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Objective: It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients. Methods: Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life. Results: Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life. Conclusions: The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.

Aortic aneurysm and aortic dissection can have a major impact on the life expectancy of Marfan syndrome (MFS) or Loeys-Dietz syndrome (LDS) patients. Although obesity can influence the development of aortic complications, evidence on whether obesity influences the development of aortic aneurysm or dissection in MFS and LDS is limited. The aim of the present study was to elucidate the relationship between aortic size and body composition, assessed by modern bioelectrical impedance analysis (BIA) in MFS/LDS-patients.In this exploratory cross-sectional study in MFS or LDS patients, enrolled between June 2020 and May 2022, 34 patients received modern BIA and magnetic resonance imaging (MRI) (n=32) or computed tomography (CT) imaging (n=2) of the entire aorta. A P value of0.05 was considered significant.Fifty-one patients (66% female; mean age: 37.7±11.7; range, 17-68 years) with MFS or LDS were enrolled; 34 patients, 27 with MFS and 7 with LDS, underwent aortic MRI or CT scanning. The mean aortic length was 503.7±58.7 mm, and the mean thoracic aortic length and abdominal aortic length were 351.5±52.4 and 152.2±27.4 mm, respectively. The aortic bulb and the ascending aorta were measured only in the non-surgically repaired patients. Fifteen MFS (88.2%) and two LDS (40.0%) patients had an aortic aneurysm. In these, the aortic bulb tended to be larger in MFS than in LDS patients [42.6×41.9×41.2While a slender habitus is commonly known for MFS and LDS patients, our data show that many MFS and LDS patients (especially female) do not fit this phenotypic characteristic and are obese, which is associated with a more severe aortic phenotype. This topic should be included in the clinical assessment of affected MFS and LDS patients, in addition to measurement of the aortic diameters. Physicians should systematically screen MFS and LDS patients for obesity, educate them about the potential risk of resulting aortic complications, and encourage them to adopt a healthy lifestyle, that includes (mild) exercise and a balanced diet.

Background: Impairments and developmental delay are often reported in infants and young children with CHD. However, currently, there is no data regarding cognitive abilities assessed by standardised intelligence tests in adults with CHD. This study assesses the cognitive function in Fontan patients compared with acyanotic CHD patients whether restrictions in cognitive function are present in adulthood and its association with health-related quality of life. Methods: Forty-four adult CHD (female n = 21 (47.7%); mean age 34.7 ± 11.9 years), 22 with Fontan circulation and 22 with acyanotic CHD, underwent the Wechsler Intelligence Scale for adults as patients during routine follow-up in 2018. The Medical Outcomes Study Questionnaire Short-Form 36 Health Survey (SF-36) assessed health-related quality of life. Results: Fontan patients showed significantly better results in the FSIQ (p = 0.020) and perceptual reasoning (p = 0.017) in comparison with patients with acyanotic CHD. All adult CHD patients showed normal IQ in subscales and full-scale IQ (FSIQ). In health-related quality of life, no association with cognitive function was found and no significant difference between both CHD groups, but trends to reduced values in acyanotic adult CHD. Conclusions: Interestingly, our study results in adult Fontan patients showed that it is possible to live an adult life with normal cognitive function and good health-related quality of life with a univentricular heart. Thus, this study could be a guidepost for more in-depth studies on cognitive function in Fontan survivors. In addition, the focus should be on health-related quality of life of adult CHD with simple CHD in particular, since a reduced health-related quality of life is not only medically based.

