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Over the past few years, the migration related problems have been increasing. This is a trend that affects not only individual countries or geographical regions but is rather a global trend. There are a number of factors both in Europe and in the world that cause the movement of people fleeing from the threat to their lives. This is especially evident in connection with Russia’s aggression against Ukraine in 2022. The issue of the protection of aliens in connection with migration is a serious challenge for the international community. This issue is particularly relevant especially in the context of international human rights law. The aim of the research concerns an in-depth analysis of the phenomena that occur today in international human rights law and in the European regional system of their protection. This is especially important in the context of aliens and migrants coming to Europe in connection with the ongoing armed conflict. In view of the events and problems facing Europe, the Member States of the European Union and the institutions of the European Union, it seems necessary to analyse and indicate the legal mechanisms that apply to the situation. The presented research concerns human rights, international law and European law. This scope of research consists in combining efforts and knowledge within the scope of all three mentioned areas. In the area of human rights protection standards, the European paradigm of the protection of aliens can be observed. This paradigm is shaped by a number of instruments, both legally binding and non-formally binding per se (soft law). First of all, these are instruments in the area of the European Union system, including the Charter of Fundamental Rights of the European Union, followed by the documents of the Council of Europe headed by the European Convention on Human Rights and the European Social Charter. In addition, this paradigm is shaped, directly or indirectly, by a number of other international regulations, especially universal law regulations, among which the Convention relating to the Status of Refugees of 1951 has a special position. It is clear that the paradigm of protection of aliens is not limited to functioning within one international organization or one legal system but must be perceived as going beyond a rigidly defined institutional framework. Therefore, it should be looked at from a slightly broader perspective encompassing mechanisms functioning not only in the legal system of the European Union or the Council of Europe, but also those resulting from the system of universal protection of human rights (UN). The attempt to carry out institutional analysis of the European paradigm of the protection of aliens and its key elements can only be made by taking into account the broad perception of the analysed issues. The European paradigm of the protection of aliens is changing, and the dynamics of these changes is determined primarily by human rights standards. This means that the studied paradigm is not a closed normative entity, and the pace and direction in which it develops is determined by the development of human rights protection standards in Europe. These standards are the main factor in this process. The Court of Justice of the European Union and the bodies applying European human rights treaties, headed by the European Court of Human Rights, play a fundamental role in shaping the model of conduct of European countries towards aliens. The subject of the analysis concerns the European paradigm of protection of aliens and the impact it has on the contemporary protection of individual rights. The research was carried out on the basis of a grant from the National Science Centre, Poland under the OPUS 12 Programme: Human Rights in the European Paradigm of the Protection of Aliens (2016/23/B/HS5/03596), headed by Professor Elżbieta Karska.

This paper analyzes the judgment of the Court of Justice of the European Union (CJEU) in the Renckhoff case (C-161/17). First, the introductory remarks outline the background of the core issue, mainly by presenting the current line of the CJEU’s case law in cases based on the same legal norm. They also highlight the distinctive character of the judgment in the Renckhoff case. Next, some relevant facts determining the law-based approach are presented, along with the key case-law theses put forward by the CJEU to justify its decision. These considerations underpin the deliberations on censorship of Internet content, based on copyright law. The observations here refer the findings to the principles of freedom of expression as featured in both national and international law. The paper ends with a summary of the author’s observations on the core issue.

This article aims to analyze the European Union law in terms of the jurisdiction applicable in cases of infringement of personal rights on the Internet. After a short introduction to issues of technical, technological and civilizational progress, consideration is given to the fact that the Internet has become a common platform of human activity. The role, characteristics, and specificity of this state of affairs are presented, and the consequences assessed. The paper then turns to the fact that the problem of determining the applicable jurisdiction in the European Union law is based on general terms from which there are normative exceptions. Such exceptions include a special jurisdiction mechanism that is analysed in this paper. With regard to the case-law of the Court of Justice of the European Union, the paper proposes ways the jurisdiction of European Union Member States should be determined in cases of infringement of personal rights on the Internet. The paper also contains postulates related to the interpretation of relevant Union legislation, which are directed to legislative and judicial authorities. The discussion is closed with a brief summary of the authors’ reflections on the analysed issues, as well as some comments on the recent relevant cases.

