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Guideline-directed medical therapy (GDMT) has clear benefits on morbidity and mortality in patients with heart failure; however, GDMT use remains low. In the multicenter, open-label, investigator-initiated ADMINISTER trial, patients (n = 150) diagnosed with heart failure and reduced ejection fraction (HFrEF) were randomized (1:1) to receive usual care or a strategy using digital consults (DCs). DCs contained (1) digital data sharing from patient to clinician (pharmacotherapy use, home-measured vital signs and Kansas City Cardiomyopathy Questionnaires); (2) patient education via a text-based e-learning; and (3) guideline recommendations to all treating clinicians. All remotely gathered information was processed into a digital summary that was available to clinicians in the electronic health record before every consult. All patient interactions were standardly conducted remotely. The primary endpoint was change in GDMT score over 12 weeks (ΔGDMT); this GDMT score directly incorporated all non-conditional class 1 indications for HFrEF therapy with equal weights. The ADMINISTER trial met its primary outcome of achieving a higher GDMT in the DC group after a follow-up of 12 weeks (ΔGDMT score in the DC group: median 1.19, interquartile range (0.25, 2.3) arbitrary units versus 0.08 (0.00, 1.00) in usual care; P < 0.001). To our knowledge, this is the first multicenter randomized controlled trial that proves a DC strategy is effective to achieve GDMT optimization. ClinicalTrials.gov registration: NCT05413447 ., (© 2024. The Author(s).)

This paper addresses the challenge of integrating wearable activity trackers into cardiovascular disease care. Despite evidence supporting the use of trackers for monitoring and promoting physical activity, implementation challenges persist in clinical settings. The paper emphasises the lack of systematic, evidence-based implementation approaches for integrating trackers. It underscores the urgent need for stakeholder collaboration between clinicians, patients, implementation scientists, researchers, health and technology partners, and the use of proven implementation science methodologies. This is crucial for bridging the gap and ensuring effective translation of wearable activity trackers into cardiovascular disease care, meeting the increasing demand from patients and clinicians., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Aims: Many heart failure (HF) patients do not receive optimal guideline-directed medical therapy (GDMT) despite clear benefit on morbidity and mortality outcomes. Digital consults (DCs) have the potential to improve efficiency on GDMT optimization to serve the growing HF population. The investigator-initiated ADMINISTER trial was designed as a pragmatic multicenter randomized controlled open-label trial to evaluate efficacy and safety of DC in patients on HF treatment., Methods and Results: Patients (n = 150) diagnosed with HF with a reduced ejection fraction will be randomized to DC or standard care (1:1). The intervention group receives multifaceted DCs including (i) digital data sharing (e.g. exchange of pharmacotherapy use and home-measured vital signs), (ii) patient education via an e-learning, and (iii) digital guideline recommendations to treating clinicians. The consults are performed remotely unless there is an indication to perform the consult physically. The primary outcome is the GDMT prescription rate score, and secondary outcomes include time till full GDMT optimization, patient and clinician satisfaction, time spent on healthcare, and Kansas City Cardiomyopathy Questionnaire. Results will be reported in accordance to the CONSORT statement., Conclusions: The ADMINISTER trial will offer the first randomized controlled data on GDMT prescription rates, time till full GDMT optimization, time spent on healthcare, quality of life, and patient and clinician satisfaction of the multifaceted patient- and clinician-targeted DC for GDMT optimization., (© 2023 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Remote monitoring devices for atrial fibrillation are known to positively contribute to the diagnostic process and therapy compliance. However, automatic algorithms within devices show varying sensitivity and specificity, so manual double-checking of electrocardiographic (ECG) recordings remains necessary., Objective: The purpose of this study was to investigate the validity of the KardiaMobile algorithm within the Dutch telemonitoring program (HartWacht)., Methods: This retrospective study determined the diagnostic accuracy of the algorithm using assessments by a telemonitoring team as reference. The sensitivity, specificity, negative predictive value (NPV), positive predictive value (PPV), and F1 scores were determined., Results: A total of 2298 patients (59.5% female; median age 57 ± 15 years) recorded 86,816 ECGs between April 2019 and January 2021. The algorithm showed sensitivity of 0.956, specificity 0.985, PPV 0.996, NPV 0.847, and F1 score 0.976 for the detection of sinus rhythm. A total of 29 false-positive outcomes remained uncorrected within the same patients. The algorithm showed sensitivity of 0.989, specificity 0.953, PPV 0.835, NPV 0.997, and F1 score 0.906 for detection of atrial fibrillation. A total of 2 false-negative outcomes remained uncorrected., Conclusion: Our research showed high validity of the algorithm for the detection of both sinus rhythm and, to a lesser extent, atrial fibrillation. This finding suggests that the algorithm could function as a standalone instrument particularly for detection of sinus rhythm., (© 2023 Heart Rhythm Society.)

Background: Various electrocardiogram (ECG)-based devices are available for home monitoring, but the reliability in adults with CHD is unknown. Therefore, we determined the accuracy of different ECG-based devices compared to the standard 12-lead ECG in adult CHD., Methods and Results: This is a single-centre, prospective, cross-sectional study in 176 consecutive adults with CHD (54% male, age 40 ± 16.6 years, 24% severe CHD, 84% previous surgery, 3% atrial fibrillation (AF), 24% right bundle branch block). Diagnostic accuracy of the Withings Scanwatch (lead I), Eko DUO (precordial lead), and Kardia 6L (six leads) was determined in comparison to the standard 12-lead ECG on several tasks: 1) AF classification (percentage correct), 2) QRS-morphology classification (percentage correct), and 3) ECG intervals calculation (QTc time ≤ 40 ms difference). Both tested AF algorithms had high accuracy (Withings: 100%, Kardia 6L: 97%) in ECGs that were classified. However, the Withings algorithm classified fewer ECGs as inconclusive (5%) compared to 31% of Kardia (p < 0.001). Physician evaluation of Kardia correctly classified QRS morphology more frequently (90% accuracy) compared to Eko DUO (84% accuracy) (p = 0.03). QTc was underestimated on all ECG-based devices (p < 0.01). QTc duration accuracy was acceptable in only 51% of Withings versus 70% Eko and 74% Kardia (p < 0.001 for both comparisons)., Conclusions: Although all devices demonstrated high accuracy in AF detection, the Withings automatic algorithm had fewest uninterpretable results. Kardia 6L was most accurate in overall evaluation such as QRS morphology and QTc duration. These findings can inform both patients and caregivers for optimal choice of home monitoring.