Improved treatment options for congenital heart disease (CHD) lead to a growing number of women with CHD at reproductive age. Due to physical and psychological burden, pregnancies in women with CHD often count for high-risk. Resulting emotional distress can adversely impact pregnancy, motherhood and fetal health. The present study aims to retrospectively investigate mental outcomes and indices of adjustment in women with CHD before, during and after pregnancy. The novel concept of illness identity is applied to explain how patients experience and integrate their CHD into their identities.Patient-reported outcome measures on mental functioning and illness identity were assessed in a sample of 121 postpartum women with CHD [mean age: 42.7±9.2 (range, 27-81) years] at the German Heart Centre Munich between August and November 2021 in a cross-sectional design. Descriptive analyses, correlations and linear regression models were calculated.Retrospectively assessed prevalence of emotional distress before giving birth was high (47.0%) and peaked shortly after childbirth in terms of elevated symptoms of postpartum depression and trauma. During the course of maternity, emotional distress decreased significantly (24.1%, P0.001). Overall, postpartum women demonstrated high scores in functional illness identity states (i.e., acceptance and enrichment) and low scores in dysfunctional states (i.e., rejection and engulfment). CHD severity was not directly associated with mental outcomes (P0.05), whereas maternal cardiovascular risk, according to the WHO classification, was significantly associated with a higher prevalence of postpartum trauma (t=2.485, P=0.015).Postpartum mental health problems, such as (postpartum) depression, anxiety, and posttraumatic stress can become a serious burden which might be detrimental to the mother's well-being and her infant's development. Present findings emphasise the urgent need for a holistic approach focusing on pregnant women with CHD starting at the prepartum stage to prevent adverse consequences and promote maternal well-being. Illness identity might become an important target construct for clinical practice as it may positively and enduringly influence mental well-being of pregnant women with CHD.

Fabry disease (FD) is a multi-organ disorder associated with severe physical and psychological impairments, particularly in adulthood. To date, comprehensive data on the psychological burden of FD are lacking. The present study assessed quality of life (QOL) in a representative cohort of adults with FD.Patient-reported outcome measures were retrospectively analyzed in 86 adults with FD (49.6±16.6 years; 62.8% female) and compared to adults with congenital heart defects (ACHD) which is another lifelong disease and affliction. QOL was assessed using the European Quality of Life 5 Dimensions 5 Levels questionnaire (EQ-5D-5L).Subjects affected by FD reported an overall reduced QOL (EQ-VAS: 71.8±20.0). Most frequently reported complaints occurred within the dimensions pain/discomfort (69.7%), daily activities (48.9%) and anxiety/depression (45.4%). Compared to ACHD, individuals with FD scored significantly lower in the areas of pain/discomfort, usual activities and mobility (all P0.05). Older age and female sex were particularly associated with diminished QOL (P=0.05).Patients with FD are at high risk for impaired QOL. They require additional support to cope with disease-related challenges. Increased attention should be directed towards improving their subjective well-being to potentially increase their QOL and long-term health outcomes.

BACKGROUND: Cardiovascular magnetic resonance feature-tracking analysis (CMR-FT) provides a quantitative assessment of myocardial contraction with potential for diagnostic and prognostic ability in a wide spectrum of diseases. Ebstein’s anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve. However, it is also considered a disorder of development affecting the global right ventricular myocardium. Aim of our study is to describe the complex contractile mechanics of the functional right ventricle (RV) in patients affected by EA through CMR-FT. METHODS: Fifty surgery-free EA patients who had undergone a complete CMR protocol at our institution between January 2017 and December 2020 were selected for the retrospective study. A historical control group of twenty-five healthy subjects was also included. CMR-FT analysis was performed at a dedicated workstation by manually tracing RV endo- end epicardial borders on steady-state-free-precession (SSFP) cine images. Strain values were calculated. Apical displacement of the tricuspid valve (TV) was measured on a 4-chamber cine image from the right atrio-ventricular junction to the functional annulus of the TV. RESULTS: EA patients presented significantly impaired RV global radial strain (GRS) and global circumferential strain (GCS) compared to controls (P16 mm/m(2)) compared to milder forms (P=0.03) and to controls (P

