Aims Patients with a pacemaker or implantable cardioverter-defibrillator are often considered for cardiac resynchronization therapy (CRT). However, limited comprehensive data are available regarding their long-term outcomes. Methods and results Our retrospective registry included 2524 patients [1977 (78%) de novo, 547 (22%) upgrade patients] with mild to severe symptoms, left ventricular ejection fraction ≤35%, and QRS ≥ 130ms. The primary outcome was the composite of all-cause mortality, heart transplantation (HTX), or left ventricular assist device (LVAD) implantation; secondary endpoints were death from any cause and post-procedural complications. In our cohort, upgrade patients were older [71 (65–77) vs. 67 (59–73) years; P < 0.001], were less frequently females (20% vs. 27%; P = 0.002) and had more comorbidities than de novo patients. During the median follow-up time of 3.7 years, 1091 (55%) de novo and 342 (63%) upgrade patients reached the primary endpoint. In univariable analysis, upgrade patients exhibited a higher risk of mortality/HTX/LVAD than the de novo group [hazard ratio (HR): 1.41; 95% confidence interval (CI): 1.23–1.61; P < 0.001]. However, this difference disappeared after adjusting for covariates (adjusted HR: 1.12; 95% CI: 0.86–1.48; P = 0.402), or propensity score matching (propensity score-matched HR: 1.10; 95% CI: 0.95–1.29; P = 0.215). From device-related complications, lead dysfunction (3.1% vs. 1%; P < 0.001) and pocket infections (3.7% vs. 1.8%; P = 0.014) were more frequent in the upgrade group compared to de novo patients. Conclusion In our retrospective analysis, upgrade patients had a higher risk of all-cause mortality than de novo patients, which might be attributable to their more significant comorbidity burden. The occurrence of lead dysfunction and pocket infections was more frequent in the upgrade group.

Although genetic predisposition to haematological malignancies has long been known, genetic testing is not yet the part of the routine diagnostics. In the last ten years, next generation sequencing based studies identified novel germline mutations in the background of familial aggregation of certain haematologic disorders including myelodysplastic syndromes (MDS) and acute myeloid leukaemia (AML). This is supported by the fact that the myeloid neoplasms with genetic predisposition represent a new category in the revised 2016 World Health Organization classification. According to the new classification, these disorders are subdivided based on the clinical and genetic features, including myeloid neoplasms with germline predisposition alone, or with pre-existing platelet disorder, cytopaenias or other organ failures. The predisposing genetic factors include mutations in the RUNX1, CEBPA, GATA2, ANKRD26, ETV6, DDX41, TERC or TERT and SRP72 genes. The genes affected in these syndromes are important regulators of haemopoiesis and are frequently implicated in leukaemogenesis, providing deeper insight into the understanding of normal and malignant haemopoiesis. Despite the growing knowledge of germline predisposing events in the background of familial myeloid malignancies, the germline genetic component is still unknown in a subset of these pedigrees. Here, we present the first study of inherited myeloid malignancies in Hungary. We identified three families with apparent clustering of myeloid malignancies with nine affected individuals across these pedigrees. All tested individuals were negative for CEBPA, GATA2, RUNX1, ANKRD26, ETV6, DDX41, TERC or TERT and SRP72 mutations, suggesting the presence of so far unidentified predisposing mutations.

Electroanatomical mapping is a useful technical tool to guide endocardial LV lead implantation. It helps to identify the location of the transseptal puncture and the use of activation mapping might facilitate location of the optimal lead positions during CRT.

Therapeutic use of hypothermia has come to the frontline in the past decade again in the prevention and in mitigation of neurologic impairment. The application of hypothermia is considered as a successful therapeutic measure not just in neuro- or cardiac surgery, but also in states causing brain injury or damage. According to our present knowledge this is the only proven therapeutic tool, which improves the neurologic outcome after cardiac arrest, decreasing the oxygen demand of the brain. Besides influencing the nervous system, hypothermia influences the function of the whole organ system. Beside its beneficial effects, it has many side-effects, which may be harmful to the patient. Before using it for a therapeutic purpose, it is very important to be familiar with the physiology and complications of hypothermia, to know, how to prevent and treat its side-effects. The purpose of this article is to summarize the physiologic and pathophysiologic effects of hypothermia.

Since the establishment of highly active antiretroviral therapy, survival rates have improved among patients with human immunodeficiency virus infection giving them the possibility to become transplant candidates. Recent publications revealed that human immunodeficiency virus-positive heart transplant recipients' survival is similar to non-infected patients. We present the case of a 40-year-old human immunodeficiency virus infected patient, who was hospitalized due to severely decreased left ventricular function with a possible aetiology of acute myocarditis, that has later been confirmed by histological investigation of myocardial biopsy. Due to rapid progression to refractory cardiogenic shock, extracorporeal membrane oxygenation implantation had been initiated, which was upgraded to biventricular assist device later. On the 35th day of upgraded support, the patient underwent heart transplantation uneventfully. Our clinical experience confirms that implementation of temporary mechanical circulatory support and subsequent cardiac transplantation might be successful in human immunodeficiency virus-positive patients even in case of new onset, irreversible acute heart failure.

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A beültethető kardiális elektronikus eszközzel élő betegek számának folyamatos növekedése következtében a rendszeres utánkövetés egyre nagyobb terhet ró az egészségügyi személyzetre. A hagyományos, személyes utánkövetés mellett az elmúlt két évtizedben a technológia fejlődésével egyre több lehetőség nyílt a betegek telemonitorozására, azaz távoli utánkövetésére. Jelentős számú, széles körű vizsgálatot végeztek a telemonitorozás lehetséges előnyeivel kapcsolatban, amelyek bizonyították létjogosultságát, így napjainkra része lett a releváns ajánlásoknak és irányelveknek. Cikkünkben áttekintést adunk a telemonitorozás alapjairól és bemutatjuk a főbb vizsgálatok eredményeit. Thepossibilitiesoftelecardiologywithcardiacimplantableelectronicdevices The exponentially increasing number of the patients wearing cardiac implantable electronic devices increases the challenge of the regular follow-ups. Beside the traditional in-office follow-up, the development of technology of last two decades has given the opportunity for remote monitoring of patients. A large number of extensive studies have been carried out to prove the potential benefits of telemonitoring, the data of these studies have been justified and influenced the relevant recommendations and guidelines. In our article, we provide an overview of the basics of telemonitoring and present the results of the main studies.