The number of inherited heart disease (IHD) studies using artificial intelligence (AI) has increased rapidly over the last years. In this scoping review, we aimed to present an overview of the current literature available on the applicability of AI within the field of IHD. The literature search resulted in eighteen articles in which an AI model was trained and tested, mostly for diagnostic and predictive purposes. The areas under the receiver operating characteristic curves ranged from 0.78-0.96, but varied between IHD types, used methods and outcome measures. Only three out of eighteen did perform validation on an external dataset and most studies did not use explainable deep learning models. To be able to integrate AI as a tool to aid physicians in their diagnoses and clinical decisions within the IHD field, generalizability has to be better evaluated and explainability of DL models has to be increased., (Copyright © 2022. Published by Elsevier Inc.)

Background: Chest CT displays chest pathology better than chest X-ray (CXR). We evaluated the effects on health outcomes of replacing CXR by ultra-low-dose chest-CT (ULDCT) in the diagnostic work-up of patients suspected of non-traumatic pulmonary disease at the emergency department., Methods: Pragmatic, multicentre, non-inferiority randomised clinical trial in patients suspected of non-traumatic pulmonary disease at the emergency department. Between 31 January 2017 and 31 May 2018, every month, participating centres were randomly allocated to using ULDCT or CXR. Primary outcome was functional health at 28 days, measured by the Short Form (SF)-12 physical component summary scale score (PCS score), non-inferiority margin was set at 1 point. Secondary outcomes included hospital admission, hospital length of stay (LOS) and patients in follow-up because of incidental findings., Results: 2418 consecutive patients (ULDCT: 1208 and CXR: 1210) were included. Mean SF-12 PCS score at 28 days was 37.0 for ULDCT and 35.9 for CXR (difference 1.1; 95% lower CI: 0.003). After ULDCT, 638/1208 (52.7%) patients were admitted (median LOS of 4.8 days; IQR 2.1-8.8) compared with 659/1210 (54.5%) patients after CXR (median LOS 4.6 days; IQR 2.1-8.8). More ULDCT patients were in follow-up because of incidental findings: 26 (2.2%) versus 4 (0.3%)., Conclusions: Short-term functional health was comparable between ULDCT and CXR, as were hospital admissions and LOS, but more incidental findings were found in the ULDCT group. Our trial does not support routine use of ULDCT in the work-up of patients suspected of non-traumatic pulmonary disease at the emergency department., Trial Registration Number: NTR6163., Competing Interests: Competing interests: All authors have completed and submitted the ICMJE form for Disclosure of Potential Conflicts of Interest. JS reported receipt of an innovation grant of the Amsterdam UMC, location AMC, University of Amsterdam and a grant of Health Care Efficiency Program of the Netherlands Organization for Health Research and Development (ZonMW:843001806). MMNPK reports to have received personal fees from the before-mentioned grants. SM reports to have received grants and personal fees from Bayer, BMS Pfizer, Boehringer Ingelheim, Daiichi Sankyo, Portola, GSK and Aspen. TSRvE reports to have received a personal fee, PhD Scholarship grant, from Amsterdam UMC., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The Apple Watch (AW) is the first commercially available wearable device with built-in electrocardiogram (ECG) electrodes to perform a single-lead ECG to detect atrial fibrillation (AF)., Methods: Patients with AF who were scheduled for electrical cardioversion (ECV) were included in this study. The AW ECGs were obtained pre-ECV and post-ECV. In case of an unclassified recording, the AW ECG was obtained up to 3 times. The 12-lead ECG was used as the reference standard. Sensitivity, specificity, and kappa coefficient were calculated., Results: In total, 74 patients were included. Mean age was 67.1 ± 12.3 years and 20.3% were female. In total 65 AF and 64 sinus rhythm measurements were obtained. The first measurement with the AW showed a sensitivity of 93.5% and specificity of 100% (κ = 0.94). A second measurement resulted in a sensitivity of 94.6% and specificity of 100% (κ = 0.95). A third measurement resulted in a sensitivity of 93% and a specificity of 96.5% (κ = 0.90). Adjudication of unclassified recordings by a physician reduced the total unclassified recordings from 27.9% to 1.6%, but also reduced the accuracy. The kappa coefficient for unclassified single-lead ECGs was 0.58., Conclusion: The single-lead ECG of the AW shows a high accuracy for identifying AF in a clinical setting. Repeating the recording once decreases the total of unclassified recordings; however, a third recording resulted in a lower accuracy and the occurrence of false-positive measurements. Unclassified results of the AW can be reduced by physicians' interpretation of the single-lead ECG; however, the interrater agreement is only moderate., (© 2022 Published by Elsevier Inc. on behalf of Heart Rhythm Society.)

Cardiac exercise stress testing (CEST) is an important diagnostic tool in daily cardiology practice. However, during intense physical activity microdroplet aerosols, potentially containing SARS-CoV-2 particles, can persist in a room for a long time. This poses a potential infection risk for the medical staff involved in CEST, as well as for the patients entering the same room afterwards. We measured aerosol generation and persistence, to perform a risk assessment for SARS-CoV-2 transmission through aerosols during CEST. We find that during CEST, the aerosol levels remain low enough that SARS-CoV-2 transmission through aerosols is unlikely, with the room ventilation system producing 14 air changes per hour. A simple measurement of CO 2 concentration gives a good indication of the ventilation quality., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Author(s).)

