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Background & Aims: Early identification of worsening HF enables timely adjustments to prevent hospitalization. Recent studies show the HeartLogic™ algorithm detects congestion and reduces HF events. However, it is unclear which patients benefit most. Therefore, this study aims to identify and characterize HF patients who benefit most from CIED-based remote monitoring with HeartLogic™., Methods: In this multicenter retrospective study, patients with a CIED and HeartLogic™ algorithm under structured follow-up were included. Patients were classified as having "substantial benefit" or "no benefit" from monitoring., Results: In total, 242 patients were included (male n = 190, 79%, median age 61 years [IQR 61-77]). Median follow-up was 1.2 years [IQR 1.1-2.7]. Among 378 alerts, 266 were true positive (70%) and 112 false positive (30%). Of the 242 patients, 69 (29%) were classified as having "substantial benefit", while 173 (71%) had "no benefit" from HeartLogic™ monitoring. Univariate and multivariate analysis showed that patients with "substantial benefit" had higher NYHA functional class (OR 2.64, P = 0.004), higher NT-ProBNP (OR 1.02, P = 0.003), higher serum creatinine (OR 1.10, P < 0.001), lower LVEF (OR 1.19, P = 0.004), more severe mitral regurgitation (OR 2.16, P = 0.006), higher right ventricular end diastolic volume (OR 1.05, P = 0.040), higher pulmonary artery pressures (OR 1.19, P = 0.003), and were more likely to use loop diuretics (OR 2.79, P = 0.001). Among patients with "substantial benefit," the positive predictive value (PPV) of HeartLogic™ to detect congestion was 92%., Conclusion: The utilization of CIED-based HeartLogic™ driven HF care demonstrated pronounced efficacy, predominantly in patients exhibiting characteristics of HF at a more advanced disease stage., Competing Interests: Declaration of competing interest The department of Cardiology of the LUMC reports receiving unrestricted research and educational grants from Boston Scientific Corporation, Medtronic, and Biotronik. The department of cardiology of OLV Aalst reports receiving research and educational grants from Boston Scientific, Medtronic, Biotronik, Abbott St Jude Medical, and Microport and an educational and research grant from Boston Scientific with reference number ISRRM11793. The funders were not involved in study design, collection, analysis, interpretation of data, the writing of this article, or the decision to submit it for publication. ADE receives consultancy and speaker fees from Boston Scientific Corporation and Medtronic. SLMAB receives speaker fees from Boston Scientific Corporation and Medtronic. The other authors have no conflict of interest to declare., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Heart failure (HF) admissions are burdensome, and the mainstay of prevention is the timely detection of impending fluid retention, creating a window for medical treatment intensification. This study evaluated the accuracy and performance of a Triage-HF-guided carepath in real-world ambulatory HF patients in daily clinical practice. In this prospective, observational study, 92 adult HF patients (71 males (78%), with a median age of 69 [IQR 59-75] years) with the Triage-HF algorithm activated in their cardiac implantable electronic devices (CIEDs), were monitored. Following high-risk alerts, an HF nurse contacted patients to identify signs and symptoms of fluid retention. The sensitivity and specificity were 83% and 97%, respectively. The positive predictive value was 89%, and negative predictive value was 94%. The unexplained alert rate was 0.05 alerts/patient year, and the false negative rate was 0.11 alerts/patient year. Ambulatory diuretics were initiated or escalated in 77% of high-risk alert episodes. In 23% (n = 6), admission was ultimately required. The median alert handling time was 2 days. Fifty-eight percent (n = 18) of high-risk alerts were classified as true positives in the first week, followed by 29% in the second-third weeks (n = 9), and 13% (n = 4) in the fourth-sixth weeks. Common sensory triggers included an elevated night ventricular rate (84%), OptiVol (71%), and reduced patient activity (71%). The CIED-based Triage-HF algorithm-driven carepath enables the timely detection of impending fluid retention in a contemporary ambulatory setting, providing an opportunity for clinical action.

