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Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.

AIMS: To develop a suite of quality indicators (QIs) for the evaluation of the care and outcomes for adults with pulmonary arterial hypertension (PAH). METHODS AND RESULTS: We followed the European Society of Cardiology (ESC) methodology for the development of QIs. This included (i) the identification of key domains of care for the management of PAH, (ii) the proposal of candidate QIs following systematic review of the literature, and (iii) the selection of a set of QIs using a modified Delphi method. The process was undertaken in parallel with the writing of the 2022 ESC/European Respiratory Society (ERS) guidelines for the diagnosis and treatment of pulmonary hypertension and involved the Task Force chairs, experts in PAH, Heart Failure Association (HFA) members and patient representatives. We identified five domains of care for patients with PAH: structural framework, diagnosis and risk stratification, initial treatment, follow-up, and outcomes. In total, 23 main and one secondary QIs for PAH were selected. CONCLUSION: This document presents the ESC QIs for PAH, describes their development process and offers scientific rationale for their selection. The indicators may be used to quantify and improve adherence to guideline-recommended clinical practice and improve patient outcomes. ispartof: EUROPEAN JOURNAL OF HEART FAILURE vol:25 issue:4 pages:469-477 ispartof: location:England status: published

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.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.

Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED.

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)—by definition—have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care.

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.

Funding Acknowledgements Type of funding sources: None. BACKGROUND Myocardial work (MW) estimation by pressure-strain loops (PSL) allows a non-invasive evaluation of myocardial performance, as recently demonstrated in adult patients. Aim of this study is to provide the reference values for global myocardial work index (MWI), constructive work (MCW), wasted work (MWW), and work efficiency (MWE) in a group of healthy children. METHODS Assessment of MW was performed using a commercially available software package (Echopac, GE). MW was measured from PSLs areas, derived from non-invasive LVP curves combined with strain acquired speckle tracking echocardiography (STE). After calculating GLS, values of brachial blood pressure were inserted and the time of valvular events by echocardiography were indicated, then the software was able to measure non-invasive PSLs. RESULTS Two-dimensional (2D) standard and speckle-tracking echocardiography were performed in 90 healthy children (mean age 9.9 ±4.9 [1-17] years, females: 57%) together with the assessment of MW by means of PSLs. Mean ± standard deviation, 5° and 95° percentile values for global MWI, MCW, MWW, and MWE in the whole population were 1769 ± 254 mm Hg, (1354-2193); 2201 ± 290 mm Hg, (1657-2658); 78 ± 47 (29-163) mm Hg%; 96 ± 1.8 (92-99)%, respectively. CONCLUSIONS The assessment of MW is feasible in healthy children. This study provides useful 2-dimensional echocardiographic reference ranges for novel indices of non-invasive MW.

Aims Women’s participation is steadily growing in medical schools, but they are still not sufficiently represented in cardiology, particularly in cardiology leadership positions. We present the contemporary distribution of women leaders in cardiology departments in the World Health Organization European region. Methods and results Between August and December 2020, we applied purposive sampling to collect data and analyse gender distribution of heads of cardiology department in university/third level hospitals in 23 countries: Austria, Azerbaijan, Belgium, Bosnia-Herzegovina, Croatia, France, Germany, Greece, Italy, North Macedonia, Morocco, Poland, Portugal, Russia, Serbia, Slovakia, Slovenia, Spain, Switzerland, Tunisia, Turkey, Ukraine, and the UK. Age, cardiology subspecialty, and number of scientific publications were recorded for a subgroup of cardiology leaders for whom data were available. A total of 849 cardiology departments were analysed. Women leaders were only 30% (254/849) and were younger than their men counterpart (♀ 52.2 ± 7.7 years old vs. ♂ 58.1 ± 7.6 years old, P = 0.00001). Most women leaders were non-interventional experts (♀ 82% vs. ♂ 46%, P < 0.00001) and had significantly fewer scientific publications than men {♀ 16 [interquartile range (IQR) 2–41] publications vs. ♂ 44 (IQR 9–175) publications, P < 0.00001}. Conclusion Across the World Health Organization European region, there is a significant gender disparity in cardiology leadership positions. Fostering a diverse and inclusive workplace is a priority to achieve the full potential and leverage the full talents of both women and men.

