Background/Objectives: Progress in diagnostic and therapeutic strategies has resulted in an increasing prevalence of adults with congenital heart disease (ACHD), including those involving genetically determined syndromes. This study aimed to characterize prevalence, congenital phenotypes, heart failure (HF) stages, comorbidity burden, and current medical management of ACHD and concomitant genetically determined syndromes enrolled in a prospective HF-focused registry. Methods: The PATHFINDER-CHD Registry is a German-based (est. 2022) multicenter observational registry. This web-based platform consecutively tracks ACHD patients across the heart failure spectrum, including those with current or prior HF, as well as those at high structural or functional risk. HF stage was classified using a modified ACC/AHA scheme adapted for CHD; functional capacity was graded according to the Perloff classification. Baseline demographics, CHD anatomy, prior surgical/interventional treatment, cardiac and extracardiac comorbidities, and medication were collected from medical records. Results: Among 1987 enrolled ACHD, 107 (5.4%) had a genetic syndrome (n = 65, 60.7% women; mean age 33.5 ± 9.4 years; range 18-68). Most common syndromes were trisomy 21 (n = 49; 45.8%) and 22q11.2 deletion (n = 27; 25.2%); 31 patients (30.0) had rarer syndromes. Predominant CHD diagnoses were atrioventricular septal defect (n = 42, 39.3%), tetralogy of Fallot (n = 19, 17.8%), and pulmonary atresia with ventricular septal defect (n = 7, 6.5%). A systemic left ventricle was present in 102 (95.3%); 40 (37.4%) had primarily cyanotic CHD, and 7 (6.5%) an Eisenmenger physiology. Most patients (n = 71; 66.4%) had undergone definite surgical repair; 25 patients (23.3%) had at least one catheter intervention, including transcatheter valve implantation in 17 cases (15.9%). HF stage was mainly B (n = 30, 28.0%) or C (n = 75, 70.1%). Perloff functional class I/II was present in 97 (90.7%). Leading cardiac comorbidities included intrinsic aortopathy (n = 49, 45.8%), pulmonary arterial hypertension (n = 12, 11.2%), and arrhythmias (n = 10, 9.3%). Frequent extracardiac comorbidities were thyroid dysfunction (n = 34, 31.8%), kidney disease (n = 16, 15.0%), hyperuricemia (n = 13, 12.1%), and depression (n = 15, 14.0%). Pharmacotherapy was used in 66 patients (61.7%). Beta-blockers (n = 25, 23.4%) were common, while ACEi/ARB (n = 9, 8.4%), diuretics (n = 10, 9.3%), MRAs (n = 8, 7.5%), and SGLT2 inhibitors (n = 3; 2.8%) were infrequently prescribed; no patient received ARNI or digitalis. For targeted treatment of pulmonary arterial hypertension, phosphodiesterase-5 inhibitors (n = 7, 6.5%), endothelin receptor antagonists (n = 6, 5.6%), or prostacyclin analogues (n = 1, 0.9%) were used. As oral anticoagulants, vitamin K antagonists or direct oral anticoagulants (DOACs) were prescribed in 17 cases (15.9%). Forty-one patients (38.3%) received thyroid hormone replacement. Conclusions: Syndromic ACHD constitute a small but clinically high-risk subgroup within an HF-oriented registry, marked by complex CHD, substantial cardio-extracardiac multimorbidity (notably aortopathy, PAH, thyroid disease, renal dysfunction, depression), and low utilization of contemporary HF therapies. These data support specialized, interdisciplinary, longitudinal care pathways and prospective studies addressing outcomes and evidence-based HF management in syndromic ACHD.