We present the case of a 52-year-old woman with cardiogenic shock and refractory right ventricular failure due to spontaneous dissection of the right coronary artery. She remained dependent on mechanical support for several weeks. Both a right ventricular assist device implant and a bidirectional cavopulmonary anastomosis were explored as long-term support options. A history of malignancy and possible right ventricular functional recovery resulted in a decision in favour of the bidirectional cavopulmonary anastomosis and concomitant tricuspid valve annuloplasty. Postoperatively her clinical condition improved significantly, and she could be discharged home. Echocardiography showed normalization of right ventricular dimensions and slight improvement of right ventricular function., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Heart failure (HF) is the principal cause of morbidity and mortality in adults with congenital heart disease (ACHD). Robust evidence-based treatment options are lacking., Objectives: This study aims to evaluate the safety, tolerability, and short-term HF-related effects of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in a real-world ACHD population., Methods: All patients with ACHD treated with SGLT2i in 4 European ACHD centers were included in this retrospective study. Data were collected from 1 year before starting SGLT2i to the most recent follow-up. Data on side effects, discontinuation, mortality, and hospitalizations were collected., Results: In total, 174 patients with ACHD were treated with SGLT2i from April 2016 to July 2023. The mean age was 48.7 ± 15.3 years, 72 (41.4%) were female, and 29 (16.7%) had type 2 diabetes mellitus. Ten (5.7%) patients had mild, 75 (43.1%) moderate, and 89 (51.1%) severe congenital heart disease. HF was the most frequent starting indication (n = 162, 93.1%), followed by type 2 diabetes (n = 11, 6.3%) and chronic kidney disease (n = 1, 0.6%). At median follow-up of 7.7 months (Q1-Q3: 3.9-13.2 months), 18 patients (10.3%) reported side effects, 12 (6.9%) permanently discontinued SGLT2i, and 4 (2.3%) died of SGLT2i-unrelated causes. A significant reduction in the HF hospitalization rate was observed from 6 months before to 6 months after starting SGLT2i (relative rate = 0.30; 95% CI: 0.14-0.62; P = 0.001)., Conclusions: SGLT2i generally seem safe, well-tolerated, and potentially beneficial in patients with ACHD. SGLT2i was associated with a 3-fold reduction in the 6-month HF hospitalization rate. These results warrant prospective randomized investigation of the potential benefits of SGLT2i for patients with ACHD., Competing Interests: Funding Support and Author Disclosures Dr Neijenhuis has received support from the Foundation “De Drie Lichten” (Hilversum, the Netherlands), AstraZeneca, and the Leiden University Medical Center research council Cardio-Vascular cluster Themes for Innovation funding. Dr Zemrak has received speaker fees from Abbott Laboratories. Dr Rotmans has received an unrestricted research grant from AstraZeneca. Dr Jongbloed has received support from the Leiden University Medical Center research council Cardio-Vascular cluster Themes for Innovation funding; and has received a personal grant from the NWO/ZonMw (The Hague, the Netherlands), the Bontius Foundation (Leiden, the Netherlands), and the Rembrandt Institute (Leiden, the Netherlands). Dr Egorova has received support from the Leiden University Medical Center research council Cardio-Vascular cluster Themes for Innovation funding; and has received consultancy and speaker fees from Boston Scientific Corporation and Medtronic Inc. The Department of Cardiology of the Leiden University Medical Center has received unrestricted research and educational grants from Boston Scientific Corporation, Medtronic, and Biotronik. The funders were not involved in study design, collection, analysis, interpretation of data, the writing of this paper, or the decision to submit it for publication. No artificial intelligence programs contributed to the compilation of the submitted manuscript. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Survival and quality-of-life of left ventricular assist device (LVAD) recipients improved significantly because of growing experience and technological advances. However, LVAD-related complication rates, including recurrent episodes of congestion, remain high. Early detection of fluid retention to provide a time-window for medical intervention is the pillar in preventing hospitalizations. The multisensory HeartLogic TM algorithm accurately detected impending congestion in ambulant heart failure patients. The aim of the current study is to investigate the feasibility of HeartLogic TM -driven care in LVAD patients., Methods: Consecutive LVAD destination therapy patients were followed-up according the structured HeartLogic TM -based heart failure carepath. An alert triggered a device check-up, and the heart failure team contacted the patient to evaluate for signs and symptoms of impending congestion. An alert was adjudicated as true positive or unexplained. An episode of congestion not preceded by an alert was deemed as a false negative., Results: Data from 7 patients were included: the median age was 67 years [IQR 61-71], 71% were male and 71% had a non-ischemic aetiology. Total follow-up entailed 12 patient-years. All patients experienced at least one alert. In total, 33 alerts were observed. Majority of alerts (70%, n = 23) were driven by congestion and one alerts (15%) were clinically meaningful but not primarily fluid-retention-related (e.g., altered hemodynamic triggered by a pump thrombosis). Of all the alerts, five (15%) were classified as an unexplained alert, and during follow-up, four false negative episodes were documented., Conclusions: HeartLogic TM -driven care with continuous monitoring to detect impending fluid retention in LVAD patients was feasible and deserves further prospective validation.

Competing Interests: Disclosures None.

The unique oligomeric alkaliphilic laccase-like oxidases of the ascomycete C. geniculata VKM F-3561 (with molecular masses about 1035 and 870 kDa) were purified and characterized for the first time. The ability of the enzymes to oxidize phenylpropanoids and phenolic compounds under neutral environmental conditions with the formation of previously unknown di-, tri-, and tetrameric products of transformation was shown. The possibility to obtain industrially valuable compounds (dihydroxybenzyl alcohol and hydroxytyrosol) from caffeic acid using laccase-like oxidases of C. geniculata VKM F-3561 has been shown. Complete nucleotide sequence of the laccase gene, which is expressed at the peak of alkaliphilic laccase activity of the fungus, and its promoter region were determined. Based on the phylogenetic analysis of the nucleotide sequence, the nearest relationship of the isolated laccase gene with similar genes of fungi of the genera Alternaria , Bipolaris , and Cochliobolus was shown. Homologous model of the laccase structure was predicted and a proton channel was found, which was presumably responsible for the accumulation and transport of protons to T2/T3-copper center in the alkaliphilic laccase molecule and providing the functional activity of the enzyme in the neutral alkaline environment conditions.

Background: The implantable cardiac defibrillator-based HeartLogic algorithm aims to detect impending fluid retention in patients with heart failure (HF). Studies show that HeartLogic is safe to integrate into clinical practice. The current study investigates whether HeartLogic provides clinical benefit on top of standard care and device telemonitoring in patients with HF., Methods: A multicenter, retrospective, propensity-matched cohort analysis was performed in patients with HF and implantable cardiac defibrillators, and it compared HeartLogic to conventional telemonitoring. The primary endpoint was the number of worsening HF events. Hospitalizations and ambulatory visits due to HF were also evaluated., Results: Propensity score matching yielded 127 pairs (median age 68 years, 80% male). Worsening HF events occurred more frequently in the control group (2; IQR 0-4) compared to the HeartLogic group (1; IQR 0-3; P = 0.004). The number of HF hospitalization days was higher in controls than in the HeartLogic group (8; IQR 5-12 vs 5; IQR 2-7; P = 0.023), and ambulatory visits for diuretic escalation were more frequent in the control group than in the HeartLogic group (2; IQR 0-3 vs 1; IQR 0-2; P = 0.0001)., Conclusion: Integrating the HeartLogic algorithm in a well-equipped HF care path on top of standard care is associated with fewer worsening HF events and shorter duration of fluid retention-related hospitalizations., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: After the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients., Methods: In this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included., Results: Eighty-one patients, 69 % male and median age 21.0 [18.5-22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of >40 mm), 22 (27 %) patients had an acute coronary take-off angle (<30°), and 5 (6 %) patients had an interarterial course of the RCA (2 %) or LCA (4 %). Neo-aortic or coronary artery re-intervention occurred in 10 (12 %) patients. All 10 patients had neo-aortic dilatation or coronary take-off angle of <30° on baseline CTA., Conclusion: This study reports a prevalence of 43 % of neo-aortic dilatation, 6 % of interarterial coronary course and 27 % for acute coronary take-off angle (<30°) at a median term of 21.0 years post ASO. All patients with a neo-aortic re-intervention or coronary artery re-intervention during follow-up had a maximum neo-aortic diameter of >40 mm or a coronary take-off angle of <30° at baseline CTA. This hypothesis generating study suggests that an active surveillance in patients with neo-aortic dilation and/or an acute angulation of < 30° post ASO might be considered and requires prospective evaluation., 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., (© 2023 The Authors.)

