; p=0.01) and sRV dysfunction were associated with HF admission. By multivariable analysis, CRT (HR 8.8-95%, CI 2.9 to 26.6; p=0.0001) and age (HR 1.1%-95%, CI 1.01 to 1.15; p<0.0001) were independently associated with poorer outcome. CONCLUSION:In this retrospective study in the largest population thus far described with an sRV, CRT implant was not associated with improved survival, even after controlling for key confounders.

BACKGROUND:Little is known regarding pulmonary hypertension (PH) in adults with a systemic right ventricle. OBJECTIVES:This study evaluates the hemodynamic profile of PH in patients with transposition of great arteries palliated with an atrial switch repair (TGA-AS) and congenitally corrected TGA (CCTGA). METHODS:This was a retrospective cohort study of adults with TGA-AS or CCTGA who had undergone invasive hemodynamic assessment. Exclusion criteria were single ventricle anatomy, previous Rastelli, arterial switch, or double switch operation. PH was defined by a mean pulmonary artery pressure (mPAP) >20 mm Hg and subtypes according to 2022 European Society of Cardiology guidelines. The primary combined clinical endpoint was death, heart transplantation, or need for mechanical circulatory support. RESULTS:A total of 261 patients were studied (TGA-AS, n = 161 and CCTGA, n = 100). PH prevalence was similar in both groups (65% vs 69%, P = 0.74). PH subtype was precapillary in 24%, isolated postcapillary (IpcPH) 25%, combined postcapillary and precapillary 51% (similar in both groups). The relationship between pulmonary capillary wedge pressure and mPAP was overall similar between 2 groups but there was greater variability in pulmonary capillary wedge pressure once mPAP >40 mm Hg in the TGA-AS group. The incidence of the primary endpoint was similar in both groups (24 v 29%; P = 0.39). Factors associated with the primary clinical endpoint were elevated PVR (in particular, in the TGA-AS subgroup) and lower aortic pulsatility index (both groups). CONCLUSIONS:In this large study evaluating the hemodynamic phenotype in patients with a systemic RV referred for catheterization, PH was common and most commonly the combined postcapillary and precapillary subtype.

Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arteries. Progressively increasing pulmonary artery pressure may lead to fatal failure of the right ventricle, necessitating aggressive drug treatment. Sotatercept, a novel drug for PAH-targeted therapy, shows promising results in clinical trials. Nevertheless, efficacy in the heterogeneous PAH population and long-term outcomes have yet to be investigated. This case report illustrates the life-changing effects of sotatercept in a patient with heritable PAH associated with a TBX4 mutation. Our patient faced near immobility, oxygen dependency, and was listed for lung transplantation in November 2023. Following sotatercept administration from July 2024 onward, she started sporting activities and has been removed from the transplantation waiting list. Thereby, this case illustrates an exceptional response to sotatercept in a patient with TBX4-associated heritable PAH. Positive effects persist 1 year after initiation without significant side effects.

Pregnancy in women with rare vascular diseases is highly challenging, as it can be associated with significant maternal and foetal risks, requiring complex and multidisciplinary management. Care across countries remains insufficiently characterized, and both patients and healthcare providers across Europe highlight the need for better structures and organization to support this critical and potentially life-threatening phase. To describe current practices, perspectives, and challenges faced by patients and healthcare professionals in the management of pregnancy in women with rare vascular diseases. A survey developed by the European Reference Networks (ERN) pregnancy working group was extended with VASCERN-specific questions, resulting in a 13-item questionnaire. The survey was distributed to healthcare providers and patient representatives (ePAGs). Qualitative data from open-ended responses were analysed using an inductive thematic approach. 36 responses were collected from 145 invited VASCERN members, resulting in a 25