AIMS:Cardiac rehabilitation (CR) is an essential component of long-term care for adults with congenital heart disease (ACHD). This study provides novel patient-reported data on barriers to cardiac rehabilitation in ACHD, a population that has so far been insufficiently studied in this respect. The objective was to investigate knowledge, sources of information, barriers to access, and expectations regarding CR in ACHD and to analyse age-specific and sex-specific differences. METHODS AND RESULTS:A pragmatic cross-sectional survey was conducted between January and November 2025 as part of routine follow-up visits in the ACHD outpatient clinic. A total of 402 ACHD were included. After plausibility checks, 345 participants were analysed (49.9% women; mean age 40.7 years in both groups). The severity of the congenital heart defect was predominantly subjectively rated as moderate to severe. Self-rated general health decreased significantly with increasing age. Only 24.2% actively searched for information about CR and reported poorer health status compared with those who did not search. The main information sources were the internet and interpersonal contacts. Women more frequently used self-help groups and peer support services. Regardless of active information seeking, about one quarter reported having been informed about rehabilitation options by physicians. Social services were used less frequently but rated particularly helpful. The majority preferred receiving information during personal physician consultations (86.3%). Women additionally more often preferred brochures and telephone counselling, while younger participants more often preferred digital formats. ACHD-specific CR programmes were known to only 32.8%. Procedural knowledge gaps were pronounced. Only 31.5% knew how to apply for CR, and the process was predominantly perceived as difficult. Overall, 34.4% had already participated in rehabilitation programmes. Older participants participated more frequently but also reported more frequent application rejections. Family obligations prevented participation in 9.8% and were significantly more common among women. Psychological barriers were reported by 14.6%. Expectations regarding rehabilitation were high. More than 90% expected physical improvement, and 61.2% expected psychological stabilization, with women significantly more often rating psychological support as very important. CONCLUSION:Despite high expectations, the utilization of CR among ACHD remains low. Substantial knowledge and procedural deficits as well as age-specific and sex-specific barriers indicate the need for multimodal, target group-specific information strategies; structured referral processes; low-threshold support in the application and access process; and strong integration of psychosocial components.

Adults with congenital heart disease (ACHD) represent a steadily growing patient group whose care poses new medical, organizational, and socio-medical challenges. While survival rates have increased significantly thanks to modern pediatric cardiology and cardiac surgery, residual and sequelae conditions almost always remain. Many patients experience heart failure, cardiac arrhythmias, pulmonary vascular diseases, aortic diseases, or psychosocial stress, as well as cardiac and non-cardiac comorbidities.Differentiated follow-up care requires specialized expertise and lifelong structured care, in which the specific characteristics of heart defects must be taken into account. There are significant differences compared to acquired heart diseases! Structured prevention, prehabilitation, and rehabilitation must be guaranteed as integral components of holistic care in the future.

Pulmonary Hypertension (PH) is characterized as a hemodynamic disorder defined by a mean pulmonary arterial pressure (mPAP) exceeding 20 mmHg at rest. Classification into distinct subtypes is guided by measurements of pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR): Precapillary PH: PAWP ≤ 15 mmHg accompanied by PVR > 2 Wood Units (WU), isolated Postcapillary PH: PAWP > 15 mmHg with PVR ≤ 2 WU and combined Pre- and Postcapillary PH: PAWP > 15 mmHg with PVR > 2 WU.Exercise-Induced PH refers to a pathophysiological condition in which resting mPAP is within normal limits but exhibits an exaggerated rise during physical exertion. This is quantified by a slope of mPAP relative to cardiac output exceeding 3 mmHg per liter per minute between rest and activity.The foundational framework for the clinical classification of PH, comprising five principal groups, remains unchanged. Nevertheless, several updates have been introduced: Reintegration of long-term responders to calcium channel blockers as a distinct subset within idiopathic pulmonary arterial hypertension (IPAH), Inclusion of new subcategories under Group 2 PH, which encompasses PH associated with left heart disease and Revision of Group 3 PH to categorize patients based on underlying pulmonary pathology rather than solely functional impairment.Additionally, Mitomycin-C and Carfilzomib have been recognized as pharmacologic agents with a confirmed causal relationship to the development of pulmonary arterial hypertension (PAH) and have thus been added to the list of definitively associated drugs.