Background: The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date., Methods: In this single-centre retrospective observational cohort study, patients who underwent percutaneous VVC closure between 2017 and 2023 were identified., Results: Thirteen patients underwent percutaneous VVC closure (77 % female, age at intervention 24 ± 4 years, 77 % systemic left ventricle, 77 % extracardiac tunnel, median conduit size 16 [16-20]mm). Indications for closure were symptoms and/or significant exercise-related hypoxia. Mean Fontan pressure was 10±4 mmHg. The VVC originated from tributaries of the vena cava superior (VCS) and connected to pulmonary veins (8 VVC, 32 %), VCS to systemic atrium (3 VVC, 12 %), VCS to coronary sinus (3 VVC, 12 %) and tributaries of vena cava inferior to pulmonary veins (11 VVC, 44 %). Twenty-three VVC were occluded using coils and/or plugs. No periprocedural complications occurred. At first follow-up at least 6 months after closure (n = 11), 9 patients (82 %) reported symptom reduction. Saturation at rest and peak exercise increased significantly (96 ± 3 to 98 ± 1 %, p = 0.040; 89 ± 3 to 93 ± 5 %, p = 0.024, respectively). Exercise capacity remained unchanged., Conclusions: VVC typically connect the tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible, safe, and associated with symptom reduction and a significant rise in resting and exercise oxygen saturation., 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., (© 2023 The Authors.)

Objective: Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction. Pharmacological options for sRV failure remain poorly defined. This study aims to investigate the tolerability and effects of sacubitril/valsartan on sRV failure in adult patients with sRV., Methods: In this two-centre, prospective cohort study, all consecutive adult patients with symptomatic heart failure and at least moderately reduced sRV systolic function were initiated on sacubitril/valsartan and underwent structured follow-up., Results: Data of 40 patients were included (40% female, 30% ccTGA, median age 48 (44-53) years). Five patients discontinued therapy during titration. Median follow-up was 24 (12-36) months. The maximal dose was tolerated by 49% of patients. No episodes of hyperkalaemia or renal function decline occurred. Six-minute walking distance increased significantly after 6 months of treatment (569±16 to 597±16 m, p=0.016). Serum N-terminal-prohormone brain natriuretic peptide (NT-proBNP) levels decreased significantly after 3 months (567 (374-1134) to 404 (226-633) ng/L, p<0.001). Small, yet consistent echocardiographic improvements in sRV function were observed after 6 months (sRV global longitudinal strain: -11.1±0.5% to -12.6±0.7%, p<0.001, and fractional area change: 20% (16%-24%) to 26% (19%-30%), p<0.001). The linear mixed-effects model illustrated that after first follow-up moment, no time effect was present for the parameters., Conclusions: Treatment with sacubitril/valsartan was associated with a low rate of adverse effects in this adult sRV cohort. Persisting improvement in 6-minute walking test distance, NT-proBNP levels and echocardiographic parameters of sRV function was observed in an on-treatment analysis and showed no differential response based on sex or anatomy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics., (© 2023 The Authors.)

Implantable cardioverter defibrillators (ICDs) significantly contribute to the prevention of sudden cardiac death in selected patients. However, it is essential to identify those who are likely to not have benefit from an ICD and to defer a pulse generator exchange. Easily implementable guidelines for individual risk stratification and decision making are lacking. This study investigates the 1-year mortality of patients who underwent an ICD or cardiac resynchronization therapy with defibrillator function (CRT-D) pulse generator replacement in a contemporary real-world tertiary hospital setting. The cause of death and patient- and procedure-related factors are stratified, and predictive values for 1-year mortality are evaluated. Patients with a follow-up of ≥365 days (or prior mortality) after an ICD or CRT-D exchange at the Leiden University Medical Center from 1 January 2018 until 31 December 2021 were eligible. In total, 588 patients were included (77% male, 69 [60-76] years old, 59% primary prevention, 46% ischemic cardiomyopathy and 37% mildly reduced left ventricular ejection fraction (LVEF)). Patients undergoing a CRT-D replacement or upgrade had a significantly higher 1-year all-cause mortality (10.7% and 11.9%, respectively) compared to patients undergoing ICD (2.8%) exchange ( p = 0.002). LVEF ≤ 30%, New York Heart Association class ≥ 3, estimated glomerular filtration rate ≤ 30 mL/min/m 2 and haemoglobin ≤ 7 mmol/L were independently associated with mortality within 1 year after pulse generator replacement. There is a growing need for prospectively validated risk scores to weight individualized risk of mortality with the expected ICD therapy benefit and to support a well-informed, shared decision-making process.

Introduction: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce., Method: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records., Results: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction., Conclusion: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

A case of unilateral vascular hypoplasia is presented. A female patient was born with a complex aortic arch anatomy - a double aortic arch with an interrupted left arch. Surgical correction was performed at the age of 3 months. The patient was also noted to have had an ipsilateral large infantile haemangioma. These findings raised the suspicion of the diagnosis of PHACE syndrome. PHACE syndrome is an acronym for Posterior fossa abnormalities, Haemangioma, Arterial anomalies, Cardiac anomalies and Eye anomalies. Future research is needed to elucidate the underlying pathophysiology in PHACE syndrome., 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. The patient gave her consent regarding this publication., (© 2023 The Authors.)