Niniejszy artykuł dokonuje podejścia analitycznego dotyczącego możliwości implementacji zasad odpowiedzialności prawnej niewolników na płaszczyznę prawa nowych technologii w postaci tzw. sztucznej inteligencji. W pierwszej kolejności wyjaśniono zakres przedmiotowy supozycji badawczej w ramach uwag wprowadzających, gdzie przedstawiona została istota problemu naukowego. Następnie, biorąc pod uwagę określony porządek metodologiczny, uznano za celowe dokonanie aproksymacji pojęcia SI oraz przedstawienia jej znaczenia w politykach krajowych, wraz z przedstawieniem postulatów de lege ferenda w tych zakresach. Powyższe ułatwiło zrozumienie pełnego zakresu przedmiotowego pojęcia SI i w konsekwencji umożliwiło przejście do prezentacji zasad odpowiedzialności niewolników w prawie rzymskim. W związku z tym osiągalne stało się podjęcie próby implementacji tychże zasad na obszar wyznaczony specyfiką SI, gdzie w formie postulatów de lege ferenda zaproponowano przyczynek zasad odpowiedzialności prawnej SI na podstawie rzymskich regulacji dotyczących niewolników z niezbędnymi zmianami wynikającymi z aktualnego pojmowania prawa. Artykuł kończy się zwięzłym podsumowaniem zawierającym autorskie spostrzeżenia w badanej materii, wraz z niezbędnymi postulatami de lege ferenda.

Radiotherapy is a common treatment modality for cancer. In patients with cancer and implanted cardiac electrotherapy devices, radiotherapy may however interfere with the operation of these devices. In severe cases, this malfunction can lead to irreparable damage to such devices, which requires their replacement. Experience has shown that, with appropriate treatment conditions and safety procedures in place, the risk of radiotherapy causing such disturbances in patients with implantable cardiac devices is low. The rules for managing oncology patients with implantable devices during radiotherapy were included in the latest guidelines published by the European Society of Cardiology concerning cardiac pacing and resynchronization therapy.

ImportanceMavacamten, a cardiac myosin inhibitor, improved peak oxygen uptake (pVO2) in patients with symptomatic obstructive hypertrophic cardiomyopathy (HCM) in the EXPLORER-HCM study. However, the full extent of mavacamten’s effects on exercise performance remains unclear.ObjectiveTo investigate the effect of mavacamten on exercise physiology using cardiopulmonary exercise testing (CPET).Design, Setting, and ParticipantsExploratory analyses of the data from the EXPLORER-HCM study, a randomized, double-blind, placebo-controlled, phase 3 trial that was conducted in 68 cardiovascular centers in 13 countries. In total, 251 patients with symptomatic obstructive HCM were enrolled.InterventionsPatients were randomly assigned in a 1:1 ratio to mavacamten or placebo.Main Outcomes and MeasuresThe following prespecified exploratory cardiovascular and performance parameters were assessed with a standardized treadmill or bicycle ergometer test protocol at baseline and week 30: carbon dioxide output (VCO2), minute ventilation (VE), peak VE/VCO2 ratio, ventilatory efficiency (VE/VCO2 slope), peak respiratory exchange ratio (RER), peak circulatory power, ventilatory power, ventilatory threshold, peak metabolic equivalents (METs), peak exercise time, partial pressure of end-tidal carbon dioxide (PETCO2), and VO2/workload slope.ResultsTwo hundred fifty-one patients were enrolled. The mean (SD) age was 58.5 (11.9) years and 59% of patients were male. There were significant improvements with mavacamten vs placebo in the following peak-exercise CPET parameters: peak VE/VCO2 ratio (least squares [LS] mean difference, −2.2; 95% CI, −3.05 to −1.26; P P P = .001), and peak PETCO2 (LS mean difference, 2.0 mm Hg; 95% CI, 1.12-2.79; P P = .02). There was a significant improvement in nonpeak-exercise CPET parameters, such as VE/VCO2 slope (LS mean difference, −2.6; 95% CI, −3.58 to −1.52; P P Conclusions and RelevanceMavacamten improved a range of CPET parameters beyond pVO2, indicating consistent and broad benefits on maximal exercise capacity. Although improvements in peak-exercise CPET parameters are clinically meaningful, the favorable effects of mavacamten on submaximal exertional tolerance provide further insights into the beneficial impact of mavacamten in patients with obstructive HCM.Trial RegistrationClinicalTrials.gov Identifier: NCT03470545

The subject of the paper is the functioning of Article 79 of the GDPR in the Polish legal system and in other countries. The above provision establishes the right to an effective judicial remedy against a controller or processor, which is to guarantee the protection of personal data in relation to the data subjects. The paper submits to a dogmatic and comparative analysis those regulations that concern the right to an effective legal remedy in the field of personal data protection based on the example of Polish legal regulations, but also with reference to France, Germany and Great Britain to comprehensively study solutions on the issues in question.