INTRODUCTION:Reduced exercise performance is common in patients with repaired tetralogy of Fallot (rToF) and relates to adverse outcomes. The effects of high-intensity interval training (HIIT) in rToF are unknown. Furthermore, it is unknown if home-based, online-monitored exercise training is effective in rToF. We aimed to examine the feasibility, safety and effects of home-based HIIT in patients with rToF. METHODS:Patients aged 12-30 years (n=34, median age 16 (IQR 14-23)) with rToF were randomised to a 12-week home-based HIIT programme or standard of care (SOC). HIIT was prescribed in three 30 min sessions/week, with heart rate telemonitoring. SOC included exercise as usual. Co-primary end points were mean differences in peak oxygen consumption (VO2) and maximal workload, analysed using linear mixed-effects (LME) models. Secondary end points were: echocardiography, ECG and MRI-derived measures of cardiac structure and function. RESULTS:No serious adverse events occurred. After HIIT, peak VO2 increased from 38.3 (34.0-42.5) mL/kg/min to 40.9 (36.6-45.2) mL/kg/min (∆ 4.0 mL/kg/min, compared with control period (95% CI 1.52 to 6.53)), workload increased from 204 (174-234) W to 218 (188-248) W (∆ 17 W, compared with control period (95% CI 5 to 30)). No changes in cardiac volumes and ejection fraction were observed after HIIT. VO2 improved after HIIT independent of baseline fitness, body mass index and biventricular size. Notably, lower right ventricular ejection fraction (RV EF) was associated with greater improvement. CONCLUSION:HIIT significantly enhances exercise capacity in adolescents and young adults with rToF without adverse effects on cardiac structure or function, although the sample size limits the detection of subtle changes and can be successfully performed at home with online monitoring. This intervention offers a feasible alternative to traditional, on-site supervised exercise programmes, for patients with rToF, including those with lower RV EF. TRIAL REGISTRATION NUMBER:NTR2731.

OBJECTIVE:Regular physical exercise has well-known health benefits and is generally considered safe for adults with congenital heart disease (ACHD). However, many individuals with ACHD remain insufficiently physically active. This study explored the barriers and needs related to physical exercise as experienced by people with ACHD to inform the development of tailored strategies that support and promote increased physical activity. METHODS:Qualitative study using semistructured interviews conducted between March and May 2023. The interview guide was based on the Fear Avoidance Model, Tampa Scale for Kinesiophobia Heart and European Society of Cardiology guidelines on sports cardiology and exercise for cardiovascular diseases. Interviews were coded and thematically analysed to identify specific physical exercise barriers and needs. RESULTS:Data saturation was reached after interviewing 19 individuals living with ACHD (median age 46 years (range 24-75), 10 women). Thematic analysis identified four main barriers: (1) physical symptoms and negative past experiences, (2) alienation from peers, (3) perceived decline in physical fitness over time and (4) lack of knowledge about personal physical boundaries. Two needs were identified: (1) personalised, disease-specific exercise information and advice and (2) structured support and guidance from healthcare professionals. CONCLUSIONS:People with ACHD face multiple barriers to engaging in physical exercise. There is a clear need for specific, personalised exercise advice from healthcare providers and the development of long-term programmes and interventions to overcome relevant barriers.

Background: Little is known about the very long-term outcome in Tetralogy of Fallot (ToF) patients. Objectives: To prospectively evaluate clinical outcome and quality-of-life after surgical repair of ToF. Methods: Single-centre, longitudinal cohort-study evaluating every decade 144 ToF patients who underwent surgical repair <15 years of age between 1968 and 1980. Results: Evaluated were 66 patients (92 %) of the 72 eligible survivors (58 % male, median age at study 48.5 years) with a median follow-up of 45 (range 39-52) years. Cumulative survival at 50 years was 71 % and 84 % when excluding 30-days mortality, while event-free survival was 9 %. Reintervention (40 %) and symptomatic arrhythmias (21 %) were the most common complications, although ventricular tachycardia (VT) was rare (7 %). Cardiac magnetic resonance imaging showed a right ventricular ejection fraction <45 % in 45 % of the cohort and 42 % had a diminished left ventricular function. The aortic root diameter increased over time to >40 mm in 45 % of patients. VO(2)max was reduced in 53 % of patients but stable over time. Self-perceived quality-of-life was stable and comparable to the general Dutch population. Early post-operative arrhythmias, pre-operative low oxygen saturation of the left atrium, VT and declining exercise capacity over time were predictive for mortality, transannular patch for both arrhythmia and pulmonary valve replacement. Surgery post-1975 was protective for pulmonary valve replacement. Conclusion: Only 9 % of ToF patients is alive without a major event at 50 years after surgical repair. Reintervention and symptomatic arrhythmias are especially common, although symptomatic VT is rare. Exercise capacity and self-perceived quality-of-life remained stable.

