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Arrhythmogenic right ventricular cardiomyopathy (ARVC) may be complicated by heart failure. Management of advanced heart failure in this context is challenging.We reviewed our center's experience with advanced heart failure therapies in patients with ARVC. Three rapidly deteriorating patients with ARVC with biventricular heart failure were found. Their management and outcomes are presented. Data on ventricular fibrosis were available in 2 of them and are also included.The first patient underwent initially successful paracorporeal pulsatile biventricular assist device (BiVAD) implantation. However, a large ischemic stroke occurred 2 weeks later, and the patient died after 2 months. The second patient underwent urgent BiVAD implantation after extracorporeal membrane oxygenation support because of cardiogenic shock, but his course was complicated by multiorgan failure due to systemic infection and the patient died. The last patient, being at Interagency Registry for Mechanically Assisted Circulatory Support 3-4 profile, underwent heart transplant with uneventful recovery. Extensive fibrosis was present in both ventricles of 2 patients undergoing pathology examination.Patients with ARVC and advanced biventricular heart failure are characterized by extensive ventricular fibrosis and considerable risk, but data on their management are limited. Biventricular circulatory support is associated with suboptimal outcomes, and prioritization for heart transplant seems preferable.

Myocarditis is an inflammatory disease of the myocardium diagnosed through a combination of histological, immunological and immunohistochemical criteria. Its clinical presentation varies from an acute coronary-like symptoms to heart failure. Diagnostic workup includes elevated biomarkers, ECG and echocardiographic findings. Cardiac magnetic resonance is the most important examination providing information on both ventricular function and tissue characterization. However, in the case of critically ill patients, CMR should be replaced with endomyocardial biopsy (EMB) which remains the gold standard in myocarditis diagnosis. EMB provides information on both the etiology and prognosis thus affecting the therapeutic approach to the patient. For example, virus positive myocarditis benefits from antiviral treatment while in virus negative ones, immunosuppression is more appropriate. Mechanical circulatory support (MCS) is often necessary in patients presenting with cardiogenic shock. MCS includes intra-aortic balloon pump, temporary percutaneous or even surgically implanted ventricular assist devices and extracorporeal membrane oxygenation (ECMO). ECMO essentially bypasses the heart and provides adequate oxygenation to peripheral organs. Due to the increased afterload under ECMO support, it seems reasonable to be combined with intra-aortic balloon pump or percutaneous VAD implantation to promote left ventricular unloading and potential recovery.

AIMS: Data on sex and left ventricular assist device (LVAD) utilization and outcomes have been conflicting and mostly confined to US studies incorporating older devices. This study aimed to investigate sex-related differences in LVAD utilization and outcomes in a contemporary European LVAD cohort. METHODS AND RESULTS: This analysis is part of the multicentre PCHF-VAD registry studying continuous-flow LVAD patients. The primary outcome was all-cause mortality. Secondary outcomes included ventricular arrhythmias, right ventricular failure, bleeding, thromboembolism, and the haemocompatibility score. Multivariable Cox regression models were used to assess associations between sex and outcomes. Overall, 457 men (81%) and 105 women (19%) were analysed. At LVAD implant, women were more often in Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile 1 or 2 (55% vs. 41%, P = 0.009) and more often required temporary mechanical circulatory support (39% vs. 23%, P = 0.001). Mean age was comparable (52.1 vs. 53.4 years, P = 0.33), and median follow-up duration was 344 [range 147-823] days for women and 435 [range 190-816] days for men (P = 0.40). No significant sex-related differences were found in all-cause mortality (hazard ratio [HR] 0.79 for female vs. male sex, 95% confidence interval [CI] [0.50-1.27]). Female LVAD patients had a lower risk of ventricular arrhythmias (HR 0.56, 95% CI [0.33-0.95]) but more often experienced right ventricular failure. No significant sex-related differences were found in other outcomes. CONCLUSIONS: In this contemporary European cohort of LVAD patients, far fewer women than men underwent LVAD implantation despite similar clinical outcomes. This is important as the proportion of female LVAD patients (19%) was lower than the proportion of females with advanced HF as reported in previous studies, suggesting underutilization. Also, female patients were remarkably more often in INTERMACS profile 1 or 2, suggesting later referral for LVAD therapy. Additional research in female patients is warranted. ispartof: ESC HEART FAILURE vol:10 issue:2 ispartof: location:England status: Published online