Highly specialized methods of selecting participants for clinical trials have been in use for a long time in the medical sciences. An example is offered by oncological pre-profling, which involves the use of genetic biomarkers for a preliminary identification of oncological patients for clinical trials. We should expect further developments in this area, due to the rapid progress being made in e-health technologies and patients’ growing use of mobile applications. Failure to use new technologies in recruitment could even lead to failure in carrying out a clinical trial due to a shortage of participants. This issue is particularly important due to the regulatory framework for profiling adopted under GDPR (the General Data Protection Regulation). GDPR is of crucial importance for the R&D activities pursued by the pharmaceutical industry and in personalized medicine, and one of the biggest challenges is the practical application of its rules in profiling activities. Te same applies to the use of data obtained during clinical trials at the stage when the medical product is about to be launched on the market. The aim of this paper is to present the GDPR rules to facilitate the use of profiling tools for the recruitment of participants for clinical trials. It includes a proposal for the amendment of the EU pharmaceutical law to introduce statutory grounds for profiling.

This research paper analyses legal aspects of the application of modern technologies for practical use in the energy sector in the form of a Smart City. The issue of Smart Environment in the light of renewable energy sources was classified as the main research axis. The main goal of the paper, from the methodological point of view, involves decoding the impact and importance of renewable energy sources for the full implementation of the Smart City concept. First, the article presents introductory remarks regarding the general outline of technical, technological and civilization progress. Then, the essence of the Smart City concept and its pillars have been presented. This permitted the authors to move on to identify the most important legal aspects of the issue discussed in this paper and to search for potential problems and suggest possible solutions. The paper ends with a concise summary containing the authors’ observations on the subject and de lege ferenda postulates.

Assess the diagnostic value of 18-F FDG PET/CT in cardiac implantable electronic devices (CIED) infections in facilitating diagnostic process and optimizing decision-making process.Study group (n = 21) patients with initial suspected diagnosis of CIED-related infection or fever of unknown origin and patients referred for device removal due to infection. Control group (n = 13) patients with implanted CIED, who underwent PET/CT due to other non-infectious indications and had no data for infectious process in follow-up.PET/CT scan showed pocket infection in 12 patients (including 1 in whom infection was not finally diagnosed-the examination was performed early after the implantation procedure-1.5 months), increased tracer uptake in intravascular lead part in 3 patients, and increased uptake in intracardiac part in 5 patients.We found that sensitivity, specificity, positive predictive value, and negative predictive value of the diagnosis made by PET/CT in generator pocket infection was 91.7%, 70%, 78.6%, 87.5% and in lead-dependent intracardiac infection 100%, 47.1%, 35.7%, 100% respectively. PET/CT scan enabled reclassification of diagnosis from possible to definite CIED-related infection in 6 out of 9 patients, and to excluded in 3 out of 9.Establishing diagnosis of device related infections may be challenging due to non-specific symptoms. Incorporation of PET/CT scan in the diagnostic schema can improve accuracy and timing of the diagnosis and help to assess the extent of infection. PET/CT is more useful in local than systemic infectious process related to cardiac implanted electrotherapy device.Trial registration Consent of the bioethics committee nr IK-NP.-0021-85/1465/14. Registration in the www.clinicaltrials.gov database: NCT02196753.

Tensions related to the presence of religion, its symbols or moral judgments in the public space are noticeable signs in many contemporary societies. Research shows an increasing level of prejudice against believers, which translates into emerging microaggressions. This phenomenon of questioning and hostility towards religion is surprisingly also the case in societies where the majority consists of religious adherents. The aim of this article is to present and analyse the research results on the extent of prejudices and manifestations of hostility towards believers in 2019 in Poland, on a representative sample of respondents. First, the religious situation in Poland will be briefly discussed, in particular the way the Poles experience religious practices. This will be followed by discussion of the most common manifestations of microaggression included in the classification proposed by David R. Hodge, and a legal analysis of the Polish penal system. Finally, we will present a hypothesis regarding the nature of these phenomena and the challenges they represent to religious freedom.