Background: eHealth programs can lower blood pressure but also drive healthcare costs. This study aims to review the evidence on the effectiveness and costs of eHealth for hypertension and assess commonalities in programs with high effect and low additional cost., Results: Overall, the incremental decrease in systolic blood pressure using eHealth, compared to usual care, was 3.87 (95% confidence interval (CI) 2.98-4.77) mmHg at 6 months and 5.68 (95% CI 4.77-6.59) mmHg at 12 months' follow-up. High intensity interventions were more effective, resulting in a 2.6 (95% CI 0.5-4.7) (at 6 months) and 3.3 (95% CI 1.4-5.1) (at 12 months) lower systolic blood pressure, but were also more costly, resulting in €170 (95% CI 56-284) higher costs at 6 months and €342 (95% CI 128-556) at 12 months. Programs that included a high volume of participants showed €203 (95% CI 99-307) less costs than those with a low volume at 6 months, and €525 (95% CI 299-751) at 12 months without showing a difference in systolic blood pressure. Studies that implemented eHealth as a partial replacement, rather than addition to usual care, were also less costly (€119 (95% CI -38-201 at 6 months) and €346 (95% CI 261-430 at 12 months)) without being less effective. Evidence on eHealth programs for hypertension is ambiguous, heterogeneity on effectiveness and costs is high (I2 = 56-98%)., Conclusion: Effective eHealth with limited additional costs should focus on high intensity interventions, involve a large number of participants and use eHealth as a partial replacement for usual care., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Aims: There is limited quantitative evidence on the effect of symptom-driven telemonitoring for cardiac arrhythmias on patient-reported outcomes. We evaluated the effect of a symptom-driven remote arrhythmia monitoring programme on the patient-reported health-related quality of life (HRQoL), sense of safety, physical limitations, and self-management., Methods and Results: This was an observational retrospective longitudinal study of the symptom-driven HartWacht-telemonitoring programme using a remote single-lead electrocardiogram monitoring system. Real-world patient data from participants who were enrolled in the telemonitoring programme for (suspected) symptomatic atrial fibrillation (AF) between July 2017 and September 2019 were evaluated. Primary outcomes were the patient-reported generic HRQoL, disease-specific HRQoL, sense of safety, physical limitations, and self-management at date of enrolment, 3 months and 6 months of follow-up. Outcomes were compared to a historical control group consisting of AF patients receiving standard care. A total of 109 participants in the HartWacht programme [59 men (54%); mean age 61 ± 11 years; 72% diagnosed AF] were included in complete case analysis. There was no significant change in HRQoL and sense of safety during follow-up. A significant improvement in the perceived physical limitations was observed. The level of self-management declined significantly during follow-up. Comparisons to the historic control group ( n  = 83) showed no difference between the patient-reported disease-specific HRQoL, sense of safety and physical limitations at 6 months of follow-up., Conclusion: Symptom-driven remote arrhythmia monitoring for AF does not seem to affect HRQoL and sense of safety, whereas the perceived physical limitations tend to improve. Patient-reported self-management declined during the first 6 months of participation., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Introduction: An increased focus on shared decision-making and patient empowerment in cardiology and on patient outcomes such as quality of life (QoL), depression, and anxiety underline the importance of high-quality patient education. Studies focusing on digital means of patient education performed in other disciplines of medicine demonstrated its positive effect in these areas. Therefore, a review of the current literature was performed to (i) evaluate the status of innovative, digitalized means of patient education in cardiology and (ii) assess the impact of digital patient education on outcome parameters (i.e., patient knowledge (or health literacy), QoL, depression, anxiety, and patient satisfaction)., Method: A review of the current literature was performed to evaluate the effect of digitalized patient education for any purpose in the field of cardiology. Medline and EMBASE were searched for articles reporting any digital educational platform used for patient education up to May 2020. The articles were compared on their effect on patient knowledge or health literacy, QoL, depression or anxiety, and patient satisfaction., Results: The initial search yielded 279 articles, 34 of which were retained after applying in, and exclusion criteria. After full-text analysis, the total number of articles remaining was 16. Of these, 6 articles discussed the use of smartphone or tablet applications as a means of patient education, whereas 3 reviewed web-based content, and 7 evaluated the use of video (2 three-dimensional videos, from which one on a virtual reality headset)., Conclusion: This review demonstrates that digital patient education increases patient knowledge. Overall, digital education increases QoL and lowers feelings of depression and anxiety. The majority of patients express satisfaction with digital platforms. It remains important that developers of digital patient education platforms remain focused on clear, structured, and comprehensible information presentation., (© 2021 The Author(s). Published by S. Karger AG, Basel.)