Aims: Given the compelling evidence on the effectiveness of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the conventional heart failure population, SGLT2i deserve exploration in systemic right ventricular (sRV) failure. The initial experience with dapagliflozin in sRV failure patients is described, with a focus on tolerability and short-term effects on clinical outcomes., Methods and Results: Ten patients (70% female, median age 50 years [46.5-52]) with symptomatic sRV failure who received dapagliflozin 10 mg per day on top of optimal medical therapy between 04-2021 and 01-2023 were included. Within 4 weeks, no significant changes in blood pressure, electrolytes, or serum glucose occurred. Creatinine and estimated glomerular filtration rate (eGFR) showed a slight decline (88 ± 17 to 97 ± 23 µmol/L, p  = 0.036, and 72 ± 14 vs. 66 ± 16 ml/min/1.73m 2 , p  = 0.020, respectively). At 6 months follow-up ( n  = 8), median NT-proBNP decreased significantly from 736.6 [589.3-1193.3] to 531.6 [400.8-1018] ng/L ( p  = 0.012). Creatinine and eGFR recovered to baseline levels. There were no significant changes in echocardiographic systolic sRV or left ventricular function. New York Heart Association class improved significantly in 4 out of 8 patients ( p  = 0.046), who also showed an improvement in the 6-minute walk test or bicycle exercise test performance. One female patient developed an uncomplicated urinary tract infection. No patients discontinued treatment., Conclusion: Dapagliflozin was well-tolerated in this small cohort of sRV failure patients. While the early results on the reduction of NT-proBNP and clinical outcome parameters are encouraging, large-scale prospective studies are warranted to thoroughly evaluate the effects of SGLT2i in the growing sRV failure population., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Neijenhuis, Nederend, Jongbloed, Kiès, Rotmans, Vliegen, Jukema and Egorova.)

In December 2018 the patient over 60 years old sought medical help with complaints of persistent cough. Based on computed tomography data there were identified the sings (symptoms) of right lung lower lobe atelectasis. To run this patient diagnostics there were performed 6 bronchoscopies narrowed down by bronchi checks up only and described the right lung central cancer picture. At the same time the biopsy taken during one of the bronchoscopies appeared non informative. Fine needle biopsy of mediastinal lymph nodes and bronchial wall was performed twice: at Tomsk Cancer Research Institute (it was suspected B-cell lymphoma based on biopsy examination) and at The Loginov Moscow Clinical Scientific Center (signs of inflammation based on biopsy examination). The video-assisted thoracoscopy with lymph node dissection of the right lung root was performed in January 2020. As a surgical material analysis result reactive adenopathy was determined. Therefore, during more than 1 year of diagnostic research the genesis of right lung lower lobe atelectasis was not established. The patient was directed to Central Tuberculosis Research Institute for diagnosis verification. Based on clinical and radiological picture Central Tuberculosis Research Institute colleagues suspected a foreign body in the intermediate bronchus. During an endoscopic examination it was revealed stenosis, biopsies were performed, but the foreign body could not be identified. In the pathomorphological laboratory of Central Tuberculosis Research Institute there were conducted histological examination of the material after endobronchial cryobiopsy and rigid needle biopsy. Both gave an unexpected result: invasive mycosis of the bronchial wall. As a conclusion the decision was taken to apply antimycotic therapy on an outpatient basis. As a result, clear clinical and radiological positive dynamics was obtained. In these favorable conditions for endoscopic examination, it was performed vitally essential cryoextraction of foreign body. The foreign body turned out to be a fragment of a spongy bone of a centimeter size. These actions have let to unlock the right lung lower lobe.

In the transposition of the great arteries (TGA), alterations in hemodynamics and oxygen saturation could result in fibrotic remodeling, but histological studies are scarce. We aimed to investigate fibrosis and innervation state in the full spectrum of TGA and correlate findings to clinical literature. Twenty-two human postmortem TGA hearts, including TGA without surgical correction (n = 8), after Mustard/Senning (n = 6), and arterial switch operation (ASO, n = 8), were studied. In newborn uncorrected TGA specimens (1 day-1.5 months), significantly more interstitial fibrosis (8.6% ± 3.0) was observed compared to control hearts (5.4% ± 0.8, p = 0.016). After the Mustard/Senning procedure, the amount of interstitial fibrosis was significantly higher (19.8% ± 5.1, p = 0.002), remarkably more in the subpulmonary left ventricle (LV) than in the systemic right ventricle (RV). In TGA-ASO, an increased amount of fibrosis was found in one adult specimen. The amount of innervation was diminished from 3 days after ASO (0.034% ± 0.017) compared to uncorrected TGA (0.082% ± 0.026, p = 0.036). In conclusion, in these selected postmortem TGA specimens, diffuse interstitial fibrosis was already present in newborn hearts, suggesting that altered oxygen saturations may already impact myocardial structure in the fetal phase. TGA-Mustard/Senning specimens showed diffuse myocardial fibrosis in the systemic RV and, remarkably, in the LV. Post-ASO, decreased uptake of nerve staining was observed, implicating (partial) myocardial denervation after ASO.

Background: Anomalous aortic origin of a right coronary artery (AAORCA) with an interarterial course merits further evaluation; however, robust risk assessment strategies for myocardial ischemia and sudden cardiac death are currently lacking. The aim of this study is to explore the potential role of fractional flow reserve (FFR), instantaneous wave-free ratio (iFR), and intravascular ultrasound (IVUS) in patients with AAORCA., Methods: Consecutive adult patients with AAORCA with an interarterial course were included. Computed tomography angiography, noninvasive ischemia detection, and FFR, iFR, and IVUS were performed at baseline and during adrenaline-induced stress. External compression was evaluated with IVUS., Results: Eight patients (63% female, mean age: 53 ± 9.5 years) were included. Five patients (63%) were symptomatic, and computed tomography angiography revealed high-risk anatomy of the AAORCA in all patients. Only in 1 (12.5%) patient FFR and iFR were positive; however, this was attributed at large to concomitant diffuse atherosclerosis. In 2 of 8 (25%), IVUS revealed external compression; however, the ostial coronary surface area remained unchanged. In all patients, a conservative treatment strategy was pursued. During a mean follow-up of 29.3 months (standard deviation ±2.6 months), symptoms spontaneously disappeared in 4 of 5 (80%) and no adverse cardiac events occurred in any of the patients., Conclusions: Despite the presence of high-risk anatomy in all patients, none had proven ischemia prompting a conservative treatment strategy. No adverse cardiac events occurred during follow-up, and in the majority of patients, symptoms spontaneously disappeared. Therefore, FFR, iFR, and IVUS with pharmacologic stress merit further investigation and might contribute to ischemia-based risk stratification and management strategies in adult patients with AAORCA., (© 2023 The Author(s).)