The present studies consider the influence of exclusion on aggressive behavior depending on the target of the aggression and the gender of the aggressor. Previous studies have shown that after exclusion, aggressive behavior occurs toward the excluder as well as toward innocent bystanders. However, there is also evidence that men have a greater tendency toward direct aggression and women have a greater tendency toward displaced aggression. In Studies 1 (N = 89) and 2 (N = 120), participants were ostracized, in Studies 3 (N = 88) and 4 (N = 133), participants were rejected. Then, aggressive behavior toward the excluder or bystander was measured. The results confirmed the gender differences hypothesis, but only in the case of ostracism. After ostracism, more retaliatory aggression was observed in men, and more displaced aggression was noted in women. Rejection resulted in generalized aggressive behavior directed toward the excluder and the bystander in men and women. The results are discussed in light of Sexual Selection Theory.

Modern cardiac pacemakers are equipped with a function that allows the heart rate to adapt to the current needs of the patient in situations of increased demand related to exercise and stress ("rate-response" function). This function may be based on a variety of mechanisms, such as a built-in accelerometer responding to increased chest movement or algorithms sensing metabolic demand for oxygen, analysis of intrathoracic impedance, and analysis of the heart rhythm (Q-T interval). The latest technologies in the field of rate-response functionality relate to the use of an accelerometer in leadless endocavitary pacemakers; in these devices, the accelerometer enables mapping of the mechanical wave of the heart’s work cycle, enabling the pacemaker to correctly sense native impulses and stimulate the ventricles in synchrony with the cycles of atria and heart valves. Another modern system for synchronizing pacing rate with the patient’s real-time needs requires a closed-loop system that continuously monitors changes in the dynamics of heart contractions. This article discusses the technical details of various solutions for detecting and responding to situations related to increased oxygen demand (e.g., exercise or stress) in implantable pacemakers, and reviews the results of clinical trials regarding the use of these algorithms.

Titin truncating variants (TTNtv) are known as the leading cause of inherited dilated cardiomyopathy (DCM). Nevertheless, the clinical course is not fully understood and it is unclear whether circulating cardiac biomarkers are helpful in the detection and risk assessment. We sought to assess: 1) early signs of cardiotitinopathy including serum biomarkers: high-sensitivity cardiac troponin T (hs-cTnT) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in clinically stable patients, and 2) indicators of outcome among TTNtv carriers. Our single-centre cohort included 108 carriers (including 70 DCM patients) from 43 families. Clinical, laboratory and follow-up data were analyzed. The earliest abnormality was left ventricular dysfunction, present in 8, 26 and 47% of patients in the 2nd, 3rd and 4th decade of life, respectively. It was followed by symptoms of heart failure and elevation of NT-proBNP, linked to severe (persistent or transient) left ventricular systolic dysfunction, and later by arrhythmias, preceding both malignant ventricular arrhythmia (MVA) and end-stage heart failure (esHF). Hs-cTnT serum levels were increased in the late stage of the disease only. During the median follow-up of 5.2 years both MVA and esHF occurred in 12% of TTNtv carriers. In multivariable analysis, NT-proBNP level ≥650 pg/ml was the best predictor of both composite endpoint (MVA and esHF), and of MVA alone. We conclude that assessment of circulating cardiac biomarkers is not useful in the detection of cardiotitinopathies but may be helpful in the risk assessment.

Older age and high morbidity of the society contribute to a growing number of patients with cardiac implantable electronic devices (CIEDs) requiring effective cancer treatment, including radiotherapy (RT). The effect of RT on a CIED may vary depending on the type and physical parameters of radiation, location of the treated lesion, indications for electrotherapy, and the type of CIED. In the most dramatic scenarios, it may cause an irreversible damage to the CIED, with serious clinical consequences. The lack of precise guidelines may limit the access to RT for many patients with CIEDs who would otherwise benefit from the therapy or may lead to a therapy without taking the necessary precautions, which may worsen the prognosis. Therefore, clear and unequivocal recommendations for assessing patient eligibility for RT are aimed at ensuring that adequate precautions are taken as well as at providing patients with concomitant cardiovascular and oncologic diseases with access to safe and effective RT.

This paper discusses the judicial dialogue between the European Court of Human Rights and the Court of Justice of the European Union regarding the placing of hyperlinks on the internet. Firstly, the case law of the CJEU in the light of the linkage is analysed. This shows the scope of restrictions created by this judicial authority in the area of copyright. Secondly, the judgment of the ECHR will be cited and analysed as regards the relationship between placing hyperlinks on the internet and freedom of expression. There is a judicial dialogue with the CJEU, which focuses attention on the human rights aspect of hyperlinks. Thirdly, the correlation between the jurisprudence of the CJEU and the ECHR will be analysed, including a functional interpretation. The paper ends with a discussion about the potential of this judicial dialogue for the wider purpose of building an optimal model for European dialogue.