Background: Phospholamban (PLN) p.Arg14del mutation carriers are known to develop dilated and/or arrhythmogenic cardiomyopathy, and typical electrocardiographic (ECG) features have been identified for diagnosis. Machine learning is a powerful tool used in ECG analysis and has shown to outperform cardiologists., Objectives: We aimed to develop machine learning and deep learning models to diagnose PLN p.Arg14del cardiomyopathy using ECGs and evaluate their accuracy compared to an expert cardiologist., Methods: We included 155 adult PLN mutation carriers and 155 age- and sex-matched control subjects. Twenty-one PLN mutation carriers (13.4%) were classified as symptomatic (symptoms of heart failure or malignant ventricular arrhythmias). The data set was split into training and testing sets using 4-fold cross-validation. Multiple models were developed to discriminate between PLN mutation carriers and control subjects. For comparison, expert cardiologists classified the same data set. The best performing models were validated using an external PLN p.Arg14del mutation carrier data set from Murcia, Spain (n = 50). We applied occlusion maps to visualize the most contributing ECG regions., Results: In terms of specificity, expert cardiologists (0.99) outperformed all models (range 0.53-0.81). In terms of accuracy and sensitivity, experts (0.28 and 0.64) were outperformed by all models (sensitivity range 0.65-0.81). T-wave morphology was most important for classification of PLN p.Arg14del carriers. External validation showed comparable results, with the best model outperforming experts., Conclusion: This study shows that machine learning can outperform experienced cardiologists in the diagnosis of PLN p.Arg14del cardiomyopathy and suggests that the shape of the T wave is of added importance to this diagnosis., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Adults with congenital heart disease (ACHD) often suffer from deterioration related to cardiac arrhythmias, hypertension (HT) or heart failure (HF), frequently occurring between planned visits. Mobile health (mHealth) could improve management through remote monitoring by enabling swift therapeutic response and detecting new diagnoses., Methods: We performed a prospective study employing mHealth in ACHD patients, weekly monitoring heart rhythm, weight and blood pressure. In case of consecutive threshold exceeding measurements or in case of new diagnosis, patients were contacted and if needed the treating physician was consulted. Inclusion criteria were: palpitations within the last three years (with or without arrhythmia diagnosis) or HF NYHA class ≥ II. We evaluated the detection of recurrences and new diagnosis of arrhythmias, HT and HF, adherence and patient experience (Net Promotor Score (NPS))., Results: In total, 109 of the 268 invited ACHD patients were enrolled, 80 with palpitations, 13 with HF, 16 experienced both, mean age 45 (±13) years, 33% male. Median follow-up was 12 (Q1-Q3;9-14) months, 91 patients initiated all measurements (heart rhythm, weight and blood pressure). In 25% of the patients with diagnosed arrhythmias (14/56) recurrences of arrhythmias were detected; 13% of the patients with undiagnosed palpitations (4/32) were diagnosed with novel arrhythmias. In 38% of the patients with HT at baseline (6/16), treatment adjustment was necessary, 4% of the patients without HT (4/76) received novel HT diagnosis. Diuretics were adjusted in 7% of the patients with HF (2/29). Adherence was > 70% in 77% of the patients that started weekly measurements (70/91). Patients that were female, older of age and experienced palpitations at inclusion were more likely to acquire an adherence of > 70%. NPS was completed by 68 patients, 57 patients (84%) were promotors or neutral, and 11 patients (16%) were critics., Conclusions: mHealth offers advantages in the management of selected ACHD patients; it enabled early detection of recurrences and new diagnosis of arrhythmias, hypertension and heart failure, which lead to swift therapeutic response or remote reassurance. Furthermore, mHealth was well accepted with high adherence and positive patient experience., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: The purpose of this prospective randomised controlled trial was to assess whether home-based, self-selected exercise training is safe, results in high compliance and improves exercise capacity in symptomatic adults with congenital heart disease (CHD)., Methods: Forty adults with moderate or severe CHD (40 ± 12 years, 56% male, New York Heart Association [NYHA] II/III 37/3) were randomly assigned, stratified by CHD complexity, either to home-based exercise training or usual care. The exercise training protocol consisted of three exercise sessions per week for six consecutive months. Patients were free to choose any sports of their preference., Results: Thirty-four patients (each randomisation group n = 17) completed the protocol and were analysed. The majority was involved in high-dynamic sports (76%); none had to discontinue the training programme due to exercise-related adverse events. More than 70% adhered to the exercise programme at or above the target training level. Peak VO 2 increased significantly in the exercise group by +1.7 ± 2.7 ml∙kg∙min -1 (p = 0.025), whereas it remained unchanged in the control group by +0.8 ± 2.2 ml∙kg∙min -1 (p = 0.184). No significant changes were found in serum N-Terminal pro-brain natriuretic peptide levels or quality of life in either randomisation group or between groups., Conclusions: In symptomatic adults with moderate or severe CHD, home-based exercise training of their preference appeared safe, with good compliance and favourable effects on exercise capacity. Our results demonstrate that it is appropriate to stimulate our patients to regularly perform moderate to vigorous physical activities, in absence of medical restrictions., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or with an atrial switch corrected transposition of the great arteries. The aim of the current study was to investigate the longer-term clinical outcomes after valsartan treatment., Methods: From 2006 to 2009, 88 adults were randomly allocated 1:1 to either valsartan or placebo for three consecutive years. Endpoints were defined as overall survival and freedom from clinical events (arrhythmia, heart failure, tricuspid valve surgery, death)., Results: Cardiac drug use and median follow-up after trial close-out (8.3 years) was similar between the randomization groups. Six patients (valsartan n = 3, placebo n = 3) died in 364 and 365 person-years (P = 0.999). No difference in the composite or separate clinical endpoints was found between the randomization groups, with corresponding long-term event-free survival rates of 50% and 34%. Nevertheless, in symptomatic patients valsartan significantly reduced the risk for events compared to placebo (HR 0.37, 95% CI 0.17-0.92). Analysis for repeated events and on-treatment analysis with any renin-angiotensin-aldosterone-system-inhibitor did not alter these results., Conclusions: Valsartan treatment in systemic RV patients did not result in improved survival at longer-term follow-up, but was associated with decreased risk of events in symptomatic patients., (Copyright © 2018. Published by Elsevier B.V.)

Background: Scarce data on crowdfunding report a maximal funding of €10.000,-, and state that research is needed to attract attention of larger granting organizations. The aims of this project were 1) to fund an eHealth study in grown-up congenital heart disease (GUCH) patients 2) to contemplate on critical success factors., Methods: After peer review of the Dutch Heart Foundation a project was published at a donation platform, which was open for donations during a predetermined period of two months. Copywriters were hired to create an easy-to-understand message to donors. A video teaser was created with a motivated patient, and rewards were available. The crowdfunding targeted €25.000 and the Dutch Heart Foundation doubled the donations to €50.000, and return of donations were guaranteed in case this was not met., Results: Initially, donations came from the investigators' private inner circle. In total, 44 potential donors were contacted, but refused to donate originally. Multiple (social) media campaigns were published to promote the project, and an offline mailing was sent to contributors to the Dutch Heart Foundation. During the project support emerged, resulting in extra donations and public awareness. In the lasts three weeks, after sufficient private donations, five major donors decided to support the project. The project became a big success: the predetermined target was exceeded and a total of €74.450,- was raised., Conclusion: Innovative crowdfunding gave the opportunity to start eHealth research in GUCH patients. Critical success factors include support of a professional organization, support of stakeholders, and easy-to-understand messages., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Introduction: Mobile health (mHealth), an advanced form of eHealth is expected to drastically change the field of traditional healthcare in the near future as wearables and mobile applications are rapidly increasing in number. The majority of patients with congenital heart disease (CHD) now reach adulthood and this relative young patient population seems particularly suited for mHealth, as they require lifelong follow-up, experience high morbidity burden, and were raised in this digital era. In patients with acquired heart disease the potential of eHealth has been demonstrated, yet data are still inconclusive. Areas covered: In this review of the current literature we evaluated the effect of various eHealth interventions in patients with CHD. Our search resulted in a mere 10 studies, which comprised mostly of children or adolescents with severe CHD. Home-monitoring of saturation and weight through mHealth was found to be beneficial in patients after palliation procedures, and video conferencing was found to have a positive effect on anxiety and healthcare utilization. Expert commentary: Due to high morbidity and mortality in patients with CHD and the promising results of eHealth interventions, further research is desperately needed.