Background: Counseling of Implantable Cardioverter-defibrillator (ICD) patients with regard to individual risks and benefits is challenging. An evidence-based decision aid tailored to the needs of Dutch ICD patients is not yet available. The objective of this pilot project was to structurally evaluate the current clinical practice in The Netherlands and the ICD patient experience, in order to develop an online decision aid to facilitate shared decision making in ICD procedures., Methods: Between June 2016 and December 2017, a Dutch web-based decision aid was developed according to the Patient Decision Aid Standards (IPDAS) using the RAND-UCLA/multi-stepped Delphi model. Development process consisted of 5 stages in which the Dutch clinical practice was reviewed (stage 1), patients' needs and their history of decision making was structurally assessed (stages 2A and B) and a modified Delphi consensus process was performed with an expert panel consisting of representatives from different medical fields (stage 3). Results from stages 1-3 were used to design and structure the content of an online-based decision aid (stage 4) which was finally evaluated in a usability testing by patients in stage 5., Results and Conclusion: This study describes the evidence-based approach to the development of the Dutch ICD decision aid. In our population, levels of shared decision-making experience were low. The ICD decision aid was structurally developed for the Dutch ICD patient population. Our upcoming multicenter stepped wedge clustered randomized trial will further evaluate the ICD decision aid in clinical practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Yilmaz, Egorova, Schalij, Spierenburg, Verbunt and van Erven.)

Background: Patients with transposition of the great arteries (TGA) after an atrial switch or congenitally corrected TGA (ccTGA) are prone to systemic right ventricular (sRV) failure. Tricuspid valve (TV) regurgitation aggravates sRV dysfunction. Timely TV surgery stabilizes sRV function, yet the development of atrioventricular (AV)-conduction disturbances in the course of sRV failure can contribute to sRV dysfunction through pacing-induced dyssynchrony. This study aims to explore the incidence, timing, and functional consequences of AV-block requiring ventricular pacing after TV surgery in patients with sRV., Methods: Consecutive adolescent and adult patients with an sRV who underwent TV surgery between 1989 and 2020 and followed-up at our center were included in this observational cohort study., Results: The data of 28 patients (53% female, 57% ccTGA, and a mean age at surgery 38 ± 13 years) were analyzed. The mean follow-up was 9.7 ± 6.8 years. Of the remaining 22 patients at the risk of developing high degree AV-block after TV surgery, 9 (41%) developed an indication for chronic ventricular pacing during follow-up, of which 5 (56%) within 24 months postoperatively (3 prior to hospital discharge). The QRS duration, a surrogate marker for dyssynchrony, was significantly higher in patients with chronic left ventricular pacing than in patients with native AV-conduction (217 ± 24 vs. 116 ± 23 ms, p = 0.000), as was the heart failure biomarker NT-pro-BNP [2,746 (1,242-6,879) vs. 495 (355-690) ng/L, p = 0.004] and the percentage of patients with ≥1 echocardiographic class of deterioration of systolic sRV function (27 vs. 83%, p = 0.001). Of the patients receiving chronic subpulmonary ventricular pacing ( n = 12), 9 (75%) reached the composite endpoint of progressive heart failure [death, ventricular assist device implantation, or upgrade to cardiac resynchronization therapy (CRT)]. Only 4 (31%) patients with native AV-conduction ( n = 13) reached this composite endpoint ( p = 0.027)., Conclusion: Patients with a failing sRV who undergo TV surgery are prone to develop AV-conduction abnormalities, with 41% developing an indication for chronic ventricular pacing during 10 years of follow-up. Patients with chronic subpulmonary ventricular pacing have a significantly longer QRS complex duration, have higher levels of the heart failure biomarker NT-pro-BNP, and are at a higher risk of deterioration of systolic sRV function and progressive heart failure., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Nederend, Jongbloed, Kiès, Vliegen, Bouma, Regeer, Koolbergen, Hazekamp, Schalij and Egorova.)

Aim: Early detection of impending fluid retention and timely adjustment of (medical) therapy can prevent heart failure related hospitalizations. The multisensory cardiac implantable electronic device (CIED) based algorithm HeartLogic TM aims to alert in case of impending fluid retention. The aim of the current analysis is to evaluate the performance of the HeartLogic TM guided heart failure care path in a real-world heart failure population and to investigate whether the height of the index and the duration of the alert state are indicative of the degree of fluid retention., Methods: Consecutive adult heart failure patients with a CIED and an activated HeartLogic TM algorithm were eligible for inclusion. Patients were followed up according to the hospital's heart failure care path. The device technician reviewed alerts for a technical CIED checkup. Afterwards, the heart failure nurse contacted the patient to identify impending fluid retention. An alert was either true positive or false positive. Without an alert a patient was true negative or false negative., Results: Among 107 patients, [82 male, 70 (IQR 60-77) years, left ventricular ejection fraction 37 ± 11%] 130 HeartLogic TM alerts were available for analysis. Median follow up was 14 months [IQR 8-23]. The sensitivity to detect impending fluid retention was 79%, the specificity 88%. The positive predictive was value 71% and the negative predictive value 91%. The unexplained alert rate was 0.23 alerts/patient year and the false negative rate 0.17 alerts/patient year. True positive alerts [42 days (IQR 28-63)] lasted longer than false positive alerts [28 days (IQR 21-44)], p = 0.02. The maximal HeartLogic TM index was higher in true positive alerts [26 (IQR 21-34)] compared to false positive alerts [19 (IQR 17-24)], p < 0.01. Patients with higher HeartLogic TM indexes required more intense treatment (index height in outpatient setting 25 [IQR 20-32], day clinic treatment 28 [IQR 24-36] and hospitalized patients 45 [IQR 35-58], respectively), p < 0.01., Conclusion: The CIED-based HeartLogic TM algorithm facilitates early detection of impending fluid retention and thereby enables clinical action to prevent this at early stage. The current analysis illustrates that higher and persistent alerts are indicative for true positive alerts and higher index values are indicative for more severe fluid retention., Competing Interests: AE is a local sub-investigator for the PREEMPT-HF study. RT, SB, and AE received a speaker's honorarium from Boston Scientific in the past 5 years. The Department of Cardiology reports receiving unrestricted research and educational grants from Boston Scientific Corporation, Medtronic, and Biotronik. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Feijen, Egorova, Treskes, Mertens, Jukema, Schalij and Beeres.)