This paper discusses issues relating to the possibility of implementing telemedicine solutions within the framework of the Polish healthcare system financed from public funds. Firstly, as part of introductory remarks, an outline of technical, technological and civilizational progress, as well as its correlation with the law and social behaviours is made. Secondly, due to the lack of a legal definition of e-health, the definitions proposed in literature and created by selected international and non-governmental organizations are recalled. In this way the essence of this concept is revealed. Thirdly, based on relevant international documents, the overall potential of e-health is presented. This shows the various benefits that the use of avant-garde solutions within the healthcare system can bring. Fourthly, a catalogue of the possible barriers to the deployment of e-health solutions is presented. From the presentation, it becomes clear that such processes will not run seamlessly. Fifthly, an outline of the right to healthcare is provided on the basis of instruments of national and international law, indicating the most important elements of the right. Sixthly, a forecast of the demand for telemedicine services in Poland is presented. Seventhly, the sociological concept of the so-called digital exclusion is introduced and the occurrence of this phenomenon in the Polish society is discussed. The paper ends with a brief summary, in which the author considers all the arguments presented and offers his own position and de lege ferenda proposals for the Polish legislator.

Background: Remote monitoring of cardiovascular implantable electronic devices allows the assessment of system effectiveness, arrhythmia occurrence, and indirectly, clinical changes. Medical interventions can be performed earlier because of a faster transfer of information to the monitoring site, even in the case of asymptomatic arrhythmias or abnormalities in the operation of the system. Aims: The aim of the study was to assess the effectiveness of remote monitoring of implantable cardioverter-defibrillators and evaluation in an outpatient setting during 12-month follow-up. Methods: We analyzed 176 patients at 10 sites (men, 84.1%). The mean (SD) age of the patients was 60.7 (12.5) years (range, 20–86 years), and mean (SD) follow-up period was 405 (70) days (range, 131–723 days). Results: A total of 354 outpatient and 514 remote follow-up visits were conducted. Episodes of arrhythmias and device malfunctions were detected with similar frequency in outpatient visits and in remote visits. During the study period, patient sense of safety increased. More patients preferred joined remote and outpatient visits as the optimal healthcare model. As the patient survey showed, the greatest benefit of the CareLink network was fast intervention and an increased sense of safety. Conclusions: The strategy of remote monitoring appeared to be feasible, safe, and patient friendly, demonstrating that the majority of patients do not require an additional in -person visit within 1 year from the device implantation just to confirm the proper functioning of the implantable cardioverter--defibrillators.

The aim of the article is to analyse the process of harmonizing the value added tax (VAT) in the European Union, and in particular the factors behind the decision to build a common VAT system based on the country of destination principle instead of, as initially assumed, the country of origin principle. Characterizing the conditions in which this change in approach took place, the specifi c features of public fi nances of these countries were presented, in particular the relation of public expenditure as well as the general level of taxation to GDP. Large differences between EU Member States in this area are one of the main factors making these countries not agree to resign from their sovereignty in shaping taxes. This, in turn, affects the direction and possibilities of the tax harmonization process in the Union, including in the fi eld of VAT. The transitional VAT system operating in the EU since 1993 has been pragmatically adapted to the needs arising from the development of intra-EU trade. Its transformation planned in 2022 to become a new defi nitive system based on the country of destination principle requires the support of Member States for major legislative changes necessary for its implementation.

BACKGROUND: Atrial fibrillation (AF) is a common complication in patients with hypertrophic cardiomyopathy (HCM) and may contribute to high cardiovascular morbidity and mortality. Therefore, it is important to assess parameters associated with AF in HCM patients. HYPOTHESIS: The aim of the study was to evaluate AF prevalence in patients with HCM and to investigate risk factors for AF. METHODS: Five hundred and forty‐six HCM patients aged below 65 were included into analysis. Clinical and echocardiographic parameters were analyzed. RESULTS: In 141 patients (25.8%) AF episodes were recorded. The following factors were identified as risk factors for AF in patients with HCM: age ≥ 45 years (OR 2.38, CI 1.40‐4.05, P = 0.001), past history of presyncope or syncope (OR 2.25, CI 1.35‐3.74, P = 0.002), non‐sustained ventricular tachycardia (nsVT) (OR 2.70, CI 1.60‐4.57, P