Introduction: The number of grown-up congenital heart disease (GUCH) patients is steadily increasing. Unfortunately, the majority of these patients suffer from late sequelae, with heart failure being the most common cause of death. Exercise training is beneficial and safe in patients with acquired heart failure, as well as in asymptomatic GUCH patients. However, its effect remains unknown in symptomatic GUCH patients. This could cause reticence on positive sports advice, with possible counterproductive effects. Areas covered: A review of current literature was performed to evaluate the effect of exercise training in symptomatic (NYHA≥2) GUCH patients. The search yielded a mere three studies including symptomatic patients, and another six studies including also patients in NYHA 1 without making clear distinction between the NYHA subgroups. Expert commentary: Suboptimal trial designs, low patient numbers, and homogeneity of investigated cardiac anomalies make this review insufficient to draw definite conclusions. However, all studies describe overall positive effects of exercise training in symptomatic GUCH patients in terms of exercise capacity and quality of life. There were no safety concerns. Larger-scaled, randomized controlled trials are needed to obtain certainty.

Background: The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot., Methods: The REDEFINE trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) is an investigator-initiated, multicenter, prospective, 1:1 randomized, double-blind, placebo-controlled study. Adults with repaired tetralogy of Fallot and RV dysfunction (RV ejection fraction [EF] <50%) but without severe valvular dysfunction were eligible. Patients were randomly assigned between losartan (150 mg daily) and placebo with target treatment duration between 18 and 24 months. The primary outcome was RV EF change, determined by cardiovascular MRI in intention-to-treat analysis., Results: Of 95 included patients, 47 patients received 150 mg losartan daily (age, 38.0±12.4 years; 74% male), and 48 patients received placebo (age, 40.6±11.4 years; 63% male). Overall, RV EF did not change in patients allocated to losartan (n=42) (44.4±5.1% to 45.2±5.0%) and placebo (n=46) (43.2±6.3% to 43.6±6.9%). Losartan did not significantly improve RV EF in comparison with placebo (+0.51%; 95% confidence interval, -1.0 to +2.0; P =0.50). No significant treatment effects were found on secondary outcomes: left ventricular EF, peak aerobic exercise capacity, and N-terminal pro-brain natriuretic peptide ( P >0.30 for all). In predefined subgroup analyses, losartan did not have a statistically significant impact on RV EF in subgroups with symptoms, restrictive RV, RV EF<40%, pulmonary valve replacement, or QRS fragmentation. However, in a post hoc analysis, losartan was associated with improved RV EF in a subgroup (n=30) with nonrestrictive RV and incomplete remodeling (QRS fragmentation and previous pulmonary valve replacement) (+2.7%; 95% confidence interval, +0.1 to +5.4; P =0.045)., Conclusions: Losartan had no significant effect on RV dysfunction or secondary outcome parameters in repaired tetralogy of Fallot. Future larger studies may determine whether there might be a role for losartan in specific vulnerable subgroups., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02010905., (© 2017 American Heart Association, Inc.)