Background: The PATHFINDER-CHD Registry is a prospective, multicenter, non-interventional registry across tertiary care centers in Germany. The aim is to analyze real-world data on adults with congenital heart defects (ACHD) or hereditary connective tissue disorders who have manifest heart failure (HF), a history of HF, or are at significant risk of developing HF. This analysis investigates the prevalence and clinical impact of overweight and obesity in this unique population. Methods: As of 1st February, 2025, a total of 1490 ACHD had been enrolled. The mean age was 39.4 ± 12.4 years, and 47.9% were female. Patients were categorized according to Perloff’s functional class and the Munich Heart Failure Classification for Congenital Heart Disease (MUC-HF-Class). Results: The most common congenital heart disease (CHD) in this cohort was Tetralogy of Fallot, transposition of the great arteries, and congenital aortic valve disease. Marfan syndrome was the most common hereditary connective tissue disease. Of the patients, 46.1% were classified as overweight (32.8%) or obese (13.3%), while 4.8% were underweight. The highest prevalence of overweight (47.1%) was observed among patients who had undergone palliative surgery, whereas untreated patients showed the highest proportion of normal weight (57.2%). Cyanotic patients were predominantly of normal weight. Patients with univentricular circulation exhibited significantly lower rates of overweight and obesity (35%; p = 0.001). Overweight and obesity were statistically significantly associated with arterial hypertension, diabetes mellitus, and sleep apnea (all p < 0.001). High BMI was linked to increased use of HF-specific medications, including SGLT2 inhibitors (p = 0.040), diuretics (p = 0.014), and angiotensin receptor blockers (p = 0.005). Conclusions: The data highlight the clinical relevance of overweight and obesity in ACHD with HF, emphasizing the need for individualized prevention and treatment strategies. The registry serves as a critical foundation for the optimization of long-term care in this population.

Die pulmonale Hypertonie (PH) stellt einen hämodynamisch definierten pathologischen Zustand dar, welcher durch einen mittleren pulmonalarteriellen Druck (mPAP) > 20 mmHg charakterisiert ist. Die differenzialdiagnostische Zuordnung erfolgt anhand des pulmonalarteriellen Wedge-Drucks (PAWP) und des pulmonalen Gefäßwiderstands (PVR): präkapilläre PH: PAWP ≤ 15 mmHg bei gleichzeitig erhöhtem PVR > 2 Wood Units (WU), isolierte postkapilläre PH: PAWP > 15 mmHg, PVR ≤ 2 WU und kombinierte post- und präkapilläre PH: PAWP > 15 mmHg und PVR > 2 WU. Die belastungsinduzierte PH ist definiert als normwertiger mPAP in Ruhe, jedoch pathologischer Drucksteigerung unter körperlicher Belastung, definiert durch eine Steigung des mPAP relativ zum Herzzeitvolumen (HZV) von > 3 mmHg/L/min. Die etablierte klinische Klassifikation der PH bleibt in ihrer Grundstruktur mit 5 Hauptgruppen erhalten. Modifikationen beinhalten: Re-Etablierung der Subgruppe der Langzeitresponder auf Kalziumkanalblocker innerhalb der idiopathischen pulmonalarteriellen Hypertonie (IPAH), Erweiterung der Gruppe 2 durch zusätzliche Subkategorien bei PH infolge Linksherzerkrankungen und verfeinerte Untergliederung der Gruppe 3 auf Basis spezifischer pulmonaler Grunderkrankungen statt rein funktioneller Einschränkungen. Die Wirkstoffe Mitomycin-C und Carfilzomib wurden neu in die Liste der Pharmaka mit gesicherter Assoziation zur Entwicklung einer PAH aufgenommen.