Background An increasing number of patients with previously repaired congenital heart disease (CHD) present with pulmonary arterial hypertension (PAH). This can occur immediately after repair (residual PAH) or years later. Case presentation We present the case of a young woman who underwent repair of a ventricular septal defect in later childhood. Three years after repair, she was found to have significant residual PAH. She remained stable on PAH therapies, but a decade later decided to become pregnant against medical advice. She deteriorated during pregnancy and required escalation of PAH therapies and eventual admission to the intensive care unit, with an uneventful delivery at 32 weeks. Despite successful delivery, she remained symptomatic post-partum, with evidence of disease progression at right heart catheterisation. Conclusions All patients with repaired CHD should undergo routine screening for PAH. Early diagnosis and expert management, including the use of PAH therapies, is recommended to optimise outcome. Pregnancy is contraindicated in PAH patients, including patients with CHD, and requires an expert multidisciplinary approach to reduce morbidity and mortality when patients opt to proceed.

Background Most of North and Western European countries recognized long ago the importance of specialized ACHD programmes and have today well-established Centre of excellence for management of ACHD patients. In contrast, even though Central and South-eastern (CESE) European region geographically comprise almost half of the European continent, little is known about ACHD status in vast majority of its countries. Purpose We aim to provide for the first time contemporary comprehensive overview of ACHD situation in CESE Europe. Methods We obtained data regarding current national ACHD status from 19 CESE European countries. Over the country national cardiac society, the ESC Working Group on ACHD has identified physician/s actively involved in ACHD care in these countries. Country's representative/s filled out an extensive survey regarding the provision of care for the year 2017 or/and 2018 comprised from five main questionnaires: country and hospital ACHD information, clinical activity information, interventional, and surgical cardiac procedures, infrastructure and staffing, health care system including funding, and education. Results The majority of countries have specialized ACHD centre; 13 out of 19 countries have a national tertiary centre. The median number of adult cardiologists and cardiologists specifically involved in ACHD care per country is 400 and 3, respectively. The median number of ACHD centres per country was one; year of establishment was 2007. Six countries have no dedicated centre. With the exception of Albania, these countries were relatively young, all have significantly lower GDP/capita in comparison with other CESE countries with an established service (p=0.005). The median number of outpatient visits and hospital admission per year was 900 and 135, respectively; cardiac catheter interventions and cardiac operations yearly were 49 and 40. Thirteen countries have a public health care system funded by their government, while six have also a small portion of private reimbursement. However, all countries have a financial cap imposed on ACHD care per hospital, leading to a patient waiting list and a restriction in the number of procedures. Conclusion In the past decades, the CESE European region has made significant progress in the state-of-the art ACHD care. The majority of countries nowadays have established ACHD services with a substantial patient workload comparable to the rest of Europe. Moreover, most centres are equipped with the necessary infrastructure. Contemporary provisions of ACHD care in the CESE European region is nevertheless challenged by generally lower financial resources, lower staffing levels and de-facto caps on the possibility to perform certain necessary procedures in comparison with Western European countries. These government healthcare financial constraints also restrict the required expansion in terms of numbers and complexity of surgical and interventional procedures locally. Funding Acknowledgement Type of funding source: None

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.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).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.

The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.