Background: Although QRS duration >180 ms has prognostic value in adults with tetralogy of Fallot (TOF), its sensitivity to predict mortality is low. Fragmented QRS complexes, a simple measurement on ECG, are related to myocardial fibrosis and dysfunction in patients with TOF. Our objective was to determine whether QRS fragmentation predicts major outcomes in TOF., Methods: This multicentre study included adult patients with TOF from a prospective registry. Notches in the QRS complex in ≥2 contiguous leads on a 12-lead ECG, not related to bundle branch block, were defined as QRS fragmentation, which was classified as none, moderate (≤4 leads) or severe (≥5 leads). The primary and secondary outcomes were all-cause mortality and clinical ventricular arrhythmia, respectively., Results: A total of 794 adult patients with TOF (median age 27 years, 55% male; 52% no QRS fragmentation, 32% moderate, 16% severe) were included. During long-term (median 10.4 years) follow-up, 46 (6%) patients died and 35 (4%) patients had ventricular arrhythmias. Overall, 10-year survival was 98% in patients without fragmented QRS complexes, 93% in patients with moderate QRS fragmentation and 81% in patients with severe QRS fragmentation. In multivariable Cox hazards regression analysis, extent of QRS fragmentation (HR: 2.24/class, 95% CI 1.48 to 3.40, p<0.001) remained independently predictive for mortality, whereas QRS duration was not predictive (p=0.85). The extent of QRS fragmentation was also independently predictive for ventricular arrhythmia (HR: 2.00/class, 95% CI 1.26 to 3.16, p=0.003)., Conclusions: The extent of QRS fragmentation is superior to QRS duration in predicting mortality in adult patients with TOF and may be used in risk stratification., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Renin-angiotensin-aldosterone system (RAAS) inhibition with angiotensin II receptor blockers or angiotensin-converting enzyme inhibitors is beneficial in patients with acquired left ventricular dysfunction. Adult patients with tetralogy of Fallot (TOF) with right ventricular (RV) dysfunction are at high risk for heart failure, arrhythmias, and sudden cardiac death. However, the efficacy of RAAS inhibition has not been established in these patients., Methods: The REDEFINE is an investigator-initiated, multicenter, prospective, randomized, double-blind, placebo-controlled trial to study the effects of the angiotensin II receptor blocker losartan (target dosage of 150 mg once daily) in adult patients with TOF. Patients with RV dysfunction in the absence of severe valvular dysfunction are eligible for inclusion. The primary end point is the change in RV ejection fraction after 18 to 24 months, as measured by cardiovascular magnetic resonance imaging. In addition, laboratory measurements, echocardiography, and cardiopulmonary exercise testing are performed., Conclusion: The REDEFINE trial will study the effects of RAAS inhibition with losartan in TOF patients with RV dysfunction., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aims: The right ventricle (RV) remodels early after pulmonary valve replacement (PVR) in tetralogy of Fallot (TOF) patients. Previously reported preoperative thresholds to achieve early postoperative RV normalization were consistently close to 80 mL/m(2) for end-systolic volume (ESV) and 160 mL/m(2) for end-diastolic volume (EDV). Our objective was to determine whether these thresholds were also associated with mid-to-late RV normalization and clinical events., Methods and Results: Out of a multicentre cohort of 157 TOF patients who had undergone PVR, in 65 patients (62% male, age 29 ± 8 years, homograft in 98%) cardiovascular magnetic resonance (CMR) imaging was performed preoperatively and >3 years (6.3 years, interquartile range: 4.9-9.5) postoperatively. Mid-to-late haemodynamic outcome was classified as: 'RV normalization' [RV ejection fraction (EF) > 48% and RV EDV < 108 mL/m(2)] in 14 of 65 (22%) patients, 'intermediate' in 34 of 65 (52%) patients, and 'suboptimal' (RV EF < 45% and RV EDV > 120 mL/m(2)) in 17 of 65 (26%) patients. Preoperative RV ESV < 80 mL/m(2) was strongly associated with favourable mid-to-late haemodynamic outcome in a proportional odds model [common odds ratio (OR): 0.04 for worse class, 95% confidence interval (CI): 0.01-0.17]. During 7.8 ± 4.0 years follow-up after PVR, adverse clinical events (death, sustained ventricular tachycardia, or heart failure) occurred in 18 of 106 (17%) patients with preoperative CMR available. Patients with preoperative RV ESV > 95 mL/m(2) were at increased risk for unfavourable mid-to-late haemodynamic outcome (common OR: 25.5, 95% CI: 5.35-122) and events (hazard ratio: 2.89, 95% CI: 1.03-8.11)., Conclusion: In TOF patients who had undergone PVR, the best preoperative threshold to achieve mid-to-late RV normalization was RV ESV < 80 mL/m(2). Patients with preoperative RV ESV > 95 mL/m(2) were at increased risk for suboptimal haemodynamic outcome and adverse clinical events. Our findings may assist in timing of PVR., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Background: In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability., Methods: This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR., Results: A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively)., Conclusions: Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objective: Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR., Methods: In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR., Results: A total of 129 patients (61% men, age at PVR 32.9±10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4±4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/m(2), 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m(2) or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors., Conclusions: In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objectives: The aim of the present study is to determine the long-term effects of a ten-week exercise training program in adult patients with a systemic right ventricle., Methods: All patients who participated in a 2009 randomized controlled trial were approached. At approximately three years of follow-up from initial baseline, patients underwent cardiopulmonary exercise testing, filled out two quality of life questionnaires, and NT proBNP levels were measured. All examinations were performed according to the protocols of the 2009 trial. In addition, patients were asked about their current sports habits., Results: Of the 54 patients who were randomized in the 2009-trial 40 participated in the current re-evaluation (male 50%, ccTGA 35%, age 36 ± 10 years, intervention group n=22, control group n=18). After three years, no persistent effect of exercise training on V'O₂peak training remained (-2% of predicted, 95% CI -3% to 5%; p=.56). However, patients who already participated in regular sports or exercise at baseline (n=23/40 (58%)) showed higher V'O₂peak of 13% of predicted (95% CI 4% to 23%; p>.01) and a decrease of 62% in plasma NT-proBNP (95% CI -115% to -10%; p>.03) during follow-up, when compared to patients who did not. Moreover, sports were associated with a lower incidence of clinical events (p=.032)., Conclusion: Short-term beneficial effects of exercise training did not persist over a three-year follow-up period. However, sports participation at baseline was associated with better exercise capacity, lower neurohormone levels, and increased event-free survival., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objectives: The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events., Background: In patients with congenitally or atrially corrected transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia., Methods: At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events., Results: A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m(2) and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors., Conclusions: Patients with a right ventricular end-diastolic volume index above 150 ml/m(2) and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: Aortic coarctation (CoA) occurs as a "simple" isolated disorder, and in a more "complex" form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation., Methods and Results: Cardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39 ± 12 years, male 59%) were followed with CMR (follow-up 5.3 ± 1.8 years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2 ± 2.0mm/5 years (range 0-7.2mm/5 years). History of VSD (β=1.77, P=0.004) and an increased left ventricular mass index (β=0.02, P=0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4mm/5 years versus 1.5mm/5 years respectively. (P=0.03)., Conclusion: Adult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2mm/5 years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: The role of angiotensin II receptor blockers in patients with a systemic right ventricle has not been elucidated., Methods and Results: We conducted a multicenter, double-blind, parallel, randomized controlled trial of angiotensin II receptor blocker valsartan 160 mg twice daily compared with placebo in patients with a systemic right ventricle caused by congenitally or surgically corrected transposition of the great arteries. The primary end point was change in right ventricular ejection fraction during 3-year follow-up, determined by cardiovascular magnetic resonance imaging or, in patients with contraindication for magnetic resonance imaging, multirow detector computed tomography. Secondary end points were change in right ventricular volumes and mass, Vo(2)peak, and quality of life. Primary analyses were performed on an intention-to-treat basis. A total of 88 patients (valsartan, n=44; placebo, n=44) were enrolled in the trial. No serious adverse effects occurred in either group. There was no significant effect of 3-year valsartan therapy on systemic right ventricular ejection fraction (treatment effect, 1.3%; 95% confidence interval, -1.3% to 3.9%; P=0.34), maximum exercise capacity, or quality of life. There was a larger increase in right ventricular end-diastolic volume (15 mL; 95% confidence interval, 3-28 mL; P<0.01) and mass (8 g; 95% confidence interval, 2-14 g; P=0.01) in the placebo group than in the valsartan group., Conclusions: There was no significant treatment effect of valsartan on right ventricular ejection fraction, exercise capacity, or quality of life. Valsartan was associated with a similar frequency of significant clinical events as placebo. Small but significant differences between valsartan and placebo were present for change in right ventricular volumes and mass., Clinical Trial Registration: URL: http://www.controlled-trials.com. Unique identifier: ISRCTN52352170.

Objective: To assess whether exercise training in adult patients with a systemic right ventricle (RV) improves exercise capacity and quality of life and lowers serum N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels., Design: Multi-centre parallel randomized controlled trial., Participants: Patients with a systemic RV due to congenitally or surgically corrected transposition of the great arteries., Methods: Fifty-four adult patients with a systemic RV, were randomized using unmarked opaque envelopes to an intervention group (n = 28) with three training sessions per week for 10 consecutive weeks, and a control group (n = 26). Randomization was stratified by participating centre. At baseline, and follow-up, we determined maximal exercise capacity (V'O(2peak)), serum NT-proBNP levels, and quality of life by means of the SF-36, and the TAAQOL Congenital Heart Disease questionnaires. The final analysis was performed by linear regression, taking into account the stratified randomization., Results: Forty-six patients were analysed (male 50%, age 32 ± 11 years, intervention group n = 24, control group n = 22). Analysis at 10 weeks showed a significant difference in V'O(2peak) (3.4 mL/kg/min, 95% CI: 0.2 to 6.7; P = 0.04) and resting systolic blood pressure (-7.6 mmHg, 95% CI: -14.0 to -1.3; P = 0.03) in favour of the exercise group. No significant changes were found in serum NT-proBNP levels or quality of life in the intervention group or in the control group nor between groups. None of the patients in the intervention group had to discontinue the training programme due to adverse events., Conclusion: In adult patients with a systemic RV exercise training improve exercise capacity. We recommend to revise restrictive guidelines, and to encourage patients to become physically active. (, Trial Registration: The study was registered at http://trialregister.nl. Identifier: NTR1909.).