Objectives:To evaluate survival, clinical outcome and quality of life (QoL) of patients up to 49 years after surgical ventricular septal defect (VSD) closure. Methods:Single-center, longitudinal cohort study evaluating consecutive patients with surgical VSD closure between 1968 and 1980 with extensive cardiac and QoL evaluation every decade. Results:Of the original cohort of 174 patients, 39 died (22 %), 8 were lost to follow-up and 38 had not participated previously. Survival rate at 49 years follow-up was 77 % and 86 % when excluding early postoperative mortality. Of the 89 eligible survivors, 76 (85 %) were evaluated (59 % male, median age 49 [44-54] years) with a median follow-up of 44 (range 40-49) years after surgery. Event-free survival at 49 years was 50 %, with symptomatic arrhythmias (10 %), pacemaker implantation (8 %) and VSD-related interventions (3 %) being common complications. At last follow-up, 58 % had left atrial dilation, 25 % had aortic regurgitation and 5 patients (7 %) had a residual VSD. Early postoperative arrhythmias predicted mortality. Both left ventricular (LV) and right ventricular ejection fraction remained stable, with only 1 % having an LV ejection fraction below 45 % at last follow-up. Exercise capacity and VO2max were mildly reduced in 33 % and 49 % of the patients while self-perceived QoL was stable and comparable with the general Dutch population. Conclusion:Half of the patients with surgical VSD closure had an event-free survival at 49 years. Pacemaker implantation was often needed. Early postoperative arrhythmias predicted mortality. QoL was good and remained stable over time.

BACKGROUND:Adults with transposition of the great arteries (TGA) and systemic morphologic right ventricle (SRV) have a high prevalence of SRV failure with accompanying tricuspid valve regurgitation (TR). It is unknown if tricuspid valve surgery (TVS) impacts clinical outcomes in this setting. METHODS:Data were gathered from an international cohort of patients with TGA and SRV. From this group, patients who had previously undergone tricuspid valve surgery (TVS) were propensity matched with patients who had not undergone surgery and followed for 10.5 ± 4.6 years. The impact of surgery on the probability of the combined endpoint of death/transplant/MCS was investigated. Variables associated with favorable outcomes were explored. RESULTS:Among 2562 patients with TGA and SRV, 140 patients with prior TVS were propensity matched to 140 controls without previous TVS. In the TVS group, primary end point was noted in 20 cases with 15 deaths (10.7 %), 3 transplant listings (2.1 %) and 5 MCS implants (3.6 %). Among controls, primary end point occurred in 23 patients (16.4 %) with 15 deaths (10.7 %), 7 transplant listings (5.0 %) and 2 MCS implants (1.4 %). There was no difference between groups in event free survival. Lower rates of combined endpoint were observed in TVS group with moderate-severe TR and mild-moderate SRV dysfunction. Sensitivity analyses to address potential immortal-time bias were performed in the subset who underwent surgery during the observation period and produced similar results. Among patients with severe SRV dysfunction there was no difference in primary outcome between groups. Similarly, no difference in outcomes was observed between congenitally corrected (ccTGA) and atrial corrected transposition of the great arteries (acTGA) patients. CONCLUSIONS:For acTGA and ccTGA patients with SRV and moderate-severe TR, surgical intervention prior to development of severe RV dysfunction is recommended. More data are required to determine those with severe SRV dysfunction who may benefit most.

