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Background: Infective endocarditis (IE) remains major cause of morbidity and mortality in adult congenital heart disease (ACHD). Limited data exists on ACHD with IE in Central and South-Eastern European (CESEE) countries. The aim of this study is to characterize contemporary management and assess outcomes of ACHD with IE in CESEE region., Methods: Data on ACHD patients with IE from 9 tertiary centres in 9 different CESEE countries between 2015 and 2020 was included. Baseline demographics, clinical presentation, indication for surgery, outcomes, hospital and all-cause-1-year mortality were studied., Results: A total of 295 ACHD patients (mean age 40 ± 14 years) with IE were included. Median time from symptoms onset to establishing diagnosis was 25 (11-59) days. The majority of patients (203, 68.8%) received previous empiric oral antibiotic therapy. The highest incidence of IE was observed on native and left sided valves, 194(65.8%) and 204(69.2%), respectively. More than half had a vegetation size ≥10 mm (164, 55.6%); overall 138 (46.8%) had valve complications and 119 (40.3%) had heart failure. In-hospital mortality was 26 (8.8%)., Conclusion: There is clear delay in establishing IE diagnosis amongst ACHD patients in CESEE countries. Adequate diagnosis is hampered by common prescription of empiric antibiotics before establishing formal diagnosis. Hence, patients commonly present with associated complications requiring surgery. Hospital treatment and survival are, nevertheless, comparable to other Western European countries. Improved awareness and education of patients and medical profession regarding IE preventive measures, risks, signs, and symptoms are urgently needed. Empiric antibiotic prescription before blood cultures are taken must be omitted., Competing Interests: Declaration of Competing Interest No conflicts of interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Aims: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region., Methods and Results: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005)., Conclusion: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent., (© 2020 European Society of Cardiology.)

Objectives: The purpose of this work was to study maternal and fetal outcomes of women with uncorrected congenital heart disease (CHD)., Background: Globally, CHD is an important cause of maternal morbidity and mortality in women reaching reproductive stage. Data are lacking from larger cohorts of women with uncorrected CHD., Methods: The 10-year data from the European Society of Cardiology EORP ROPAC (EURObservational Research Programme Registry of Pregnancy and Cardiac disease) registry of women with uncorrected CHD were analyzed., Results: Of 5,739 pregnancies in 53 countries, 3,295 women had CHD, 1,059 of which were uncorrected cases. Of these, 41.4% were from emerging countries. There were marked differences between the cardiac defects in uncorrected cases versus those in corrected CHD cases with primary shunt lesions (44.7% vs. 32.4%, respectively), valvular abnormalities (33.5% vs. 12.6%, respectively), and Tetralogy of Fallot and pulmonary atresia (0.8% vs. 20.3%, respectively; p < 0.001). In patients with uncorrected CHD, 6.8% were in modified World Health Organization risk class IV, approximately 10% had pulmonary hypertension (PH), and 3% were cyanotic prior to pregnancy. Maternal mortality and heart failure (HF) in the women with uncorrected CHD were 0.7% and 8.7%, respectively. Eisenmenger syndrome was associated with a very high risk of cardiac events (65.5%), maternal mortality (10.3%), and HF (48.3%). Coming from an emerging country was associated with higher pre-pregnancy signs of HF, PH, and cyanosis (p < 0.001) and worse maternal and fetal outcomes, with a 3-fold higher rate of hospital admissions for cardiac events and intrauterine growth retardation (p < 0.001)., Conclusions: Marked differences between cardiac conditions in pregnant women with uncorrected CHD and those in corrected CHD were found, with a markedly worse outcome, particularly in women with Eisenmenger syndrome and from emerging countries., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD)., Methods: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire)., Results: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0-2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4-2.0] (n = 6) per year, with 1.1% [95%CI 0.5-2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5-8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80-93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05)., Conclusions: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)