BACKGROUND: Patients with congenital heart defects (CHD) are prone to residua, sequels and complications from the underlying anomaly, where cardiac arrhythmias are one of the major causes for hospitalization, morbidity and mortality. The importance of the subcutaneous implantable loop recorder (ILR) for the detection and documentation of significant arrhythmias has increased over the last years. To date, however, there is little data on ILR use in the CHD population. METHODS: In this single center, retrospective observational study, all CHD-patients with an ILR were identified who were under care of the German Heart Center Munich between February 2015 and January 2019. The primary endpoint of the study was the detection or exclusion of significant arrhythmias during follow-up in CHD-patients who had received an ILR. The secondary endpoint was to determine whether ILR findings influenced patient management, defined as initiation or adjustment of medication, cardioversion, electrophysiologic study, catheter ablation, or implantation of cardiac implantable electronic devices (CIEDs) such as pacemakers (PM) or implantable cardioverter-defibrillators. RESULTS: An ILR was implanted in 33 CHD-patients (mean age, 43±20 years; 42.4% female) with CHD. During a mean observation period of 697±433 days, clinically relevant arrhythmias, correlating with the patients’ complaints and symptoms, were detected in 19 patients (59.4%), encompassing supraventricular tachycardia (n=10), supraventricular or ventricular ectopic beats (n=10), non-sustained ventricular tachycardia (n=2), ventricular tachycardia (n=2), and bradycardia (n=2). In 9 patients (28.1%) the detected arrhythmia was considered an event requiring treatment. Treatment modalities included catheter ablation (n=5), modification of antiarrhythmic drug regime (n=2), adaptation of anticoagulation therapy (n=2), or implantation of a subcutaneous ICD (n=1). Regarding the occurrence of cardiac arrhythmias or a related need for therapeutic intervention, no significant differences were identified with respect to WHO functional class, the presence of pulmonary arterial hypertension or reduced resting peripheral oxygen saturation. CONCLUSIONS: In symptomatic CHD-patients at risk for life-threatening cardiac events, ILR has a considerable complementary diagnostic value for the detection and differentiation of benign and malignant arrhythmias. Considering the overall low risk of complications, ILR implantation should be considered in patients with CHD of any complexity who need medium or long-term arrhythmia monitoring, especially if short-term Holter monitoring cannot provide sufficient diagnostic certainty.

BACKGROUND Adolescents and adults with native Ebstein's anomaly (EA) are at the benign part of the Ebstein spectrum, having survived infancy without surgery. In this population, surgical indication and timing remain objects of controversy and depend, among other factors, on exercise capacity. PURPOSE To better understand the pathophysiology of exercise adaptation in native EA. STUDY TYPE Retrospective. POPULATION Ten patients with unoperated EA (age range 18-61 years) and 13 healthy subjects as controls. FIELD STRENGTH/SEQUENCE Balanced steady-state free precession cine and phase contrast flow sequences at 1.5 T. ASSESSMENT We measured volumes and flows at rest and during submaximal exercise. Hemodynamic parameters including stroke volume (SV), cardiac index (CI), ejection fraction (EF), and tricuspid regurgitation (TR) were calculated. STATISTICAL TESTS We used nonparametric Mann-Whitney U-test and Wilcoxon signed-rank test. A P-value of

Currently, due to medical advances, more than 500 000 patients with congenital heart defects (CHD) are living in Germany, including more than 330 000 adults. In CHD, the aspect of transition from childhood and adolescence to adulthood is becoming increasingly important to ensure long-term success of the provided treatments. This is so important because despite primarily successful treatments, residua and sequels as well as (non-) cardiac comorbidities may influence the chronic course of the disease and lead to increased morbidity and mortality. Adults with congenital heart defects in Germany are insufficiently managed by existing specialized and accredited care structures, despite the great need for follow-up, and their care is largely provided by primary care physicians (general practitioners and internists) and non-specialized cardiologists.Optimized, future-oriented care requires regular follow-up and the possibility of interdisciplinary, integrated medical care of CHD in the long-term course.

