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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.

Congenital Heart Disease (CHD) is the most commonly diagnosed congenital malformation in newborns [ 1 ]. It includes a broad spectrum of congenital heart conditions, with some of them presenting with life-threatening symptoms. However, the advancement in diagnosis and treatment of even the most complex types of CHD has led to significant improvement in the long- term outcomes of these patients.

Left ventricle non-compaction cardiomyopathy (LVNC) is still controversial on its pathogenesis and diagnosis. Some consider it an embryological abnormality with arrest of the physiological compaction of the endocardium and the myocardium, others an acquired lesion, as if it was a frustrated attempt at hypertrophy

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

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH–CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD–PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD.

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.

Cardiovascular imaging represents a fundamental part in the management of pregnant women with known or suspected cardiovascular disease. However, the available imaging modalities may carry different risks to the mother and fetus. Echocardiography is the safest imaging modality in pregnancy. It is the method of choice for evaluation of valvular and congenital heart disease, ventricular function, aortic disease, and assessment of hemodynamic parameters. Magnetic resonance is relatively safe and is invaluable for the evaluation of complex congenital heart disease, aortic disease, cardiomyopathies and myocarditis. Gadolinium can be harmful to the fetus and should be limited in pregnancy. Radiation exposure can also be deleterious to the fetus. The risk depends on the radiation dose and the gestational age at the time of exposure. Although common cardiovascular procedures that use ionizing radiation usually do not exceed radiation dose associated with significant fetal adverse effects, they should, nevertheless, be avoided whenever possible. If indicated, they should be performed preferably after the 12th week of gestation. In pregnancy, computed tomography is utilized in the diagnosis of pulmonary embolism, while coronary angiography is indicated in acute coronary syndrome. When deciding on imaging modality in pregnancy, both maternal and fetal benefits and risks must be taken into consideration.

Congenital heart disease (CHD) is the most common inborn defect, affecting approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to dramatic improvements in patients' quality of life and long-term survival. Nevertheless, despite these innovations many patients require life-long follow-up and are at risk of numerous complications, namely, residual or progressive hemodynamic lesions, arrhythmia, sudden cardiac death, and the development of heart failure and pulmonary arterial hypertension (PAH), despite timely surgical intervention. Ill advisedly, some patients are also lost to follow-up, assuming that their early life surgical or catheter-based intervention was curative, hence missing out on invaluable lifetime screening and timely interventions that may be necessary. Additionally, there are many patients with undiagnosed or unoperated CHD in the developing world presenting later in life with irreversible complications. Even in this modern era of diagnosis and screening, PAH-CHD remains a burden in patients with CHD, with approximations of up to 10% in this heterogeneous group and is associated with an adverse impact on quality of life and survival.

The systemic right ventricle (SRV), defined as the morphological right ventricle supporting the systemic circulation, is relatively common in congenital heart disease (CHD). Our review aimed at examining the current evidence, knowledge gaps and technical considerations regarding implantable cardiac electronic device therapy in patients with SRV. The risk of sinus node dysfunction (SND) after atrial switch repair and/or complete heart block in congenitally corrected transposition of great arteries requiring permanent pacing increases with age. Similar to acquired heart disease, indication for pacing includes symptomatic bradycardia, SND and high degree atrioventricular nodal block. Right ventricular dysfunction and heart failure also represent important complications in SRV patients. Cardiac resynchronisation therapy (CRT) has been proposed to improve systolic function in SRV patients, although indications for CRT are not well defined and its potential benefit remains uncertain. Amongst adult CHD, patients with SRV are at the highest risk for sudden cardiac death (SCD). Nevertheless, risk stratification for SCD is scarce in this cohort and implantable cardioverter-defibrillator indication is currently limited to secondary prevention. Vascular access and the incidence of device-related complications, such as infections, inappropriate shocks and device system failure, represent additional challenges to implantable cardiac electronic device therapy in patients with SRV. A multidisciplinary approach with tertiary expertise and future collaborative research are all paramount to further the care for this challenging nonetheless ever increasing cohort of patients.

ObjectiveTo describe the intervention spectrum, complexity, and safety outcomes of catheter-based interventions in a contemporary adult congenital heart disease (ACHD) tertiary cohort.MethodsAll patients over 16 years who underwent a catheter-based intervention for ACHD in our centre between 2000 and 2016 were included. Baseline demographics, clinical characteristics, indications for and complexity of intervention, procedural complications and early and mid-term mortality were analysed.ResultsOverall, 1644 catheter-based interventions were performed. Intervention complexity ranged from simple (67.5%) to intermediate (26.4%) and to high (6.1%). Commonly performed procedures were atrial septal defect (33.4%) and patent foramen ovale closure (26.1%) followed by coarctation of the aorta (11.1%) and pulmonary artery interventions (7.0%). Age at index intervention was 40±16 years, 758 (46.1%) patients were male, 73.2% in New York Heart Association (NYHA) class I, 20.2% in NYHA class II, whereas 6.6% in NYHA class III/IV. In-hospital mortality was 0.7%. Median postinterventional length of stay was 1 day. Complications occurred in 129 (7.9%) with major adverse events in 21 (1.3%). One-year postintervention survival rates were 98.7% (95% CI 98.2 to 99.2). Over the study period, there was a notable shift in intervention complexity, with a predominance of simple procedures performed in early years and more complex procedures in later years. Furthermore, the case mix during the study broadened (pConclusionThis study shows an increasing complexity and expanding variability of ACHD catheter-based interventions, associated with low major complications, short hospital stays and low early and mid-term mortality. Ongoing investment in ACHD catheter interventions is warranted.

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.

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.

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.

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.

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 …

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure at rest ⩾ 25 mmHg as assessed by right heart catheterization. Haemodynamically PH can be further distinguished into pre-capillary PH depending on the presence of a raised left atrial or pulmonary wedge pressure. Post-capillary PH is common in patients with congenital heart disease, in whom systemic ventricular dysfunction, valve disease, outflow tract obstruction or pulmonary venous stenosis can lead to a rise in post-capillary pressures. In this chapter, we discuss the pathogenesis, impact and management of postcapillary PH in congenital heart disease.

The Fontan operation was introduced in 1971 and represented a milestone in the management of congenital heart disease with univentricular heart physiology. Even today it remains the main palliation and a life-saving procedure for children born with a functionally univentricular cardiac anatomy that is not amenable to biventricular repair. The surgical palliation consists of the separation of the systemic and pulmonary venous returns without the use of a subpulmonary ventricle. Since its original description with an atriopulmonary anastomosis, multiple modifications of the procedure have been applied.1 Today, more than 40 years after the initial procedure, we are witnessing a substantial number of adult patients living with Fontan circulation. All Fontan variants share similar physiology in which one effective ventricle delivers oxygenated blood to the systemic circulation and gradient between central venous pressure and left atrial pressure drives passively deoxygenated blood into the pulmonary arteries and the lungs. The Fontan physiology is —in part—paradoxical in that it imposes systemic venous hypertension with concomitant pulmonary …

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.

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 …

Intracardiac fistulas are rare complications of infective endocarditis that contribute to the complexity of surgical management, and impose an additional hemodynamic burden on the already challenged heart. We report on a case of successful surgical management of a paravalvular communication between the left ventricle and the left atrium via an abscess cavity.