U svrhu obilježavanja pete godišnjice sustavne brige za odrasle bolesnike s prirođenom srčanom bolesti organizirali smo u Nacionalnom centru u Kliničkom bolničkom centru Zagreb sastanak uz prikaz kompleksnih kliničkih slučajeva iz tog područja. Zbog pandemije COVID a 19 sastanak je otkazan, ali je umjesto toga održan multidisciplinarni konzilij uz sudjelovanje vrhunskoga međunarodnog stručnjaka. Prikazano je više bolesnika i doneseni zaključci o njihovu daljnjem liječenju. Ovdje iznosimo podatke o četirima bolesnicima: o dva bolesnika sa slučajno otkrivenom kasnom dijagnozom prirođene srčane bolesti (o jednom s atrijskim septalnim defektom i drugom s Fallotovom tetralogijom), o bolesnici sa slomom Fontanove cirkulacije, liječenom transplantacijom srca te o bolesnici s transpozicijom velikih krvnih žila i problemima nastalima nakon operacije. Uz prikaze bolesnika, u raspravama su iznesene dvojbe u vezi s liječenjem te najvažniji zaključci vezani uz svaki pojedini slučaj., To mark the fifth anniversary of systematic care for adult patients with congenital heart disease, we organized a meeting at the National Center at the University Hospital Centre Zagreb with the intention of presenting complex clinical cases from the field. The meeting was canceled due to the COVID-19 pandemic, but a multidisciplinary council was held with the participation of an international top expert. Several patients were presented and conclusions were made about their further treatment. Herein we present four interesting cases: two patients with an accidental late diagnosis, one with atrial septal defect and a second with tetralogy of Fallot, a patient with failing Fontan circulation treated by heart transplantation, and a patient with surgically corrected transposition of great arteries with postoperative problems. In addition to patient reports, the discussions sections present dilemmas about treatment, available literature, and conclusions related to each case.

Introduction Brain natriuretic peptide (BNP) is a well-established marker for heart failure in the general population however limited data are available on the value of BNP as a diagnostic and prognostic marker in adults with congenital heart disease (ACHD). The purpose of our study is to evaluate the relation between BNP levels and biventricular systolic and diastolic function in patients with ACHD. Methods We evaluated clinically stable ACHD patients who underwent echocardiography from May 2015 until January 2018. We studied the correlation of BNP with the degree of systolic and diastolic dysfunction of the left ventricle. Moreover we also investigate the relation of BNP with the right tricuspid annular plane systolic excursion (TAPSE), the fractional area change (FAC) and the pulmonary arterial systolic pressure (PASP). Results In total, 385 patients were included in our study (median age, 43 ±12 years; 61% male). Of the 385 patients, 193 (50%) had tetralogy of Fallot, 94 (24%) had systemic RV, including patients with transposition of the great arteries (TGA) after atrial switch operation (Mustard or Senning) and congenitally corrected TGA -ccTGA , 43 (11%) had univentricular hearts and Fontan physiology, 55 (14%) had other lesions. BNP levels were 66 ± 26 pg/mL and 93, ±31pg/mL, for patients with EF > 50% and 50%> EF >35% respectively (p = 0.003). BNP levels correlate with parameters reflecting LV filling pressure, including transmitral early diastolic velocity (E) 81 ± 29pg/mL and its ratio to early diastolic annular myocardial tissue velocity (E/Ea) 77 ± 17pg/mL. (r=-0.607, p = 0.003 r=-0.598, p = 0.005, respectively) BNP levels were also significantly higher in patients with impaired tricuspid annular plane systolic excursion 87 ± 21 pg/mL (TAPSE