Aims: An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using computed tomography angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, proximal luminal narrowing, and an intramural segment. To date, no robust CTA criteria exist to determine the presence of an intramural segment. We aimed to deduct new CTA parameters to distinguish an intramural course of interarterial ACAOS., Methods and Results: Twenty-five patients with an interarterial ACAOS (64% female, mean age 46 years, 88% right ACAOS) from two academic hospitals were evaluated. Inclusion criteria were the availability of a preoperative CTA scan (0.51 mm slice thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS [defined as 'interluminal space' (ILS)] was assessed at 2 mm intervals along the intramural segment. Analysis showed a mean ILS of 0.69 ± 0.15 mm at 2 mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27 ± 0.29 mm, P  < 0.001). Interobserver agreement evaluation showed good reproducibility (intraclass correlation coefficient 0.77, P  < 0.001). Receiver operator characteristic analysis demonstrated that at a cut-off ILS of <0.95 mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity., Conclusion: The ILS is introduced as a novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of <0.95 mm is indicative of an intramural segment., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background and Objective: Balance between benefit and burden of implantable cardioverter-defibrillator (ICD) therapy is more debatable in older patients, compared to younger patients. Of around 6000 yearly implanted ICDs in the Netherlands, 1:4 is received by patients ≥75 years. We aimed to evaluate the current clinical practice in the Netherlands for ICD implants and generator replacements, with a special focus on the older ICD patients., Research Design and Methods: Cardiologists from all Dutch ICD implanting centres (n = 28) were interviewed. Questions aimed to evaluate outpatient care, pre-operative patient assessment, end-of-life-care counselling, evaluation of social and cognitive wellbeing, clinical evaluation of all patients prior to ICD replacement, and the consideration of the option to downgrade or not replace a device., Results: Implanting cardiologists from all 28 implanting centres were approached for an interview. Response rate was 86%. Management appeared diverse. An age ≥80 years was consistently reported as incentive for more extensive patient evaluation. Patients were invited for counselling prior to device replacements in only the minority (46%) of hospitals. Downgrade or non-replacement was performed in rare cases. End-of-life care discussions were not standard procedure in 67% of the hospitals. Evaluation of social and cognitive wellbeing of patients was based solely on the general clinical impression of the physician in 83%, or not at all assessed in 8% of the centres., Discussion and Implication: A structured framework for care and evaluation of cognitive and/or physical limitations is currently absent in most hospitals. At time of ICD (re-)evaluation, several factors may be considered before deciding on (continuation of) ICD therapy: patient preferences and comorbidity, the need for pacemaker therapy, primary vs. secondary prevention, procedural risks, and patient preferences., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2022. For permissions, please email: journals.permissions@oup.com.)

Aims: The Leiden Convention coronary coding system structures the large variety of coronary anatomical patterns; isolated and in congenital heart disease. It is widely used by surgeons but not by cardiologists as the system uses a surgeons' cranial view. Since thoracic surgeons and cardiologists work closely together, a coronary coding system practical for both disciplines is mandatory. To this purpose, the 'surgical' coronary coding system was adapted to an 'imaging' system, extending its applicability to different cardiac imaging techniques., Methods and Results: The physician takes place in the non-facing sinus of the aortic valve, oriented with the back towards the pulmonary valve, looking outward from the sinus. From this position, the right-hand sinus is sinus 1, and the left-hand sinus is sinus 2. Next, a clockwise rotation is adopted starting at sinus 1 and the encountered coronary branches described. Annotation of the normal anatomical pattern is 1R-2LCx, corresponding to the 'surgical' coding system. The 'imaging' coding system was made applicable for Computed Tomography (CT), Magnetic Resonance Imaging (MRI), echocardiography, and coronary angiography, thus facilitating interdisciplinary use. To assess applicability in daily clinical practice, images from different imaging modalities were annotated by cardiologists and cardiology residents and results scored. The average score upon evaluation was 87.5%, with the highest scores for CT and MRI images (average 90%)., Conclusion: The imaging Leiden Convention is a coronary coding system that unifies the annotation of coronary anatomy for thoracic surgeons, cardiologists, and radiologists. Validation of the coding system shows it can be easily and reliably applied in clinical practice., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.)

In the Netherlands, the coronavirus disease 2019 (COVID‑19) pandemic has resulted in excess mortality nationwide. Chronic heart disease patients are at risk for a complicated COVID‑19 course. The current study investigates all-cause mortality among cardiac implantable electronic device (CIED) patients during the first peak of the pandemic and compares the data to the statistics for the corresponding period in the two previous years. Data of adult CIED patients undergoing follow-up at the Leiden University Medical Centre were analysed. All-cause mortality between 1 March and 31 May 2020 was evaluated and compared to the data for the same period in 2019 and 2018. At the beginning of the first peak of the pandemic, 3,171 CIED patients (median age 70 years; 68% male; 41% ischaemic aetiology) were alive. Baseline characteristics of the 2019 (n = 3,216) and 2018 (n = 3,169) cohorts were comparable. All-cause mortality during the peak of the pandemic was 1.4% compared to 1.6% and 1.4% in the same period in 2019 and 2018, respectively (p = 0.84). During the first peak of the COVID‑19 pandemic, there was no substantial excess mortality among CIED patients in the Leiden area, despite the fact that this is group at high risk for a complicated course of a COVID‑19 infection. Strict adherence to the preventive measures may have prevented substantial excess mortality in these vulnerable patients., (© 2022. The Author(s).)