Background: Ventricular systolic and diastolic function, as measured by echocardiography, are diminished in patients with a systemic right ventricle (RV). As the clinical implications of these finding remained unknown, we aimed to identify echocardiographic parameters of systolic and diastolic ventricular function that are independent determinants of the clinical condition in these patients., Methods: Forty-six adult patients (61% male; mean age 33 [range 18-69] years) with a systemic RV underwent echocardiography to assess qualitative and quantitative systolic and diastolic function of the systemic RV and the subpulmonary left ventricle (LV). Uni- and multivariate linear regression analyses were performed to identify independent echocardiographic determinants for NYHA class, maximal exercise capacity (V'O(2peak)) and NT-proBNP levels., Results: We found qualitative assessment of RV and LV function to be significantly associated with NYHA class (RV: β= 0.26; P = 0.05 and LV: β= 0.82; P < 0.01), V'O(2peak) (RV: β=-10.4; P < 0.05 and LV: β=-18.4; P < 0.05) and NT-proBNP levels (RV: β= 0.58; P < 0.01 and LV: β= 1.40; P < 0.001). Tricuspid annulus plane systolic excursion (TAPSE) was significantly associated with NYHA class (β=-0.92; P = 0.001), V'O(2peak) (β= 18.5; P = 0.05), and serum NT-proBNP levels (β=-1.00; P < 0.05). Associations between quantitative parameters of systolic subpulmonary LV function and clinical parameters were less distinct. We found no associations between RV and LV diastolic function and clinical parameters., Conclusions: Qualitative function of the systemic RV and the subpulmonary LV, and TAPSE, are determinants of clinical condition in patients with a systemic RV. These patients' clinical condition could not be determined by echocardiographically measured diastolic RV function, and systolic and diastolic LV function., (© 2010, Wiley Periodicals, Inc.)

Background: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established., Methods: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries. The primary end point is the change in right ventricular ejection fraction as measured by cardiovascular magnetic resonance or multidetector row cardiac computed tomography in case of pacemaker patients., Conclusion: This large prospective, double-blind, randomized, placebo-controlled trial will establish the role of angiotensin II receptor blockers (valsartan) in the treatment of patients with a systemic right ventricle., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Background: Previous studies demonstrated that ventricular response to stress cardiovascular magnetic resonance (CMR) is frequently abnormal in patients with a systemic right ventricle (RV). However, the clinical implications of these findings remained unknown. We sought to evaluate whether abnormal response to stress CMR predicts adverse outcome in patients with a systemic RV., Methods: Thirty-nine adult patients (54% male; mean age 26, range 18-65 years) with a systemic RV underwent stress CMR to determine the response of RV volumes and ejection fraction (EF). During follow-up, cardiac events, defined as hospitalization for heart failure, cardiac surgery, aborted cardiac arrest, or death, were recorded. The prognostic value of an abnormal response to stress, defined as lack of a decrease in RV end-systolic volume (ESV) or lack of an increase in RV EF, was assessed., Results: We frequently observed an abnormal response to stress, as RV ESV did not decrease in 17 patients (44%), and RV EF did not increase in 15 patients (38%). After a mean follow-up period of 8.1 years, 8 (21%) patients had reached the composite end point. The inability to decrease RV ESV during stress was predictive for cardiac events with a hazard ratio of 2.3 (95% CI 1.19-88.72, P = .034), as was the inability to increase RV EF with a hazard ratio of 2.3 (95% CI 1.31-81.59, P = .027)., Conclusions: Stress CMR potentially has important prognostic value in patients with a systemic RV. Patients with a systemic RV who show abnormal cardiac response to stress have a substantially higher risk of adverse outcome., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Data on relational and sexuality issues in adult patients with congenital heart disease (CHD) are scarce. The present study aimed to evaluate relational and sexual behaviors, satisfaction, and functioning in a representative sample of patients with CHD and their partners. In addition, we aimed to evaluate the relation between sexuality parameters and quality of life. Relational and sexuality issues were assessed using a sexuality questionnaire in 133 patients (52% men, 37 ± 13 years old) with CHD (43 with coarctation of the aorta, 42 with transposition of great arteries, 36 with Marfan syndrome, and 12 with Eisenmenger syndrome), and 74 partners. Quality of life was assessed using the Dutch translation of the Medical Outcomes Study Short Form 36-Item Health Survey. Data were compared to an age- and gender-matched control group (n = 3,642). Seventy-one percent of patients with CHD were involved in a relationship, which was significantly less compared to controls (79%, p < 0.05). Nonetheless, patients perceived their relationship as more satisfactory compared to controls (p < 0.05). Overall, sexual satisfaction was equal in patients compared to controls, although they perceived lower body esteem (p < 0.001), decreased sexual esteem (p < 0.05), and more distress during sex (p < 0.001). Patients reported no more erectile and lubrication problems compared to partners and to controls. We found significant associations between most relational and sexual parameters and quality of life. In conclusion, many aspects of sexuality are affected in adult patients with CHD, whereas their partners remain relatively unaffected. Moreover, sexuality is an important aspect of quality of life in these patients. We advise physicians to be receptive to discuss sexuality issues and provide patients with adequate therapy., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Conflicting data have been published on the differences in cardiac response to exercise between patients with an atrially switched transposition of the great arteries (TGA), and patients with a congenitally corrected TGA (ccTGA). The purpose of our study was to evaluate the differences in cardiac response to exercise in these 2 patient groups with a systemic right ventricle (RV)., Methods: Thirty four patients (62% male; mean 35, range 21-69 years) with a systemic RV (20 with an atrially switched TGA and 14 with a ccTGA) were included. Exercise tests with Portapres measurements were performed to assess maximal exercise capacity (V'O(2peak)), cardiac index, stroke volume index, and heart rate augmentation. Cardiac function was assessed by Cardiovascular Magnetic Resonance or Computed Tomography, and serum NT-proBNP levels., Results: We found that both groups were able to significantly augment cardiac index during exercise. Cardiac index augmentation during exercise was positively related to V'O(2peak) (r=0.37, p<0.05), and independent of resting cardiac function. Although the increase in cardiac index during exercise was similar in both patients with TGA and with ccTGA, mechanisms to achieve this increase differed between groups. ccTGA patients increased both stroke volume and heart rate during exercise. Atrially switched TGA patients augmented heart rate during exercise, but failed to increase stroke volume., Conclusion: Mechanisms to achieve cardiac output augmentation differ between ccTGA patients and TGA patients. We suggest that therapeutic approaches should be tailored to the specific patient group to avoid counterproductive effects., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