Background All patients with congenital heart disease (CHD) are chronically ill from their cardiac disease. Despite the increasing evidence that aortic alterations are becoming relevant, the importance of aortopathy in CHD has long been underestimated. This study was conducted to determine the health status of patients and/or the provision of health services of adults with CHD (ACHD) with manifest aortopathy or at risk thereof. Methods In a questionnaire-based cross-sectional survey, the "real life"-care of ACHD was analysed, comparing patients with risk of developing aortopathy and/or manifest aortopathy. Results Of the 563 enrolled ACHD (49.6% female, mean age 35.8±12.1, 18-86 years) 56.8% (n=320) had a risk of developing aortopathy and/or manifest aortopathy. Of the 320 patients at risk, 187 (33.2% of the total number) had a proven aortopathy. Within this subgroup, the basic medical care for CHD-independent medical problems was given by primary medical care providers [family doctors/general practitioners (GP) in 89.4% (n=286), internists in 13.4% (n=43), physicians of another specialty in 2.5% (n=8)]. Almost all primary medical care providers knew about the CHD of their patients. Even for CHD-specific health problems, the basic medical care of risk patients was provided by a family doctor or GP in 56.6% (n=181) and by an internist in 18.4% (n=59). 30.0% (n=96) primarily consulted another specialist, including cardiologists. Only 32.8% of ACHD at risk had ever been referred to a CHD specialist by a GP for cardiac problems related to their CHD. In contrast, the need for advice was high for ACHD with aortopathy and related mainly to physical activity, employment and education, pregnancy, rehabilitation or health and life insurance. Only 35.5% of patients at risk indicated that their information on specific care structures for ACHD was sufficient, and a further 38.1% of patients were aware of patient organizations. Conclusions Even today, aortic involvement in ACHD is an often-overlooked condition, although considerable negative effects on morbidity and mortality exist. As aortopathy gains in importance with increasing age and complexity of CHD, almost all affected ACHD need lifelong medical advice and access to modern, scientifically based care concepts. According to the study-results, primary care providers and also patients are mostly insufficiently informed about the specialized ACHD facilities. The future goal is therefore to create a better awareness of CHD problems among both primary care physicians and the patients concerned.

Background: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. Methods: During the study period from January to June 2021, all consecutive outpatients from our ACHD clinic were eligible to participate. Clinical data were collected. An antibody test for COVID-19 was performed in all patients. Cardiovascular magnetic resonance imaging (CMR) was offered to those with a positive antibody test. Results: Overall, 420 patients (44.8% female, mean age 36.4 ± 11.6 years) participated. Congenital heart defect (CHD) complexity was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. Altogether, 28 (6.7%) patients had a positive antibody test. Out of these, 14 had an asymptomatic course. The others had mainly mild symptoms and were managed as outpatients. Furthermore, 11 patients (39.3%) had even not been aware of their infection. Fourteen patients underwent a CMR without signs of myocardial involvement in any of them. Conclusions: We observed a number of undetected cases of COVID-19 infections in our ACHD population. Reassuringly, in all cases, the infection had a mild clinical course.

Objectives We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair. Methods The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient). Results Thirty-nine patients underwent cone repair, 107 patients underwent other repair techniques, and 5 patients underwent valve replacement. The operative mortality was 1.3% (n = 2). Failed valve repair (defined as in-hospital death, conversion to replacement, or in-hospital reoperation) was less frequent after cone repair than after other repair techniques (5%, n = 2 vs 20%, n = 21, P = .039). Mean follow-up was 12.3 years (cone repair: 3.7 years). The 5-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation was lower after cone repair than after other repair techniques (8% vs 32%, P = .03). Among the patients undergoing other repair techniques, the 15-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation, severe tricuspid regurgitation, and reoperation was 58%, 37%, and 31%, respectively. During follow-up, 18 patients died (13 of cardiac and 5 of noncardiac causes). Among patients who died of cardiac causes, 10 of 13 had all 3 characteristics—moderate or greater tricuspid regurgitation, atrial fibrillation, and New York Heart Association classification III and IV—at their last medical evaluation. Conclusions Before cone repair, recurrent tricuspid regurgitation was considerable. Cone repair provided a higher rate of successful repair and a lower incidence of moderate or greater recurrent tricuspid regurgitation at the midterm follow-up.