Aims: Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements. We aimed to evaluate medication use in TGA-AtrSO patients and examine the association of use of renin-angiotensin-aldosterone system (RAAS) inhibitors and β-blockers with long-term survival., Methods and Results: We identified 150 TGA-AtrSO patients [median age 30 years (interquartile range 25-35), 63% male] included in the CONCOR registry from five tertiary medical centres with subsequent linkage to the Dutch Dispensed Drug Register for the years 2006-2014. Use of RAAS inhibitors, β-blockers, and diuretics increased with age, from, respectively, 21% [95% confidence interval (CI) 14-40], 12% (95% CI 7-21), and 3% (95% CI 2-7) at age 25, to 49% (95% CI 38-60), 51% (95% CI 38-63), and 41% (95% CI 29-54) at age 45. Time-varying Cox marginal structural models that adjusted for confounding medication showed a lower mortality risk with use of RAAS inhibitors and β-blockers in symptomatic patients [hazard ratio (HR) = 0.13 (95% CI 0.03-0.73); P = 0.020 and HR = 0.12 (95% CI 0.02-0.17); P = 0.019, respectively]. However, in the overall cohort, no benefit of RAAS inhibitors and β-blockers was seen [HR = 0.93 (95% CI 0.24-3.63); P = 0.92 and HR = 0.98 (0.23-4.17); P = 0.98, respectively]., Conclusion: The use of heart failure medication is high in TGA-AtrSO patients, although evidence of its benefit is limited. This study showed lower risk of mortality with use of RAAS inhibitors and β-blockers in symptomatic patients only. These findings can direct future guidelines, supporting use of RAAS inhibitors and β-blockers in symptomatic, but not asymptomatic patients., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objective: Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients., Methods: Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment., Results: Six-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950-358 ng/L, p<0.001). Echocardiographic systemic RV fractional area change and global longitudinal strain showed small improvements (19%-22%, p<0.001 and -11% to -13%, p=0.014, respectively). The 6 min walking distance improved significantly from an average of 564 to 600 m (p=0.011). The QOL domains of cognitive function, sleep and vitality improved (p=0.015, p=0.007 and p=0.037, respectively)., Conclusions: We describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.)

The cardiac autonomic nervous system (cANS) regulates cardiac adaptation to different demands. The heart is an asymmetrical organ, and in the selection of adequate treatment of cardiac diseases it may be relevant to take into account that the cANS also has sidedness as well as regional differences in anatomical, functional, and molecular characteristics. The left and right ventricles respond differently to adrenergic stimulation. Isoforms of nitric oxide synthase, which plays an important role in parasympathetic function, are also distributed asymmetrically across the heart. Treatment of cardiac disease heavily relies on affecting left-sided heart targets which are thought to apply to the right ventricle as well. Functional studies of the right ventricle have often been neglected. In addition, many principles have only been investigated in animals and not in humans. Anatomical and functional heterogeneity of the cANS in human tissue or subjects is highly valuable for understanding left- and right-sided cardiac pathology and for identifying novel treatment targets and modalities. Within this perspective, we aim to provide an overview and synthesis of anatomical and functional heterogeneity of the cANS in tissue or subjects, focusing on the human heart., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Zandstra, Notenboom, Wink, Kiès, Vliegen, Egorova, Schalij, De Ruiter and Jongbloed.)

Coronary artery status in adults long after the arterial switch operation (ASO) is unclear. We conducted a systematic review to provide an overview of coronary complications during adulthood and to evaluate the value of routine coronary imaging in adults after ASO, in light of current guidelines. Articles were screened for the inclusion of adult ASO patients and data on coronary complications and findings of coronary imaging were collected. A total of 993 adults were followed with a median available follow-up of only 2.0 years after reaching adulthood. Myocardial ischemia was suspected in 17/192 patients (8.9%). The number of coronary interventions was four (0.4%), and coronary death was reported in four (0.4%) patients. A lack of ischemia-related symptoms cannot be excluded because innervation studies indicated deficient cardiac innervation after ASO, although data is limited. Anatomical high-risk features found by routine coronary computed tomography (cCT) included stenosis (4%), acute angle (40%), kinking (24%) and inter-arterial course (11%). No coronary complications were reported during pregnancy (n = 45), although, remarkably, four (9%) patients developed heart failure. The 2020 European Society of Cardiology (ESC) guidelines state that routine screening for coronary pathologies is questionable. Based on current findings and in line with the 2018 American ACC/AHA guidelines a baseline assessment of the coronary arteries in all ASO adults seems justifiable. Thereafter, an individualized coronary follow-up strategy is advisable at least until significant duration of follow-up is available.

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare congenital abnormality. Computed tomography angiography (CTA) is primarily used as a diagnostic tool to evaluate the anatomy and identify potentially malignant AAOCA variants. Limited data is available on the role of CTA during postoperative follow-up. We aimed to develop an objective CTA derived parameter for diagnostic evaluation and follow-up after surgical correction of AAOCA and correlate the anatomical features to the postoperative outcome. Methods: All consecutive patients who underwent surgical repair of AAOCA from 2001 to 2018 and had pre and postoperative CTA imaging available were included. A retrospective analysis of the pre- and postoperative CTA and the outcomes was performed. The origin and course of the anomalous coronary artery and the ostial dimensions were evaluated and correlated with restenosis of operated coronary artery. To allow an accurate evaluation of the effective orifice area at diagnosis and after surgical repair we deduce and propose a new parameter-the coronary triangulated orifice area (CTOA). Results: Out of the 54 patients who underwent surgical treatment for AAOCA, 11 fulfilled the inclusion criteria. The median follow-up was 19 months [IQR 3;42]. The mean age at surgery was 41 ± 16 years, with six patients (55%) being male. Postoperatively, the angle between the proximal coronary artery and the aortic wall increased from 20 ± 5° to 28 ± 9° ( p < 0.01) and ostial diameter in the transversal plane increased from 4.1 ± 2.5 mm to 6.2 ± 2.7 mm ( p < 0.01). The median CTOA increased significantly from 1.6 mm 2 [IQR 0.9;4.9] to 5.5 mm 2 [IQR 3;11.8] ( p < 0.005). During follow-up, in three patients a restenosis of the operated coronary artery was suspected. In these patients, the CTOA only showed a limited postoperative increase of ≤ 1.4 mm 2 . Conclusions: CTA can play an important role in the evaluation of the pre- and postoperative anatomy in AAOCA patients. CTOA may be of use in conjunction with the acute angle take-off and ostial diameter order to comprehensively evaluate the operated ostium after unroofing or patch angioplasty., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Meijer, Kiès, Verheijen, Vliegen, Jongbloed, Hazekamp, Lamb and Egorova.)