Congenital heart defects (CHD) are the most common inborn cardiac anomalies, with approximately 1.35 million children born each year worldwide. Advances in medical treatment over recent decades have reduced mortality, yet morbidity remains high. Many patients now survive into adulthood but continue to have chronic heart disease and often develop complications such as heart failure, arrhythmias, pulmonary hypertension, and acquired cardiac and non-cardiac comorbidities, all of which require ongoing specialized care. Additionally, many adults with CHD (ACHD) lead a sedentary lifestyle, are overweight, and experience mental health issues, further affecting their well-being and quality of life. In this context, preventive, prehabilitative, and rehabilitative measures play an important role in reducing cardiovascular risks and enhancing overall quality of life. Preventive strategies aim to improve physical fitness, address health risks early, and support long-term well-being. Prehabilitation involves a structured, multimodal approach designed to strengthen physical and psychological resilience before planned medical interventions, thereby reducing complications and recovery times. Rehabilitation, on the other hand, facilitates recovery after treatment and promotes sustained health improvements over time. The German Pension Insurance provides programs aimed at enhancing physical fitness, promoting mental well-being, and improving quality of life, with a focus on maintaining employability and supporting occupational reintegration. However, available offerings are often not tailored to the specific needs of the heterogenous group of ACHD, limiting their potential effectiveness. The present article highlights the importance of cardiological prevention and prehabilitation in ACHD, focusing on the role of the German Pension Insurance system in helping affected adults remain employed and improve their quality of life. It explores how services can be better adapted to their needs and suggests that tailored programs, interdisciplinary collaboration, and ongoing research are essential for improving long-term outcomes in ACHD.

Background Aortic coarctation (CoA) is a congenital anomaly leading to upper-body hypertension and lower-body hypotension. Despite surgical or interventional treatment, arterial hypertension may develop and contribute to morbidity and mortality. Conventional blood pressure (BP) measurement methods lack precision for individual diagnoses and therapeutic decisions. This study evaluated the use of artificial intelligence-based pulse wave analysis (AI-PWA) to assess central aortic blood pressure (CABP) and related parameters in post-treatment CoA patients.Methods This exploratory, cross-sectional study enrolled 47 adults with CoA, between June 2023 and May 2024. Peripheral BP (PBP) was conventionally measured, and CABP was assessed using the VascAssist2 (inmediQ, Butzbach, Germany). Hypertension was defined by systolic BP≥140 mm Hg and/or diastolic BP≥90 mm Hg for PBP. Using AI-PWA, patients with systolic CABP≥130 mm Hg and/or diastolic BP≥90 mm Hg were classified as hypertensive.Results The study cohort’s age was 41.5±13.7 years, with all patients having undergone previous aortic surgery or intervention. PBP measurements showed a systolic BP of 135.4±14.4 mm Hg at the upper and 147.8±20.3 mm Hg at the lower extremities. CABP measurements were significantly lower, with a systolic BP of 114.3±15.8 mm Hg (p<0.001). Overall, 32 patients (68.1%) were diagnosed as hypertensive, either by PBP measurement (n=13/27.7%), because of antihypertensive treatment (n=9; 40.4%), or a combination of both. The measurement of PBP was more likely to indicate arterial hypertension than the measurement of CABP (n=12; 25.5% vs n=4; 8.5%). Pulse wave velocity, indicative of aortic stiffness, averaged 9.1 m/s, with higher values in 13 patients (27.7%), including 4 after end–end anastomosis, 2 after graft interposition and 7 after stent placement/angioplasty as the most recent procedure. An increased augmentation index as an indicator of arterial stiffness was observed in nine patients (19.1%). Comparing PBP and CABP in the entire collective, significant differences were found for CABP in relation to the procedure performed, with higher values in patients after prosthesis interposition as their last treatment (p<0.05).Conclusion AI-PWA provides valuable insights into cardiovascular stress in CoA patients, beyond PBP measurements. The study highlights the need to incorporate CABP measurements into clinical practice to avoid overdiagnosis of hypertension. Further research with larger cohorts is needed to validate these findings and refine management strategies for CoA patients.