Pulmonary vascular disease and pulmonary hypertension (PH) belong to the most relevant complications of congenital heart disease (CHD) in the long-term course. Although PH might lead to a severely impaired quality of life (QOL), there are no current studies comparing QOL in adults with CHD (ACHD) with and without PH. Therefore, this study aimed to systematically examine QOL in ACHD with and without PH in order to generate a more differentiated understanding of their overall health-status and to employ newly gained findings into general care of this particular patient population.In this comparative cross-sectional study, a representative sample of 803 adults with various forms of CHD with or without PH was analyzed. Data collection took place between September 2017 until February 2018 in a tertiary care center for ACHD. Medical data was retrieved from medical records. QOL was assessed using the EQ-5D-5L questionnaire. Descriptive methods, chi-square and t-tests were used to compare QOL of patients with and without PH.Of 803 patients, 752 patients had no PH [93.6%; 47.3% female; mean age: 34.9±11.83 (range, 18-86) years], 51 were identified with manifest PH [6.4%; 55.8% female; mean age: 41.9±12.17 (range, 21-69) years]. PH patients showed significantly worse overall QOL [mean no PH: 86.78±13.30 (8.2-100)Since QOL is a significant predictor of outcome, PH-CHD patients need an early provision of psychosocial, health promoting support in addition to dedicated care and targeted PH treatment. It is therefore pivotal to timely identify unique psychosocial impairments in order to enhance quantity and QOL in this particularly vulnerable patient population.

In the long-term course of treated and untreated congenital heart defects (CHD), pulmonary hypertension (PH) is one of the most relevant complications. Since PH carries a high risk for mortality and morbidity, it is important to improve the status of healthcare and medical knowledge regarding the affected patients. Therefore, this study aimed to determine the current medical care status, health-related knowledge, and specific counseling needs of adults with various forms of CHD (ACHD) who are at increased risk of developing PH, as well as those with manifest PH.In this retrospective cross-sectional study, a representative sample of 803 ACHD were analyzed. Patients were split into three groups based on CHD: low risk for PH, at risk for pre- or post-capillary PH, and manifest PH. Data collection took place between September 2017 until February 2018 in a tertiary care center for ACHD. Healthcare status and specific needs for information or advice were analyzed using a questionnaire designed by our group. The state of knowledge of the patients was assessed by comparing this questionnaire and the corresponding medical records.Both patients with manifest PH (n=51) and patients at risk to develop PH (n=629) were insufficiently informed about their health status, specific care structures available to them, and patient organizations. About 50% of the patients had specific counseling needs, especially regarding physical capability and sports, daily stress, rehabilitation measures, and pregnancy. Only 47.8% of patients with manifest PH were aware of suffering from PH (P0.001). In particular, the patients had large knowledge deficits regarding comorbidities related to their health condition.PH is a quantitatively and qualitatively underestimated residuum or sequela of CHD that significantly affects outcome and prognosis in ACHD. Multidisciplinary, structured, and specific counseling of affected individuals with corresponding risk constellations is urgently needed. A prerequisite for this is closer collaboration between primary care physicians (PCPs), such as general practitioners, family physicians, internists, or general cardiologists, and ACHD specialists. Targeted patient counseling and care could have a positive impact on the level of awareness of those affected and favorably influence their prognosis.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P =0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P =0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Covered stents perform similar to surgically implanted conduits, although the stents work inside of vessels. We present a computed tomography (CT)-based workflow for the implantation of covered stents as extravascular conduits.We selected three different use cases: 1. Connecting a left-sided partially anomalous drainage of a pulmonary vein to the left atrium. 2. Bypassing an outgrown Dacron conduit in aortic recoarctation. 3. Re-directing hepatic venous blood to the left lung in a Fontan patient with heterotaxy, connecting the innominate vein to the right pulmonary artery like a right-sided cavopulmonary connection. By postprocessing and analyzing CT scans for planning and by the use of long needles under biplane fluoroscopy for the realization of the procedure, we projected and performed the exit of a long needle out of a vessel, the re-entering of a target vessel, and the bridging of the extravascular distance by implantation of covered stents.In all three cases, the covered stents were placed successfully, connecting vessels of 15-50 mm distance from each other with very good hemodynamic results. In one case, two stents were placed consecutively, overlapping each other to accomplish an exact fitting at the connection sites to the native vessels.