Background: Current guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course., Aim: To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries., Methods: A clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained., Results: Primary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated., Conclusions: Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.

Aims: The implantable cardiac defibrillator/cardiac resynchronization therapy with defibrillator-based HeartLogic™ algorithm has recently been developed for early detection of impending decompensation in heart failure (HF) patients; but whether this novel algorithm can reduce HF hospitalizations has not been evaluated. We investigated if activation of the HeartLogic algorithm reduces the number of hospital admissions for decompensated HF in a 1 year post-activation period as compared with a 1 year pre-activation period., Methods and Results: Heart failure patients with an implantable cardiac defibrillator/cardiac resynchronization therapy with defibrillator with the ability to activate HeartLogic and willingness to have remote device monitoring were included in this multicentre non-blinded single-arm trial with historical comparison. After a HeartLogic alert, the presence of HF symptoms and signs was evaluated. If there were two or more symptoms and signs apart from the HeartLogic alert, lifestyle advices were given and/or medication was adjusted. After activation of the algorithm, patients were followed for 1 year. HF events occurring in the 1 year prior to activation and in the 1 year after activation were compared. Of the 74 eligible patients (67.2 ± 10.3 years, 84% male), 68 patients completed the 1 year follow-up period. The total number of HF hospitalizations reduced from 27 in the pre-activation period to 7 in the post-activation period (P = 0.003). The number of patients hospitalized for HF declined from 21 to 7 (P = 0.005), and the hospitalization length of stay diminished from average 16 to 7 days (P = 0.079). Subgroup analysis showed similar results (P = 0.888) for patients receiving cardiac resynchronization therapy during the pre-activation period or not receiving cardiac resynchronization therapy, meaning that the effect of hospitalizations cannot solely be attributed to reverse remodelling. Subanalysis of a single-centre Belgian subpopulation showed important reductions in overall health economic costs (P = 0.025)., Conclusion: Activation of the HeartLogic algorithm enables remote monitoring of HF patients, coincides with a significant reduction in hospitalizations for decompensated HF, and results in health economic benefits., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Inherent to its geometry, echocardiographic imaging of the systemic right ventricle (RV) is challenging. Therefore, echocardiographic assessment of systemic RV function may not always be feasible and/or reproducible in daily practice. Here, we aim to validate the usefulness of a comprehensive range of 32 echocardiographic measurements of systemic RV function in a longitudinal cohort by serial assessment of their correlations with cardiac magnetic resonance (CMR)-derived systemic RV ejection fraction (RVEF). Methods: A single-center, retrospective cohort study was performed. Adult patients with a systemic RV who underwent a combination of both CMR and echocardiography at two different points in time were included. Off-line analysis of echocardiographic images was blinded to off-line CMR analysis and vice versa. In half of the echocardiograms, measurements were repeated by a second observer blinded to the results of the first. Correlations between echocardiographic and CMR measures were assessed with Pearson's correlation coefficient and interobserver agreement was quantified with intraclass correlation coefficients (ICC). Results: Fourteen patients were included, of which 4 had congenitally corrected transposition of the great arteries (ccTGA) and 10 patients had TGA late after an atrial switch operation. Eight patients (57%) were female. There was a mean of 8 years between the first and second imaging assessment. Only global systemic RV function, fractional area change (FAC), and global longitudinal strain (GLS) were consistently, i.e., at both time points, correlated with CMR-RVEF (global RV function: r = -0.77/ r = -0.63; FAC: r = 0.79/ r = 0.67; GLS: r = -0.73/ r = -0.70, all p -values < 0.05). The ICC of GLS (0.82 at t = 1, p = 0.006, 0.77 at t = 2, p = 0.024) was higher than the ICC of FAC (0.35 at t = 1, p = 0.196, 0.70 at t = 2, p = 0.051) at both time points. Conclusion: GLS appears to be the most robust echocardiographic measurement of systemic RV function with good correlation with CMR-RVEF and reproducibility., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Zandstra, Jongbloed, Widya, ten Harkel, Holman, Mertens, Vliegen, Egorova, Schalij and Kiès.)

Aims: Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA are prone to heart failure and arrhythmias. This study evaluated feasibility, patient adherence, and satisfaction of a smart technology-based care pathway for heart failure treatment optimization in these patients., Methods and Results: Patients with symptomatic sRV failure eligible for initiation of sacubitril/valsartan were provided with four smartphone compatible devices (blood pressure monitor, weight scale, step counter, and rhythm monitor) and were managed according to a smart technology-based care pathway. Biweekly sacubitril/valsartan titration visits were replaced by electronical visits, patients were advised to continue measurements at least weekly after titration. Data of 24 consecutive sRV patients (median age 47 years, 50% female) who participated in the smart technology-based care pathway were analysed. Median home-hospital distance was 65 km (maximum 227 km). Most patients (20, 83.3%) submitted weekly measurements; 100% submitted prior to electronical visits. Titration conventionally occurs during a hospital visit. By implementing eHealth smart technology, 68 such trips to hospital were replaced by virtual visits facilitated by remote monitoring. An eHealth questionnaire was completed by 22 patients (92%), and 96% expressed satisfaction. After titration, 30 instances of remote adjustment of heart failure medication in addition to scheduled outpatient clinic visits occurred, one (4%) heart failure admission followed, despite ambulant adjustments. Five patients (21%) sent in rhythm registrations ( n  = 17), of these 77% showed sinus rhythm, whereas supraventricular tachycardia was detected in the remaining four registrations., Conclusion: These data suggest that implementation of a smart technology-based care pathway for optimization of medical treatment sRV failure is feasible with high measurement adherence and patient satisfaction., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.)