OBJECTIVE:We aim to increase knowledge on pregnancy and delivery risks in vascular Ehlers-Danlos Syndrome (vEDS). Our outcomes can contribute to establishing future guidelines for pregnancy and delivery management in women with vEDS. DESIGN:Retrospective multicentre cohort study. SETTING:Women with vEDS due to pathogenic/likely pathogenic (P/LP) COL3A1 variants are at increased risk for arterial dissection and pregnancy-related complications during pregnancy and delivery. POPULATION:Women with a P/LP COL3A1 variant were included from 2019 until 2021. METHODS:Genetic and clinical data was collected through retrospective analysis. MAIN OUTCOME MEASURES:Description of the genotype and pregnancy-related outcomes. RESULTS:We collected information about 121 pregnancies of 43 women with vEDS, including nine women with a haploinsufficient variant. Neither uterine rupture nor life-threatening or fatal vascular events occurred in the perinatal period. The miscarriage rate was 19% (23/121) and 19.1% of the live births were preterm (18/94). Miscarriages were significantly more frequent in women with a glycine substitution in COL3A1 compared to other COL3A1 variant types (19/23, 82.6%, p = 0.018). Thirty-four women had a vaginal birth (79.1%), including 1/7 with known vEDS. Eight deliveries were complicated by severe perineal tears, and six by postpartum haemorrhage. CONCLUSIONS:No pregnancy-related deaths, arterial dissections or uterine ruptures occurred in our cohort. Since no life-threatening events occurred during pregnancy and delivery, discouragement of pregnancy in all women with vEDS in current guidelines might be too strict. Based on these data, we propose a shared decision-making process.

Data on the use of mechanical circulatory support devices in pregnant women are limited. A 27-year-old woman at 27 weeks and 6 days of gestation was supported by three different mechanical circulatory support devices due to cardiogenic shock. She came into spontaneous labor, which was complicated by major hemorrhage at the cannulation site, fetal distress, and transverse position, requiring emergency cesarean section. The postpartum period was complicated by intra-abdominal bleeding and arterial occlusion of lower extremity. When using mechanical circulatory support devices in pregnant women, a multidisciplinary approach is recommended.

BACKGROUND Concerns remain regarding the high incidence of morbidity in transposition of the great arteries (TGA) patients who underwent Mustard repair. OBJECTIVES The purpose of this study was to evaluate the long-term outcome and quality of life of patients with TGA up to 48 years. METHODS Single-center, longitudinal cohort study evaluating consecutive patients with TGA who underwent Mustard repair between 1973 and 1980 at young age with extensive cardiac and subjective quality of life evaluation every decade. RESULTS Of the original cohort of 91 patients, 31 died and 2 underwent heart transplantation. Cumulative survival at 48 years was 61% (51% to 73%), with a shift in cause of death from sudden death to heart failure (HF) after 35 years of follow-up. Of the 44 eligible survivors, 34 (77%) were evaluated (71% male, median age 46 years) with a median followup of 46 (range: 40-48) years. Event-free survival was 14%, with cardiac reintervention (39%), symptomatic arrhythmia (39%), and HF (37%) being the most common events. Systemic right ventricular function (sRVF) declined over time, with 73% having an ejection fraction below 40%, and 95% having a reduced VO2max. Prolonged QRS duration was found to be a predictor for HF, early postoperative arrhythmias for late symptomatic arrhythmias, and a reduced sRVF for mortality. Self-perceived quality of life was good and remained stable over time. CONCLUSIONS Mortality and morbidity after Mustard repair are substantial, with an event-free survival of only 14% at 48 years. The clinical condition deteriorated over time, necessitating timely consideration of advanced treatment options. (JACC Adv. 2025;4:101984) (c) 2025 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY license (http://creativecommons.org/licenses/ by/4.0/).