Background: Despite recent advances in management of patients with advanced heart failure, mortality remains high. Aim of this study was to compare impact of different aetiology of ischaemic and idiopathic cardiomyopathy on early outcomes and long-term survival of patients after left ventricular assist device implantation. Methods: European Registry for Patients with Mechanical Circulatory Support (EUROMACS) gathers clinical data and follow-up parameters of LVAD recipients. Patients enrolled in the EUROMACS registry with primary diagnosis of either ischaemic ( n = 1190) or idiopathic ( n = 812) cardiomyopathy were included. Primary Endpoints were early mortality as well as long-term survival. Secondary endpoint were major postoperative adverse events, such as need for rethoracotomy. Additionally, a propensity-score matching analysis was performed for patients with ischaemic ( n = 509) and idiopathic ( n = 509) cardiomyopathy. Results: In terms of basic demographics and baseline parameters the two groups significantly differed as expected before propensity-score matching due to different aetiology of cardiomyopathy. Seven-day (52 (4.4%) versus 18 (2.2%); p = 0.009), 30-day (153 (12.9%) versus 73 (9.0%); p = 0.008) and in-hospital mortality (253 (19.7%) versus 123 (15.1%); p = 0.009) were significantly lower in the idiopathic cardiomyopathy group compared to the ischaemic cardiomyopathy group, whereas after propensity-score matching 30-day ( p = 0.169) was comparable and in-hospital mortality ( p = 0.051) was almost significant. Kaplan-Meier survival analysis revealed no significant difference in regard of long-term survival after propensity-score matching (Breslow-test p = 0.161 and LogRank-test p = 0.113). Conclusion: Though patients with ischaemic and idiopathic cardiomyopathy suffer from different cardiomyopathy aetiologies, 30-day-mortality and long-term survival of both groups were similar leading to the conclusion that covariates predominately influence mortality and survival of ischaemic and idiopathic cardiomyopathies.

AIMS: Use of left ventricular assist devices (LVADs) in older patients has increased, and assessing outcomes in older LVAD recipients is important. Therefore, this study aimed to investigate associations between age and outcomes after continuous-flow LVAD (cf-LVAD) implantation. METHODS AND RESULTS: Cf-LVAD patients from the multicentre European PCHF-VAD registry were included and categorized into those

OBJECTIVES In the third report of the European Registry for Patients with Mechanical Circulatory Support of the European Association for Cardio-Thoracic Surgery, outcomes of patients receiving mechanical circulatory support are reviewed in relation to implant era. METHODS Procedures in adult patients (January 2011-June 2020) were included. Patients from centres with 3 months). Risk factors for death were explored using univariable Cox regression with a stepwise time-varying hazard ratio (3 months). RESULTS In total, 4834 procedures in 4486 individual patients (72 hospitals) were included, with a median follow-up of 1.1 (interquartile range: 0.3-2.6) years. The annual number of implants (range: 346-600) did not significantly change (P = 0.41). Both Interagency Registry for Mechanically Assisted Circulatory Support class (classes 4-7: 23, 25 and 33%; P < 0.001) and in-hospital deaths (18.5, 17.2 and 11.2; P < 0.001) decreased significantly between eras. Overall, mortality, transplants and the probability of weaning were 55, 25 and 2% at 5 years after the implant, respectively. Major infections were mainly noted early after the implant occurred (AER(3 months): 0.45). Bilirubin and creatinine levels were significant risk factors in the early phase but not in the late phase after the implant. CONCLUSIONS In its 10 years of existence, EUROMACS has become a point of reference enabling benchmarking and outcome monitoring. Patient characteristics and outcomes changed between implant eras. In addition, both occurrence of outcomes and risk factor weights are time dependent. As a registry of the European Association for Cardio-Thoracic Surgery, the European Registry for Patients with Mechanical Circulatory Support (EUROMACS) offers a robust repository of clinical data on long-term mechanical circulatory support (MCS) from a large international community., European Association for Cardio-Thoracic Surgery (EACTS), This work was funded and supported by European Association for Cardio-Thoracic Surgery (EACTS).