Introduction Cardiac resynchronization therapy (CRT) has become a treatment of choice in patients with chronic heart failure (HF). About 25% of patients with systemic right ventricle (SRV) progress to symptomatic HF, which may be refractory to drug therapy and is commonly associated with significant morbidity and mortality. For these reasons, CRT has been emerging as an effective treatment strategy for patients with SRV failure and electrocardiographic signs of ventricular dyssynchrony. Few studies have reported the acute and long - term effects of CRT in SRV subjects, with different findings. Our study aimed to describe the experience with CRT in SRV patients in a single tertiary centre. Purpose Assess the long term efficacy of CRT in patient with SRV Materials and methods All consecutive SRV patients who underwent CRT implantation and/or upgrading between 1994 and 2018 at our tertiary centre were included. Clinical and echocardiographic parameters before and after CRT implantation were collected and analyzed. Results A total of 21 patients (mean age 47.8±14.8 years, 13 M) were implanted with CRT-P (12, 57%) or CRT-D (9,43%) during the study period. 90% of patients showed an anatomy of congenitally corrected transposition of the great arteries (CCTGA), whereas 9.5% underwent Mustard procedure for transposition of the great arteries (TGA). Among CCTGAs, 11 (52.5%) subjects underwent previous surgical procedures, including implant of a conduit between the left ventricle (LV) and the pulmonary artery (PA) in 8 (38%) patients, tricuspid valve repair in 2 (9.5%) and surgical closure of atrial septal defect in 1 (5%). Before CRT implant/upgrading, 10 (48%) patients had a moderate to severe reduction in the SRV ejection fraction (EF) and 7 (33%) had a moderate to severe tricuspid regurgitation (TR). Overall, 15 (71%) patients referred a NYHA II or III. After a median follow up of 57 months (IQR 35–83), 43% of patients showed an improvement in their functional status, which was associated with an improvement of SRV EF and TR only in 22% and 33% of these patients. On the contrary, no patient reported a worsening in NYHA class, while SRV EF decreased in 28.5% and the grade of TR worsen in 23.8% of patients. Conclusions CRT is emerging as an effective treatment for SRV dysfunction. However, criteria for implantation are not well defined and the deterioration of SRV function related to subpulmonary univentricular pacing should be considered. Moreover, TR did not improve in this study, suggesting that concurrent tricuspid valve interventions may be necessary in patients with severe TR and may facilitate the improvement in RV function achieved with CRT. Proper planning, tertiary expertise and international collaborations are all paramount in this field.

Atrijski septalni defekt (ASD) najčešća je srčana greška u odrasloj dobi. Često se kasno dijagnosticira. Potrebno je posumnjati na ovaj defekt kad se ehokardiografski registrira uvećana desna klijetka. Prikazana su dva slučaja pacijenata starijih od 50 godina s novodijagnosticiranim ASD-om. U obaju bolesnika greška se prezentirala s aritmijom ( brilacija atrija, odnosno totalni atrio-ventrikulski blok) i učinjena je uspješna kirurška, odnosno intervencijska korekcija defekta. Ako nema kontraindikacija, a najčešća je razvoj značajne plućne hipertenzije, odnosno plućne vaskularne rezistencije, preporučuje se aktivni stav, tj. zatvaranje defekta, jer znatno pridonosi kliničkom poboljšanju, ali i smanjenju mortaliteta., Atrial septal defects (ASD) are the most common heart defect in adult patients and are often diagnosed late. This defect should be suspected whenever echocardiographic imaging nds an enlarged right ventricle. We report two cases of patients with newly-diagnosed ASD that were older than 50 years of age. In both cases the patients presented with arrhythmia (atrial brillation, i.e. total atrioventricular block), and a successful correction of the defect was performed. If there are no contraindications, that is primarily the development of signi cant pulmonary hypertension i.e. pulmo- nary vascular resistance, the active approach – defect closure – is recommended, since it signi cantly contributes to clinical improvement and reduction in mortality.

While virus epidemics are nothing new to man, the scale, speed of global spread and immediacy of the COVID-19 pandemic have been truly unprecedented [ 1 ]. The entire world has been turned on its head in less than a few months, with major implications beyond disease burden and loss of life, threatening the economic status quo and human psychosocial balance and wellbeing not only for patients, but for all of us. The primary aim of our Call for Action Viewpoint was to support and protect our adult congenital heart disease (ACHD) patients and their needs during these challenging and uncertain times. This goal had to be met while we, as individuals, teams, institutions and nations, came together in a global effort to combat this aggressive virus, that appears to spare no organs or systems, nor any borders, geographic or other. As with any crisis, there is always opportunity: we are submitting herewith a vision for a different and better model of ACHD care, and for a better life journey and health care experience for our patients, that should be in place in the aftermath of the Covid-19 pandemic. Many of the points and principles discussed in this article, need not be confined to ACHD patients, but have a broader reach. And we must not forget nor neglect the most vulnerable in society at this time, namely the elderly, disabled and other dependent or disadvantaged groups in this “We Are One” global operation. Last but not least, this maybe the time to take better care of ourselves (and others) and reflect on life.