Artykuł stanowi omówienie konkretnego problemu jaki może powstać w trakcie próby wdrożenia rozwiązań telemedycznych w Polsce. Po pierwsze, ze względu na brak definicji legalnej telemedycyny zostaną przytoczone propozycje znaczenia tego pojęcia wysuwane przez doktrynę przedmiotu oraz organizacje międzynarodowe i pozarządowe, wraz ze wspólnym mianownikiem przedmiotowych definicji. Po drugie, zostanie omówione konstytucyjne prawo do opieki zdrowotnej, które zostało przewidziane w polskiej ustawie zasadniczej w kontekście wymogu równości w dostępie do opieki medycznej. Po trzecie, zostanie przedstawiona prognoza zapotrzebowania na świadczenia zdrowotne w Polsce, bazująca na odpowiednich dokumentach Głównego Urzędu Statystycznego. Po czwarte, zostanie omówione zjawisko tzw. wykluczenia cyfrowego wraz z podaniem jego powodów, skali oraz podstawowego kryterium. Artykuł zakończą konkluzje w przedmiocie rozważań, które zawierają autorskie stanowisko oraz postulaty de lege ferenda dla polskiego ustawodawcy.

The core objective of this paper is to determine the main political and economic conditions and challenges related to the possible evolution of the integration process in the euro area toward fiscal federalism and fiscal union as a way to increase the capacity of the area to cope with future economic and financial crises. The issue of a separate budget for this area is of particular interest in this article. The idea of such a budget has recently become the subject of lively public debate in the European Union and has been a factor in encouraging the European Commission to propose new solutions at the end of 2017 in the field of economic governance of the euro area, including the establishment of a European Monetary Fund. The analysis carried out in this article leads to the conclusion that under the current conditions, there is little chance of introducing such regulatory and institutional changes that would significantly increase the scale of financial transfers between the euro area member states.

This article concerns the correlation between criminal law and procedure and the technical, technological and civilisation progress in the form of possibility to stand bail in Bitcoin virtual currency (cryptocurrency) in the criminal procedure. The article, firstly, presents introductory remarks to provide information about the analysed subject. Secondly, there is an outline of the essence and mechanism of applying bail in criminal procedure. Thirdly, the process transformation of money as an element of assets is presented. Fourthly, the article presents a categorisation of digital money and an economic qualification of Bitcoin. The above provides a background for argumentation relating to the legal qualification of Bitcoin as money and presentation of the basic threats connected with this cryptocurrency. The article ends with a brief summary containing the authors’ view on the subject of these reflections and a proposal of the desired legal amendments, addressed to the Polish legislator.

This paper concerns the operation of the uncontested lex retro non agit principle in the new realities of criminal law on the example of so-called extended confiscation of property. Until recently, perpetrators who derived financial benefits from offences, as a rule, were unpunished. The new institution introduced in 2017 restored the sense of the principle of social justice. Nevertheless, this institution has provoked doubts regarding the principle of non-retroactivity of criminal law.

Titin truncating variants (TTNtv) are known as the leading cause of inherited dilated cardiomyopathy (DCM). Nevertheless, it is unclear whether circulating cardiac biomarkers are helpful in detection and risk assessment. We sought to assess 1) early indicators of cardiotitinopathy including the serum biomarkers high-sensitivity cardiac troponin T (hs-cTnT) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in clinically stable patients, and 2) predictors of outcome among TTNtv carriers. Our single-center cohort consisted of 108 TTNtv carriers (including 70 DCM patients) from 43 families. Clinical, laboratory and follow-up data were analyzed. The earliest abnormality was left ventricular dysfunction, present in 8, 26 and 47% of patients in the second, third and fourth decade of life, respectively. It was followed by symptoms of heart failure, linked to NT-proBNP elevation and severe left ventricular systolic dysfunction, and later by arrhythmias. Hs-cTnT serum levels were increased in the late stage of the disease only. During the median follow-up of 5.2 years, both malignant ventricular arrhythmia (MVA) and end-stage heart failure (esHF) occurred in 12% of TTNtv carriers. In multivariable analysis, NT-proBNP level ≥650 pg/mL was the best predictor of both composite endpoints (MVA and esHF) and of MVA alone. In conclusion, echocardiographic abnormalities are the first detectable anomalies in the course of cardiotitinopathies. The assessment of circulating cardiac biomarkers is not useful in the detection of the disease onset but may be helpful in risk assessment.