Background Higher resting heart rate has been described as a risk factor for adverse outcome in healthy individuals and cardiovascular patients. The aim of this study was to evaluate resting heart rate as risk factor in adult congenital heart disease (ACHD). Methods In this prospective observational cohort study, patients with moderate or complex ACHD were included at routine outpatient visit. Standard 12-lead ECGs were obtained in rest. Heart rate was obtained from the ECG automatically by the Modular ECG Analysis System (MEANS). The primary endpoint was all-cause mortality and the secondary endpoint was a composite of all-cause mortality and heart failure. Survival was derived using the Kaplan-Meier estimator. Subgroups based on heart rate tertiles were compared by the log-rank test. Cox proportional hazards models were adjusted for clinical factors including age, sex and diagnosis (moderate vs complex ACHD). Results A total of 556 patients were included (median age 32 years (IQR 24–41), 57.6% male). Mean heart rate was 69±13 bpm. Negative chronotropic medication was used by 74 (13.3%) patients. During a median follow-up of 10.1 (IQR 9.6–10.5) years, 36 patients (6.5%) died and 83 (14.9%) reached the secondary endpoint. Patients with higher heart rates had significantly lower survival and heart failure-free survival. After adjusting for clinical factors, heart rate remained associated with mortality (HR 1.57 per 10 bpm, 95% CI 1.26 to 1.96) and mortality or heart failure (HR 1.33 per 10 bpm, 95% CI 1.13 to 1.57). Conclusion Higher heart rate is associated with lower survival and heart failure-free survival in ACHD.

Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.

Abstract Background Low levels of physical activity is a common concern among adults with congenital heart disease (ACHD). Fear of movement, also known as kinesiophobia, has been suggested as an important contributor to these low levels of physical activity in the general cardiovascular disease population. However, levels of kinesiophobia have not been investigated in ACHD patients. Objective To investigate kinesiophobia prevalence in ACHD patients, and to identify potential predictors of fear of movement. Second, we investigated the impact of kinesiophobia on physical activity levels. Methods ACHD patients from two Dutch university hospitals participated in this study by completing a series of questionnaires, including the Tampa Scale for Kinesiophobia Heart (TSK-NL Heart). The TSK-NL Heart, with a scoring range from 13 to 52, identifies scores ≥29 as indicative of high levels of kinesiophobia. Baseline characteristics, including age, sex, NYHA class, number of cardiac surgeries, prior participation in cardiac rehabilitation, educational level, and participation in physical activity were self-reported. Descriptive statistics were used to determine prevalence of high levels of kinesiophobia. Logistic regression was used to identify factors associated with high levels of kinesiophobia and to assess its impact on physical activity (corrected for age and sex). Results A total of 794 ACHD patients (50% female, aged 45 (+/- 14) years) completed all questionnaires, whereof 232 patients (29%) reported high levels of kinesiophobia. A total of 494 patients (62%) reported to be in NYHA class 1, 249 patients (31%) in NYHA class 2, 46 patients (6%) in NYHA class 3 and 5 patients (1%) in NYHA class 4. Men were more likely to experience kinesiophobia (OR 1.53, 95% CI 1.09-2.15). Higher self-reported NYHA class was associated with increased likelihood of kinesiophobia (NYHA class 2 vs 1: OR 2.92, 95% CI 2.03-4.20; and NYHA class 3 or 4 vs 1: OR 12.61, 95% CI 6.24-25.47, see Figure). A high level of kinesiophobia decreased the probability of being physically active for at least 30 min/day during 5 days/week (OR 0.66, 95% CI 0.47-0.97). Conclusion This study reveals a considerable prevalence of kinesiophobia among ACHD patients. High levels of kinesiophobia were associated with lower levels of physical activity. The identified predictors, including sex and NYHA class, provide valuable insights for designing targeted interventions aimed at mitigating kinesiophobia and promoting increased physical activity in ACHD patients. Determinants of Fear of Movement.