The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success story of modern medicine. In the 1950s survival of children born with CHD was only approximately 15%, whereas nowadays more than 90% of these children survive well into adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients with CHD over 60 years old. Many adult patients are afflicted by residual haemodynamic lesions and also face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non-cardiac pathology etc., necessitating integrated care and all medical disciplines. We are faced with a ‘tsunami’ in terms of adult CHD numbers, disease heterogeneity, and complexity of work and interventions needed. We need to secure resources, welcome more people in our field, learn from ‘marching with our patients’, and educate better patients, public, and ourselves so that every single patient with CHD, born anywhere in the world, may reach their full life potential.

Aims To assess the utility of machine learning algorithms on estimating prognosis and guiding therapy in a large cohort of patients with adult congenital heart disease (ACHD) or pulmonary hypertension at a single, tertiary centre. Methods and results We included 10 019 adult patients (age 36.3 ± 17.3 years) under follow-up at our institution between 2000 and 2018. Clinical and demographic data, ECG parameters, cardiopulmonary exercise testing, and selected laboratory markers where collected and included in deep learning (DL) algorithms. Specific DL-models were built based on raw data to categorize diagnostic group, disease complexity, and New York Heart Association (NYHA) class. In addition, models were developed to estimate need for discussion at multidisciplinary team (MDT) meetings and to gauge prognosis of individual patients. Overall, the DL-algorithms-based on over 44 000 medical records-categorized diagnosis, disease complexity, and NYHA class with an accuracy of 91.1%, 97.0%, and 90.6%, respectively in the test sample. Similarly, patient presentation at MDT-meetings was predicted with a test sample accuracy of 90.2%. During a median follow-up time of 8 years, 785 patients died. The automatically derived disease severity-score derived from clinical information was related to survival on Cox analysis independently of demographic, exercise, laboratory, and ECG parameters. Conclusion We present herewith the utility of machine learning algorithms trained on large datasets to estimate prognosis and potentially to guide therapy in ACHD. Due to the largely automated process involved, these DL-algorithms can easily be scaled to multi-institutional datasets to further improve accuracy and ultimately serve as online based decision-making tools.

Congenital heart disease (CHD) is the most common inborn defect. Due to advances in paediatric care, surgical, and catheter procedures the number of adults with CHD has grown remarkably in recent years. Most of these patients, however, have residua from their original operation/s and require life-long care, many of them are subjected to further haemodynamic and electrophysiological interventions during adulthood. While such re-do surgical or catheter interventions together with device therapy and transplantation play a key therapeutic role, increasingly, adults with CHD require drug therapy for late complications namely heart failure (HF), arrhythmias, pulmonary and systemic hypertension, thromboembolic events, etc. Unlike other cardiovascular areas, drug therapy in adult CHD is based on scarce clinical data and remains largely empiric. Consequently, pharmacological therapies are individualized to ameliorate patients' symptoms and/or degree of haemodynamic impairment. Thus far, recommendations have been difficult to make and formalized guidelines on drug therapy are lacking. We review herewith the rationale, limited evidence and knowledge gaps regarding drug therapy in this growing cardiovascular field and discuss pharmacotherapy options in specific conditions namely HF, arrhythmias, thrombosis, pulmonary arterial hypertension, contraception, and pregnancy.