The number of adults with congenital heart disease (ACHD) is steadily increasing. The present cross-sectional study was conceived to investigate ACHD care from the perspective of patients and family practitioners (specialists for general medicine and internal medicine, general practitioners).Questionnaire-based cross-sectional study to analyze the real care situation of ACHD in Germany from the perspective of patients and primary care physicians (PCP).The questionnaire was completed by 4493 ACHD (53.7% female; 41.3 ± 16.9 years) and 1055 PCP. The majority of ACHD (79.8%) visited their PCP for noncardiac health problems but also for cardiac problems. Almost all ACHD had substantial needs for medical consultation (performance, employment etc.). Of the patients 2014 (44.8%) did not know of any certified ACHD specialists or specialized centers and 2816 (62.7%) respondents were not aware of any ACHD patient organization. Of the PCPs 87.5% had cared for ACHD of all severities due to defect-typical residual and resulting symptoms. Many were not aware of any certified ACHD specialists. Only 28.5% consulted an ACHD specialist. Only 23.5% were aware of ACHD patient organizations.General practitioners are a mainstay of ACHD care in Germany. The present study shows that ACHD and their general practitioners are largely uninformed about the specialized care structures available nationwide, despite the high level of need for specialist care. In order to keep the morbidity and mortality of affected patients low, solutions must be elaborated in future to involve and integrate primary care physicians more intensively into the already existing dedicated ACHD care structures, in cooperation with specialized pediatric cardiologists, cardiologists and centers.HINTERGRUND: Die Zahl der Erwachsenen mit angeborenen Herzfehlern (EMAH) steigt stetig. Die vorliegende Querschnittsstudie wurde konzipiert, um die EMAH-Versorgung aus Sicht der Patienten und der Hausärzte (Fachärzte für Allgemeinmedizin und für Innere Medizin, praktische Ärzte) zu untersuchen.Fragebogenbasierte Querschnittsstudie zur Analyse der realen Versorgungssituation von EMAH in Deutschland aus Sicht der Patienten sowie der Hausärzte.Der Fragebogen wurde von 4493 EMAH (53,7 % weiblich; 41,3 ± 16,9 Jahre) und 1055 Hausärzten ausgefüllt. Die Mehrheit der EMAH (79,8 %) besuchten ihren Hausarzt wegen nichtkardialer Gesundheitsprobleme, aber auch wegen kardialer Probleme. Fast alle EMAH hatten erheblichen medizinischen Beratungsbedarf (Leistungsfähigkeit, Berufsfindung etc.). 2014 Patienten (44,8 %) kannten keine zertifizierten EMAH-Spezialisten oder -Zentren. 2816 Befragte (62,7 %) waren nicht über EMAH-Patientenorganisationen informiert. 87,5 % der Hausärzte hatten EMAH aller Schweregrade wegen vitientypischer Rest- und Folgeerscheinungen betreut. Viele kannten keine zertifizierten EMAH-Spezialisten. Nur 28,5 % zogen einen EMAH-Spezialisten konsiliarisch hinzu. Nur 23,5 % kannten EMAH-Patientenorganisationen.Hausärzte sind eine tragende Säule der EMAH-Versorgung in Deutschland. Die vorliegende Studie zeigt, dass EMAH und ihre Hausärzte, trotz des großen Bedarfs an spezialisierter Betreuung, weitgehend uninformiert sind über die bundesweit vorhandenen EMAH-Versorgungsstrukturen. Um die Morbidität und Mortalität der Betroffenen niedrig zu halten, müssen Lösungen entwickelt werden, um in Kooperation mit den auf EMAH spezialisierten Kinderkardiologen, Kardiologen und Zentren die Hausärzte stärker in die vorhandenen EMAH-Versorgungsstrukturen einzubinden bzw. zu integrieren.