Background/Objectives: Heart failure (HF) poses a major challenge in managing adults with congenital heart defects (ACHD). Emerging evidence suggests that HF in ACHD increases the risk of underweight due to heightened metabolic demands, gastrointestinal complications, and psychological factors such as anxiety and depression. Despite its critical implications, few studies have examined this association. This study evaluates the relationship between HF and underweight-defined as a body mass index (BMI) < 18.5-in ACHD. Methods: The Pathfinder-CHD Registry is a prospective, observational, web-based HF registry including ACHD with manifest HF, history of HF, or significant risk for HF. It documents congenital diagnoses, HF type, comorbidities, and treatments. Patients were categorized by BMI into mild (17.00-18.49), moderate (16.00-16.99), and severe (<16.00) underweight. Results: As of September 2024, the registry enrolled 1420 adults (mean age 31.8±11.3 years; 49.2% female). Underweight was present in 59 patients (4.2%): 62.7% mild, 18.6% moderate, and 18.6% severe. Among the remaining 1361 patients, 52.8% had normal weight, 32.8% were overweight, and 14.2% were obese. Women had significantly lower metabolic body weight than men (p = 0.002). Underweight correlated with younger age (p < 0.001) and CHD type (p = 0.02). Notably, 42.9% of underweight patients had cyanotic CHD. Conclusions: Underweight is an underrecognized problem in ACHD with HF. Adults with complex CHD or connective tissue disorders are disproportionately affected. Underweight should be seen as an alarm sign requiring personalized, multidisciplinary management, including nutritional support, tailored therapy, and close monitoring to improve outcomes.

Pulmonale Hypertonie (PH) kann in jeder Lebensphase, vom Neugeborenen- bis in das Erwachsenenalter auftreten, wobei bei Kindern teils sehr spezifische, altersabhängige Besonderheiten berücksichtigt werden müssen. Pulmonale Hypertonie im Kindesalter ist kein umschriebenes Krankheitsbild, sondern beschreibt einen hämodynamischen Zustand, dem unterschiedliche Ursachen zugrunde liegen können, die eine altersgemäße, vollständige, leitliniengerechte, diagnostische Abklärung erfordern, um eine korrekte Phänotypisierung, Charakterisierung und Klassifikation sowie zielgerichtete Behandlung der zugrunde liegenden Erkrankung zu ermöglichen. In ihrem aktuellen Bericht vom Welt-Symposium zur Pulmonalen Hypertonie (WSPH) 2024 fasst die pädiatrische Arbeitsgruppe neue Daten und Entwicklungen zusammen, die zu aktualisierten Konsensempfehlungen hinsichtlich Diagnose und Behandlung der PH im Kindesalter geführt haben. Dazu gehören eine alterskorrelierte Charakterisierung und Definition der pädiatrischen PH, eine erweiterte Risikoabschätzung bei Kindern sowie ein neuer Behandlungsalgorithmus, der erstmals auch kardiopulmonale Komorbiditäten berücksichtigt. Das vorliegende Manuskript bietet ein Update über die wichtigsten Neuerungen vom WSPH, ergänzt durch Expertenkommentare unter Bezug auf rezente Literatur.

Die Zahl Erwachsener mit angeborenen Herzfehlern (EMAH, AHF) steigt stetig an und liegt in Deutschland bei mehr als 360 000, weltweit bei 50 Millionen. Bei vielen AHF tritt die pulmonale Hypertonie (PH) in Form einer pulmonalarteriellen Hypertonie (P[A]H-AHF) auf. Sie kann frühzeitig auftreten oder sich im Langzeitverlauf auch Jahrzehnte nach einer chirurgischen oder interventionellen Behandlung entwickeln und ist mit erheblicher Morbidität und Letalität behaftet. Aufgrund der anatomischen und hämodynamischen Komplexität bedarf eine P(A)H gerade bei EMAH einer differenzierten Betrachtung, Diagnostik und Therapieabwägungen als andere PH-Formen. Die bestehenden Leitlinien der Europäischen Gesellschaft für Kardiologie (ESC) und (ERS) zur PH bieten allgemein gehaltene Diagnostik- und Behandlungsansätze, die bei Weitem nicht alle für EMAH relevanten Aspekte abdecken. Darüber hinausgehend liefert das 7. Weltsymposium zur Pulmonalen Hypertonie (7. WSPH, Barcelona 2024) einen wesentlichen Beitrag zur optimierten Versorgung der Betroffenen, kommentiert die P(A)H-AHF im Kindes- und Jugendalter, aber die P(A)H-AHF bei EMAH wird weitestgehend ignoriert. Im vorliegenden Artikel werden spezifische EMAH-Aspekte aus allen Themenbereichen des 7. WSPH, im Kontext der bestehenden Leitlinien, aber auch hinsichtlich Empfehlungslücken und Zukunftsperspektiven, aus Sicht der kongenitalen Kardiologie kommentiert.