Broj je bolesnika s prirođenim srčanim greškama u odrasloj dobi (PSGO) u porastu pa premda njihov točan broj i raspodjela u Hrvatskoj nisu poznati, potreba za njihovim praćenjem i liječenjem u specijaliziranom centru postaje nužnost. Prema procjeni radi se o više od 12.000 bolesnika kod kojih se u većini slučajeva očekuje neka od kasnijih komplikacija i potreba za jednom ili više procedura. U preporukama Europskog kardiološkog društva dane su smjernice za broj potrebnih centara, kao i za njihovu organizaciju. U Kliničkom bolničkom centru Zagreb započeta je organizacija centra. Opisani su prvi koraci koji uključuju organizaciju ambulante za PSGO, redoviti multidisciplinarni konzilij, kao i formiranje registra te prvi koraci u edukaciji kardiološke zajednice. U okviru Centra u kratkom je razdoblju pregledan velik broj bolesnika, što je omogućilo uvid u probleme te skupine bolesnika. Ovdje donosimo tri prikaza slučajeva. Prvi je pacijentica s operiranom tetralogijom Fallot kod koje nije pravilno dijagnosticirana i liječena aritmija, što može dovesti do znatnog kliničkog pogoršanja. Druga je pacijentica s neprepoznatom korigiranom transpozicijom, zbog manjka iskustva kardiologa. Iako pacijentica još nema značajnih tegoba, one se očekuju u budućnosti i stoga je postavljanje dijagnoze bilo ključno. U trećem slučaju prikazana je tipična PSGO pacijentica s multiplim operacijama u djetinjstvu kod koje se trajno traže nova rješenja; u ovom slučaju nekirurška implantacija zalistka na aortnoj poziciji. Ovaj prikaz ukazuje i na posebnost liječenja te skupne mladih ljudi kojima je osobito važna kvaliteta života., Due to the growing number of patients with adult congenital heart disease (ACHD), although their exact number and distribution in Croatia is not yet known, monitoring and treating these patients in specialized centers has become a necessity. Their number is estimated at 12 000, and in most cases these patients will require one or more surgical procedures due to complications later in life. The recommendations of the European Society of Cardiology include guidelines on the necessary number of specialized centers and their organization. Organization of such a center has begun in the Zagreb Clinical Hospital Center. This article describes the first steps being taken, which include setting up a clinic for ACHD, regular multidisciplinary councils, formation of a patient registry, and first steps towards educating the cardiologic community. A large number of patients were examined in the Center recently, providing insight into the specific problems in this patient population; we will present three cases here. The first is a woman with a surgically repaired tetralogy of Fallot but with an improperly diagnosed and treated case of arrhythmia, which can lead to serious clinical deterioration. The second patient is a woman with a case of corrected transposition that went undiagnosed due to a lack of experience on part of the cardiologist. Although the patient has not experienced significant difficulties, they are expected in the future, making a correct diagnosis extremely important. The third case is a typical ACHD patient, a woman that had undergone numerous surgeries in childhood in a constant search for a long-term solution, which in this case was a non-surgical valve replacement at aortic position. This overview also outlines specific challenges faced when treating young people who put especially high value on the quality of life.

Congenital anomalies of the mitral valve may be isolated lesions or associated with other complex congenital heart defects. Each level of the mitral valve can be affected from the annulus, leaflets, chordae tendineae and papillary muscles. Isolated mitral stenosis is one of the rarest form of congenital heart disease (0.6%) but can be associated with other left side abnormalities1 . Most cases of mitral stenosis are diagnosed in early childhood leading to mitral valve repair or replacement. Unoperated congenital mitral stenosis in an adult will be limited to rather mild or moderately severe stenosis with symptoms and complications identical to rheumatic mitral valve disease. Mitral valve regurgitation due to primary mitral valve prolapse has a prevalence of 2.4% and is the most frequently diagnosed cardiac valvular abnormality. It occurs mostly as an isolated valve dysfunction but can be associated with connective tissue diseases or other cardiac abnormalities. Echocardiography is the definite diagnostic method for congenital mitral valve anomalies but in presence of multiple lesions, hemodynamic assessment by cardiac catheterization can be useful.

The increase in the number of adult female patients with congenital heart disease (ACHD) will result in pregnancy complications in this populations becoming a more common problem faced by physicians. Patients with intermediate or very complex congenital heart disease, often accompanied by reduced heart function with increased cardiac minute volume, as is the case in pregnancy, will be susceptible to health issues such as heart failure, arrhythmia, loss of pregnancy, pre-term birth, low birthweight, etc. Pregnancy can also represent a direct danger to the morbidity and mortality of the mother, but can also be associated with poorer outcomes after the pregnancy. There are dangers for the child as well, such as restricted growth or the teratogenic effects of drugs. In most patients with ACHD, pregnancy is possible but with an increased risk for the mother and child. We present five patients with moderate or complex heart defects and pregnancy. We have stratified patients have been stratified according to risk and demonstrated the need for monitoring such patients in a specialized ACHD center.