Funding Acknowledgements Type of funding sources: None. Introduction Patients with advanced heart failure are frequently referred to cardiac intensive care unit (CICU) for hemodynamic support. Despite optimum pharmacological therapy many of these patients persist in critical condition, demanding short term circulatory support until recovery or heart transplantation. Intra-aortic balloon pump is commonly used in patients with advanced heart failure in order to stabilize hemodynamically compromised patients as bridge to decision for further therapies. Purpose To investigate the outcomes of patients with acute advanced heart failure referred to cardiac intensive care unit and supported with short term mechanical support. To explore the hemodynamic effects of the Intra-aortic balloon pump therapy in these patients. Material & Method We retrospectively analyzed the data from 104 patients with acute advanced heart failure (either as worsening of existing symptoms of chronic HF or as first onset of HF). All patients had severe left ventricular dysfunction, which was frequently associated with right ventricular dysfunction and increased NT pro BNP levels. Demographic data were collected including co-morbidities. Laboratory data included NT-pro BNP, total bilirubin, serum creatinine, urea and baseline e GFR (MDRD Formula). All patients received IABP support and underwent right heart catheterization. Measurements were made before and after the insertion of the IABP. The study has been approved by the Scientific Committee and the Ethics Committee of the Hospital. Results The mean age of the sample was 44 ± 12 years. 76 patients (73, 08%) were male. 20 patients (19, 23%) were of ischemic etiology. The median time on IABP support for the entire cohort was 13 days (3 – 41, 25). There was statistically significant decrease in the NT-pro BNP levels (p Conclusions In this single center study the use of IABP improved the laboratory findings and the hemodynamic status of patients admitted in the cardiac intensive care unit with acute advanced heart failure. It was an effective method of managing patients as bridge to decision for further therapies.

This chapter focuses on cytokines and inflammatory markers. An important pathway in chronic heart failure progression is inflammatory activation. Experimental studies have shown that proinflammatory cytokines may induce many aspects of the syndrome of chronic heart failure, such as left ventricular dysfunction, pulmonary oedema, and the process of left ventricular remodelling. Although the cause of the inflammation is unknown, both infectious and non-infectious events could be operating, including interaction with the neurohormone system. Thus, inflammatory markers have emerged as potential indicators of the evolution of heart failure, ranging from their use for screening, diagnosis, determining prognosis, and guiding treatment. Indeed, the emerging association of inflammatory mediators with the pathogenesis and progression of chronic heart failure has already resulted in the development of new anti-inflammatory strategies, which might be used as adjunctive therapy in patients with chronic heart failure. Moreover, there is accumulating evidence that a critical network of interactions is formed by inflammatory and the classic neurohormonal mediators, and that many of the conventional therapies for heart failure may, at least partially, modulate the proinflammatory cytokine milieu. However, therapies tested so far have been largely disappointing.

The Hellenic Heart Failure Association has undertaken the initiative to develop a national network of heart failure clinics (HFCs) and cardio‐oncology clinics (COCs). We conducted two questionnaire surveys among these clinics within 17 months and another during the coronavirus disease 2019 outbreak to assess adjustments of the developing network to the pandemic. Out of 68 HFCs comprising the network, 52 participated in the first survey and 55 in the second survey. The median number of patients assessed per week is 10. Changes in engaged personnel were encountered between the two surveys, along with increasing use of advanced echocardiographic techniques (23.1% in 2018 vs. 34.5% in 2020). Drawbacks were encountered, concerning magnetic resonance imaging and ergospirometry use (being available in 14.6% and 29% of HFCs, respectively), exercise rehabilitation programmes (applied only in 5.5%), and telemedicine applications (used in 16.4%). There are 13 COCs in the country with nine of them in the capital region; the median number of patients being assessed per week is 10. Platforms for virtual consultations and video calls are used in 38.5%. Coronavirus disease 2019 outbreak affected provision of HFC services dramatically as only 18.5% continued to function regularly, imposing hurdles that need to be addressed, at least temporarily, possibly by alternative methods of follow‐up such as remote consultation. The function of COCs, in contrast, seemed to be much less affected during the pandemic (77% of them continued to follow up their patients). This staged, survey‐based procedure may serve as a blueprint to help building national HFC/COC networks and provides the means to address changes during healthcare crises.