The number of adults with congenital heart defects (ACHD, CHD) is constantly rising, with more than 360,000 affected individuals in Germany and 50 million worldwide. Many patients with congenital heart defects (CHD) develop pulmonary hypertension (PH), often as pulmonary arterial hypertension (PAH) associated with congenital heart defects (P[A]H-CHD). P(A)H-CHD may occur early in life or develop long-term, even decades after repair, which is associated with significant morbidity and mortality. Due to the anatomical and hemodynamic complexity of CHD, PH in this population, especially among ACHD, often requires a more differentiated nuanced approach to diagnosis and treatment compared to most forms of PH. The European society of Cardiology (ESC) guidelines provide diagnostic and treatment approaches to P(A)H-CHD, but do not cover all aspects. The current contributions from the 7th World Symposium on Pulmonary Hypertension (7th WSPH, Barcelona 2024) provides an update of the diagnosis and an overview of the optimization of PH treatment to improve care of affected individuals, but primarily focuses on children and adolescents with CHD, while recommendations for adults with P(A)H-CHD are scarce. This article comments on specific aspects covering all contents of the 7th WSPH, in the context of existing guidelines, as well as regarding gaps in recommendations and future perspectives from the standpoint of congenital cardiology for ACHD.

Background: Advances in diagnosis and treatment have led to a growing population of adults with congenital heart disease (ACHD). Despite increasing life expectancy, their clinical needs-especially in older age-remain poorly defined. Cardiac and non-cardiac comorbidities are prevalent, and emerging evidence suggests accelerated biological aging compared to the general population. However, data on older patients and geriatric patients with CHD are limited. Objectives: This study aimed to characterize patients with CHD aged ≥50 years, focusing on functional status, comorbidities, sex-specific differences, and therapeutic patterns. Methods: The PATHFINDER-CHD Registry is a prospective, observational, multicenter registry enrolling patients with CHD with manifest heart failure (HF), HF history, or high HF risk. Data include anatomy, prior treatments, comorbidities, and medication use. Results: Among 1935 patients, 297 were ≥50 years old. Most had acyanotic CHD (62%); Tetralogy of Fallot (21%) was the most frequent diagnosis. A morphologic right systemic ventricle was present in 12%, and 5% had univentricular hearts. HF was manifest in 21%; 44% were classified as ACC/AHA stage B, 51% as stage C, yet 77% were in Perloff class I/II. Common cardiovascular comorbidities included aortopathy (55%), hypertension (37%), and arrhythmia (33%). Non-cardiac comorbidities included thyroid dysfunction (25%), renal impairment (18%), and neurological disease (13%). Sex-specific differences were observed. Despite HF burden, SGLT2 inhibitors and ARNIs were used in only 17% and 8.4%, respectively. Conclusions: Older patients with CHD represent a clinically complex cohort with high comorbidity burden. The findings support the concept of accelerated aging and emphasize the need for tailored interdisciplinary care strategies.