Background: In patients with a right ventricle (RV) in the systemic position, tricuspid valve surgery for regurgitation beyond adolescence is a subject of debate. The aim of the present study was to evaluate the complications, survival, and benefit of tricuspid surgery in adult patients with an atrium-level correction for transposition of the great arteries or congenitally corrected transposition of the great arteries., Methods and Results: All adult patients (n=16; 7 men, 9 women; age 35+/-11 years) who underwent tricuspid valvuloplasty (n=8) or replacement (n=8) in the period 1999 to 2008 were included. Complications and survival were analyzed, and postoperative changes in RV function and functional class were evaluated. Tricuspid regurgitation was graded 1 to 4 according to its severity, RV dysfunction was graded as 1 to 4 (1=no dysfunction to 4=severe dysfunction), and functional status was determined according to New York Heart Association class. Although complications occurred in 11 patients, all could be managed adequately. Three patients died 109, 180, and 659 days after surgery, respectively, the first patient after tricuspid valve replacement and the latter 2 after tricuspid valvuloplasty. Overall, tricuspid valve function improved (from grade 3.1+/-0.8 to 0.9+/-1.0; P=0.001) and functional class improved (from 2.7+/-0.6 to 2.1+/-0.8; P=0.007), whereas RV function remained unchanged. After tricuspid valvuloplasty, however, recurrent moderate tricuspid valve regurgitation was observed frequently (n=3; 37%)., Conclusions: Mortality is rather low after tricuspid surgery in adult patients with mild to moderate RV dysfunction. In general, tricuspid valve function and functional class improve significantly after surgery, and systemic RV function is preserved. Tricuspid valvuloplasty, however, is associated with a high rate of recurrence of regurgitation.

Exercise is recommended in patients with left ventricular failure. Although right ventricular failure is common in patients with a systemic right ventricle (RV), no data are available on the effect of physical activity in these patients. The aim of this study was to evaluate the relation of physical activity and cardiac function, exercise capacity, and quality of life in patients with a systemic RV. Forty-seven patients (64% men, mean age 35 years, range 21 to 69) with a systemic RV (31 with an atrially switched transposition of the great arteries and 16 with a congenitally corrected transposition of the great arteries) were included. Cardiac function was assessed by cardiovascular magnetic resonance or computed tomography, exercise tests, and serum N-terminal prohormone brain natriuretic peptide (NT-pro-BNP) levels. Habitual physical activity was assessed using the Short Questionnaire to Assess Health-Enhancing Physical Activity (SQUASH) and quality of life using the Medical Outcomes Study Short Form 36-Item Health Survey. Mean systemic right ventricular ejection fraction was impaired (36.8 +/- 7.8%), as was maximal exercise capacity (78.5 +/- 23.9% of predicted). NT-pro-BNP level was increased (median 269 ng/L, range 34 to 4,476). Mean SQUASH score was 6,808 +/- 3,241, indicating a decreased level of habitual physical activity. Although patients' scores on mental quality-of-life domains were comparable to the general population, scores on most physical quality-of-life domains were significantly lower. SQUASH scores were found to be a significant predictor for exercise capacity (p <0.01) and physical quality of life (p <0.001). However, we found no relation between SQUASH scores and right ventricular ejection fraction or NT-pro-BNP. In conclusion, physical activity in patients with a systemic RV is positively associated with exercise capacity and quality of life, irrespective of cardiac performance.

Background: The method used to delineate the boundary of the right ventricle (RV), relative to the trabeculations and papillary muscles in cardiovascular magnetic resonance (CMR) ventricular volume analysis, may matter more when these structures are hypertrophied than in individuals with normal cardiovascular anatomy. This study aimed to compare two methods of cavity delineation in patients with systemic RV., Methods: Twenty-nine patients (mean age 34.7 +/- 12.4 years) with a systemic RV (12 with congenitally corrected transposition of the great arteries (ccTGA) and 17 with atrially switched (TGA) underwent CMR. We compared measurements of systemic RV volumes and function using two analysis protocols. The RV trabeculations and papillary muscles were either included in the calculated blood volume, the boundary drawn immediately within the apparently compacted myocardial layer, or they were manually outlined and excluded. RV stroke volume (SV) calculated using each method was compared with corresponding left ventricular (LV) SV. Additionally, we compared the differences in analysis time, and in intra- and inter-observer variability between the two methods. Paired samples t-test was used to test for differences in volumes, function and analysis time between the two methods. Differences in intra- and inter-observer reproducibility were tested using an extension of the Bland-Altman method., Results: The inclusion of trabeculations and papillary muscles in the ventricular volume resulted in higher values for systemic RV end diastolic volume (mean difference 28.7 +/- 10.6 ml, p < 0.001) and for end systolic volume (mean difference 31.0 +/- 11.5 ml, p < 0.001). Values for ejection fraction were significantly lower (mean difference -7.4 +/- 3.9%, p < 0.001) if structures were included. LV SV did not differ significantly from RV SV for both analysis methods (p = NS). Including structures resulted in shorter analysis time (p < 0.001), and showed better inter-observer reproducibility for ejection fraction (p < 0.01)., Conclusion: The choice of method for systemic RV cavity delineation significantly affected volume measurements, given the CMR acquisition and analysis systems used. We recommend delineation outside the trabeculations for routine clinical measurements of systemic RV volumes as this approach took less time and gave more reproducible measurements.