Aims Despite the existence of many studies, there are still limited data about the characteristics of myocarditis in Greece. This led to the creation of the Greek Myocarditis Registry aiming to document the different symptoms and treatment of myocarditis, assess possible prognostic factors, and find similarities and differences to what is already published in literature. This paper is a preliminary descriptive analysis of this Registry. Methods and results We analysed data for the hospitalization period of all patients included in the Registry from December 2015 until November 2017. Statistics are reported as frequency (%) or median and inter‐quartile range (IQR) as appropriate. In total, 146 patients were included; 83.3% of the patients reported an infection during the last 3 months. The most common symptom, regardless of the underlying infection, was chest pain (82.2%) followed by dyspnoea (18.5%), while the most common finding in clinical examination was tachycardia (26.7%). Presentation was more frequent in the winter months. ECG findings were not specific, with the repolarization abnormalities being the most frequent (60.3%). Atrial fibrillation was observed in two patients, both of whom presented with a reduced ventricular systolic function. Left ventricular ejection fraction changed significantly during the hospitalization [55% (IQR: 50–60%) on admission vs. 60% (IQR: 55–60%) on discharge, P = 0.0026]. Cardiac magnetic resonance was performed in 88 patients (61%), revealing mainly subepicardial and midcardial involvement of the lateral wall. Late gadolinium enhancement was present in all patients, while oedema was found in 39 of them. Only 11 patients underwent endomyocardial biopsy. Discharge medication consisted mainly of beta‐blockers (71.9%) and angiotensin‐converting enzyme inhibitors (41.8%), while 39.7% of the patients were prescribed both. Conclusions This preliminary analysis describes the typical presentation of myocarditis patients in Greece. It is a first step in developing a better prognostic model for the course of the disease, which will be completed after the incorporation of the patients' follow‐up data.

Funding Acknowledgements Type of funding sources: None. Background Heart failure (HF) is a heterogeneous disease with complex structural and electrophysiological derangements of the heart. Attempts to classify HF from the electrophysiological perspective are lacking. Purpose To use electrocardiographic (ECG) data for phenotypic classification of patients with HF. Methods In this retrospective cohort study, all adult patients hospitalized for HF during 2010-2016 at a tertiary center were included. Automated measurements of the first ECG obtained during the index admission were recorded. K-means clustering using premorbid conditions and selected ECG measurements were used to classify the cohort into four mutually exclusive clusters. The primary (all-cause and cardiovascular mortality) and secondary (ventricular arrhythmia (VA)) outcomes were compared between clusters using Cox regression analysis. Results In total, 2849 patients (1363 males, age 75.1 ± 13.4 years) were included. Over a mean follow-up period of 5.37 ± 4.10 years, all-cause and cardiovascular mortality occurred in 2071 (72.7%) and 600 (21.1%) patients respectively, while VA occurred in 110 patients (3.9%). Cluster 1 was characterised by a low heart rate and low ventricular activation time (VAT). Cluster 2 was characterised by old age, low absolute QRS area, and high QTc and QT dispersion. Cluster 3 was characterised by young age, and left ventricular hypertrophy (LVH), and few had history of VA. Cluster 4 was characterised by wide QRS, hypertension, ischaemic heart disease, high VAT, and high absolute T wave area. Cluster 4 had the highest and cluster 1 the lowest risks of all-cause (hazard ratio (HR) 2.96 [1.09, 1.50], p = 0.003; Figure A) and cardiovascular mortality (HR 2.90 [1.15, 2.11], p = 0.004; Figure B). Meanwhile, cluster 2 had the highest risk of VA (HR 2.23 [1.09, 3.85], p = 0.025; Figure C) while clusters 1 and 3 similarly had the lowest risks. Conclusion HF presents with clinically and electrophysiologically distinct phenotypes. Clustering analysis is useful in identifying HF phenotypes which are prognostically significant. Abstract Figures A, B, and C