Pulmonary hypertension (PH) can occur at any stage of life, from neonatal to adulthood, although very specific, age-dependent characteristics must be taken into account in children. Pulmonary hypertension in children is not a circumscribed clinical picture, but describes a haemodynamic condition that can be caused by different factors, which require an age-appropriate, complete, guideline-based diagnostic clarification in order to enable correct phenotyping, characterization and classification, as well as targeted treatment of the underlying disease. In their recent report from the World Symposium on Pulmonary Hypertension (WSPH) 2024, the pediatric task force summarized new data and developments that have led to updated consensus recommendations regarding the diagnosis and treatment of PH in childhood. These include an age-correlated characterization and definition of pediatric PH, expanded risk assessment in children, and a new treatment algorithm that includes cardiopulmonary comorbidities for the first time. This manuscript provides an update on the most important innovations of the WSPH, supplemented by expert commentary with reference to recent literature.

Congenital heart defects (CHD) represent the most common inborn organ anomaly, with more than a million newborns affected annually. Advances in diagnostics and treatment have led to significantly improved survival rates, resulting in a growing population of an estimated 50 million adults with congenital heart defects (ACHD) worldwide. As these individuals age, they often face a high burden of morbidity and complex long-term health challenges that require specialized, lifelong care. In this context, cardiological rehabilitation (CR) becomes increasingly important, not only to reduce morbidity but also to enhance patients' quality of life and support their social and occupational integration. While CR has been extensively studied and implemented for acquired heart diseases, structured rehabilitation programs tailored to the specific needs of ACHD remain limited in clinical practice and in the scientific literature. Globally, both the availability of CR and the presence of structured concepts vary widely. CR is predominantly offered in high-income countries, with Western Europe providing the most extensive services. In many low- and middle-income countries, access to CR remains limited or is sometimes not available at all. However, even in high-income settings, targeted ACHD programs are scarce, meaning that many ACHD are treated in general CR programs that do not adequately address the complexity of CHD. The present article outlines the core components of CR, provides recommendations on how these are implemented in current practice, identifies existing limitations, and discusses how services could be better aligned with the complex medical and psychosocial needs of ACHD. It also describes the role of the German Pension Insurance in funding and providing rehabilitation services in Germany. Tailored rehabilitation programs, greater integration of ACHD expertise, and targeted research are essential to improve long-term outcomes and establish patient-centered care structures for the growing ACHD population. In this way, the present paper is intended to support the development of rehabilitation programs for countries where such structures currently do not exist.

Im August 2023 wurden die neuen europäischen Leitlinien zum Management der infektiösen Endokarditis durch die Europäische Gesellschaft für Kardiologie (ESC) publiziert. Trotz der Schwere der Erkrankung mit häufig ungünstiger Prognose sowie ansteigender Inzidenz bleibt die infektiöse Endokarditis ein vernachlässigtes Gebiet der kardiovaskulären und infektiologischen Forschung. Obwohl große Evidenzlücken existieren, beinhalten die ESC-Leitlinien zahlreiche neue und aktualisierte Empfehlungen, mit dem Ziel, das Management von Patienten mit infektiöser Endokarditis zu verbessern. Wesentliche Änderungen der aktuellen ESC-Leitlinien finden sich v. a. in den Bereichen Prävention inklusive Antibiotikaprophylaxe, Rolle eines Endokarditis-Teams, diagnostische Kriterien, Paradigmenwechsel der antibiotischen Therapie hin zur Oralisierung, Timing und Indikation der chirurgischen Therapie sowie Infektionen von kardialen implantierbaren elektronischen Devices.

The number of adults with congenital heart defects (ACHDs) is steadily increasing and is about 360,000 in Germany. Congenital heart defect (CHD) is often associated with pulmonary hypertension (PH), which sometimes develops early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists, redevelops in older age, and is associated with significant morbidity and mortality. The revised European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart defects" is treated only relatively superficially in this context. After the first part commenting on a broad range of topics like definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, and gender aspects, the second part focuses on supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management [shunt lesion, left ventricular (LV) disease, univentricular hearts], interventions, intensive care, ACHD follow-up, and future perspective. In the present article, therefore, this topic is commented on from the perspective of congenital cardiology. By examining these aspects in detail, this article aims to fill the gaps in the existing guidelines and provide a more thorough understanding from the perspective of congenital cardiology.