Background Direct oral anticoagulants (DOACs) have been proven to be safe and effective in patients with acquired heart disease. However, data regarding their use in adults with congenital heart disease (ACHD) is scarce. Methods All ACHD under DOAC were retrospectively identified. Bleeding and thromboembolic events were registered. CHADS2, CHA2DS2-VASc and HASBLED scores were calculated. Risk factors for bleeding and thromboembolic events were identified. Results 215 ACHD patients (111 female, 48.4 ± 15.5 years) were included. CHD was severe in 44.2%, moderate in 23.7%, and simple in 32.1%. Indications for anticoagulation were: atrial arrhythmias (66.8%), cerebrovascular accidents (32.7%), pulmonary thromboembolism (3.7%), deep vein thrombosis (11.2%), atrial thrombi (6.5%), and Fontan circulation/TCPC (5.6%). Mean follow-up was 15.8 ± 15.8 months. Nine patients suffered a major and eight a minor bleeding. Thromboembolic events occurred in two patients. The annual risk for bleeding was 3.1%/patient/year and for thromboembolic events 0.7%/patient/year. A CHADS2-Score >2, HASBLED >3, and renal disease were identified as risk factors for bleeding (minor and major bleeding) in a univariate analysis. In the multivariate analysis, renal disease remained as an independent predictor for bleeding (HR 6.13, CI [1.04–36.27], p Conclusions A low rate of thromboembolic events was observed in ACHD patients under DOAC therapy. Bleeding complications were not negligible. Special attention has to be paid to those patients with advanced renal failure.

Background: Adults with congenital heart disease and ventricular dysfunction are prone to liver congestion, leading to fibrosis or cirrhosis but little is known about the prevalence of liver disease in atrial switch patients. Liver impairment may develop due to increased systemic venous pressures. This prospective study aimed to assess non-invasively hepatic abnormalities in adults who underwent Senning or Mustard procedures. Methods: Hepatic involvement was assessed non-invasively clinically by laboratory analysis, hepatic fibrotic markers, sonography, and liver stiffness measurements [transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)]. Results: Overall, 24 adults who had undergone atrial switch operation (13 Senning, 11 Mustard; four female; median age 27.8 years; range 24–45 years) were enrolled. In liver stiffness measurements, only three patients had values within the normal reference. All other patients showed mild, moderate or severe liver fibrosis or cirrhosis, respectively. Using imaging and laboratory analysis, 71% of the subjects had signs of liver fibrosis (46%) or cirrhosis (25%). Conclusions: Non-invasive screening for liver congestion, fibrosis or cirrhosis could be meaningful in targeted screening for hepatic impairment in patients with TGA-ASO. As expert knowledge is essential, patients should be regularly controlled in highly specialised centres with cooperations between congenital cardiologists and hepatologists.

BACKGROUND: Aortic root ectasia might induce hemostatic disorders in patients with Marfan syndrome (MFS) via altered blood flow and rheology. The aim of this study was to explore the hemostasis in patients with MFS compared with healthy controls. METHODS: In this cross-sectional case-control study we included patients with verified MFS (n=51) and sex- and age-matched healthy controls (n=50). Main criteria were the aortic root in echocardiography and cardiac magnetic resonance imaging (MRI), and the coagulation status. RESULTS: When compared with healthy controls, patients with MFS showed significantly increased diameters of the aortic roots (43.0±7.72 vs. 28.8±3.74 mm, P