Introduction Right Ventricular (RV) function has prognostic implications in end-stage heart failure (ESHF) patients. RV failure following Left Ventricular Assist Device (LVAD) implantation increases morbidity and mortality. Achieving optimal RV function before LVAD implantation is of paramount importance. Purpose Purpose was to investigate the effect of Intra-aortic Balloon Pump (IABP) on RV function optimization in patients with bi-ventricular ESHF. Methods ESHF patients with poor RV function, presenting with acutely decompensated heart failure resistant to inotropes/vasopressors, thus requiring IABP for stabilization, were prospectively enrolled. Serum biochemistry, echocardiography and invasive hemodynamics were applied and eligibility for LVAD according to RV function was determined on the basis of pre-specified criteria (Right atrium Pressure (RAP) 1.85, RAP/Pulmonary Capillary Wedge Pressure (PCWP) Results Sixteen patients aged 38±14 years were enrolled. Duration of IABP support was 62±50 (3–180) days. Three patients deteriorated requiring additional mechanical circulatory support. Two patients were stabilized without RV function improvement. In the remaining 11 patients, RV improved and fulfilled LVAD eligibility criteria (IABP responders); RA and RA/PCWP decreased from 18±6 to 10±4mmHg (p=0.0001) and from 0.60±0.19 to 0.42±0.11 (p=0.011) respectively. PAPi and RV strain improved from 1.46±0.65 to 3.20±0.58 (p=0.0001) and from −12.9±3.4% to −18.7±1.7% (p=0.0001) respectively. Significantly lower baseline NTproBNP and total bilirubin values were observed in the responders group. Six patients finally received LVAD and none suffered RV failure post-operatively (the remaining 5 were successfully transplanted). RV fibrosis correlated with post-IABP NTproBNP (r=0.91, p=0.001), total bilirubin (r=0.79, p=0.011), RAP (r=0.78, p=0.014), PAPi (r=−0.69, p=0.040), RAP/PCWP (r=0.74, p=0.022) and LV fibrosis (r=0.77, p=0.016), but not with baseline (pre-IABP) parameters. Conclusions Prolonged IABP support contributes to partial RV function recovery in patients with ESHF and bi-ventricular failure, thus leading to eligibility for LVAD implantation. RV fibrosis may predict RV response to IABP and post-IABP eligibility for LVAD. Funding Acknowledgement Type of funding sources: None.

Background Restrictive cardiomyopathy is a rare cardiac disease, for which several genes including TNNT2, MYPN, FLNC and TNNI3 have been associated with its familial form. Case presentation Here we describe a female proband with a severely manifested restrictive phenotype leading to heart transplantation at the age of 41, who was found homozygous for the novel TNNI3 mutation: NM_000363.4:c.586G > C, p.(Asp196His). Her parents were third-degree cousins originating from a small village and although they were found heterozygous for the same variant they displayed no symptoms of the disease. Her older sister who was also found heterozygous was asymptomatic. Her twin sister and her brother who were homozygous for the same variant displayed a restrictive and a hypertrophic phenotype, respectively. Their children are all carriers of the mutation and remain asymptomatic until the age of 21. Conclusion These observations point to a recessive mode of inheritance reported for the first time for this combination of gene/disease.

Danon disease is a rare X-linked cardiac and skeletal muscle disorder with multisystem clinical manifestations. Genetic defects at the lysosome-associated membrane 2 protein (LAMP2) are the cause of the disorder. Due to the rarity of the disease, there is limited progress in understanding the correlation between genotype and phenotype, and explaining the large variability of the clinical features of the disease. In this study, we report two patients, twin sisters, referred to our hospital for end stage heart failure due to dilated cardiomyopathy, requiring heart transplant evaluation. Genetic analysis, using targeted next generation sequencing, showed that the proband carried a LAMP2 missense variant, c.928G > A. The mutation was also detected in her twin sister by sanger sequencing. This variant has already been reported by other investigators and was correlated with the clinical triad of Danon disease i.e. hypertrophic cardiomyopathy, mental retardation and peripheral myopathy. The new phenotype of dilated cardiomyopathy associated with this mutation, confirms the phenotypic heterogeneity of the particular mutation, as well as of Danon disease.