We thank Dr Murakami and his colleagues for their interest in our article.1 They express concern for the potential risk of patients on β-blockers with sepsis or pneumonia, and especially patients with preexisting asplenia, developing intractable hypoglycaemia and catecholamine-resistant hypotension. There is no evidence in the literature or any plausible mechanism suggestive of a direct link between β-blockade and such …

3. Vaidiyanathan D, Prabhakar D, Selvam K, Alagesan R, Thirunavukarasu N, Muthukumar D. Isolated congenital left ventricular diverticulum in adults. Indian Heart J. 2001;53(2):211-3. Congenital diverticulum of the left ventricle is a very rare cardiac anomaly due to local embryological developmental failure. We present a case of a 22-year old male patient. He is a former athlete who as a part of preoperative examination for the planned right knee surgery made an ECG which showed signs of left ventricular hypertrophy. Two-dimensional transthoracic echocardiography revealed rare congenital heart disorder in the form of large left ventricular apical diverticulum. Diverticulum measured 5.2x2.4 cm and occupied left ventricular apex with an entrance width of 2.3 cm. Diverticulum exhibited good wall contractility with three layer structure, accentuated trabeculations as well as aberrant cords imitating third AV valve at the diverticular entry. Overall, diverticulum appeared much alike small third ventricle. The patient had normal coronary angiography findings, negative family history of sudden cardiac death and normal 24hour Holter ECG — sinus rhythm without arrhythmias. Taking into account these points, mainly the absence of associated other cardiac abnormalities and since the patient was asymptomatic we decided to follow up the patient without surgical treatment.

Interactions of MinK and e-NOS Gene Polymorphisms Appear to Be Inconsistent Predictors of Atrial Fibrillation Propensity, but Long Alleles of ESR1 Promoter TA Repeat May Be a Promising Marker. We analyzed minK, e-NOS and ESR1 gene polymorphisms in 40 patients with atrial fibrillation (AF) without major structural heart disease compared to 35 healthy controls. A missense polymorphism in the minK gene with A/G substitution at nucleotide 112 causing serine (S) to glycine (G) change, 786 T/C polymorphism in the 5' flanking region of e-NOS gene and TA polymorphism in the regulatory region of estrogen receptor ESR1 gene with long (> or = 19 TA repeats) and short alleles were examined. Only a slight increase in minK G allele frequency, but with marked excess in AG/TT combination of minK and e-NOS polymorphisms was found in the AF group. The interpretation remains tentative due to small groups precluding statistical significance of differences, possible lab flaws and inconsistencies with earlier data. However, ESR1 long allele homozygotes were strikingly more frequent in the AF than in control group, reaching statistical significance surprisingly in males (p < 0.02). Functional activity of estrogen receptors may be more critical in males than in females with abundance of circulating estrogen. Contrasting the intricate complexity of genetic polymorphisms influencing cardiac rhythm with our modest research, we would limit the conclusion to the plea for further research of ESR1 role in AF.

A 21-year-old male refugee from Syria with known but ill-defined congenital heart disease, presented with palpitations, no signs of heart failure, and near normal oxygen saturation. Chest radiography showed a globally enlarged heart with prominent hilar and pulmonary vessels ( Panel A ). Transthoracic echocardiography revealed a double-inlet-left-ventricle (DILV) with good systolic function ( Panel B ), malposition of the …

cilj: Srcano ostecenje uzrokovano karcinoidnim sindromom je rijedak, ali zanimljiv i važan uzrok trikuspidalne i pulmonalne regurgitacije koje mogu dovesti do znacajnog morbiditeta i mortaliteta uzrokovanog zatajivanjem desnoga srca. Kada se stanje lijeci medikamentozno, i u adekvatnih kandidata kirurski, može se postici znacajna korist za ukupnu kvalitetu života i dugogodisnje preživljenje. Uloga ovoga prikaza slucaja je naglasiti važnost klinicke prosudbe u lijecenju pacijenata s karcinoidnim sindromom uz zahvacanje desnoga srca, kao i prikaz tipicnih i zanimljivih ehokardiografskih zapisa.