Angeborene Herzfehler (AHF) sind die haufigsten angeborenen Organanomalien. Dank medizinischer Fortschritte erreichen heutzutage fast alle Patienten das Erwachsenenalter. Trotz primar erfolgreicher Behandlungen konnen Rest- und Folgezustande sowie (nicht-)kardiale Komorbiditaten den chronischen Krankheitsverlauf beeinflussen und zu einer erhohten Morbiditat und Mortalitat fuhren. Erwachsene mit angeborenen Herzfehlern (EmaH) in Deutschland sind trotz des grosen Nachsorgebedarfs nicht an die Versorgungsstrukturen angebunden. Daten der VEmaH-Studie („Versorgungssituation von EmaH“) belegen, dass EmaH und primar betreuende Haus- und Allgemeinarzte hinsichtlich des Komplikationspotenzials von AHF und der Notwendigkeit einer fachspezifischen Nachsorge enorme Informationsdefizite aufweisen. Eine optimierte, zukunftsorientierte Versorgung erfordert eine regelmasige Nachsorge und die Moglichkeit zur facherubergreifenden, integrierten medizinischen Versorgung von AHF im Langzeitverlauf.

Currently, due to medical advances, more than 500 000 patients with congenital heart defects (CHD) are living in Germany, including more than 330 000 adults. In CHD, the aspect of transition from childhood and adolescence to adulthood is becoming increasingly important to ensure long-term success of the provided treatments. This is so important because despite primarily successful treatments, residua and sequels as well as (non-) cardiac comorbidities may influence the chronic course of the disease and lead to increased morbidity and mortality. Adults with congenital heart defects in Germany are insufficiently managed by existing specialized and accredited care structures, despite the great need for follow-up, and their care is largely provided by primary care physicians (general practitioners and internists) and non-specialized cardiologists.Optimized, future-oriented care requires regular follow-up and the possibility of interdisciplinary, integrated medical care of CHD in the long-term course.

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class at begin of follow-up (hazard ratio: 1.96, CI 95%: 1.04–3.72, p &lt, 0.05) was the only predictor for the primary endpoint. Conclusions: Significant morbidity and mortality is present in cAVSD patients over the age of 40 years. NYHA class is predictive for a worse outcome.

Background The number of adults with congenital heart disease (ACHD) is steadily rising due to increased survival rate and improved medical resources. Accordingly, more than 330,000 ACHD are currently living in Germany. Almost all of them require lifelong specialized medical follow-up for their chronic heart disease, often accompanied by residua, sequelae, or comorbidities. Primary care physicians (PCPs) are a crucial factor in directing patients to ACHD specialists or specialized institutions, but despite all efforts, the number of ACHD under specialized care is low, the lost-to-follow-up rate is high, and the morbidity and mortality is substantial. The present cross-sectional study was designed to systematically characterize the health care of ACHD from a patient perspective, including (I) use of medical care by ACHD, (II) medical counselling needs, and (III) perceived satisfaction with health care. Methods The German-wide analysis was based on a 25-item questionnaire designed to address different aspects of medical status and health issues of ACHD from their own perspective, performed between May of 2017 and July of 2020. Results A total of 4,008 ACHD (52% female; mean age 41.9±17.2 years) completed the questionnaire. The majority of ACHD (3,524, 87.9%) reported, that they consulted their PCP for non-cardiac health problems, and 49.7% (n=1,991) consulted their PCP also for medical problems associated with the underlying CHD. Almost all ACHD reported a need for medical advice concerning exercise capacity and daily life activities, occupational skills, pregnancy, rehabilitation, genetic counselling, insurance, and retirement. A total of 1,840 (45.9%) patients were not aware of the existence of certified ACHD specialists or centers. Moreover, 2,552 (67.6%) of those surveyed were uninformed about patient organizations for ACHD. Conclusions The present study demonstrates that ACHD are largely uninformed about the ACHD care structures available nationwide, although the patients have a great need for specialized follow-up, advice, and care.

Congenital heart diseases (CHD) can be associated with liver dysfunction. The cause for liver impairment can result out of a wide spectrum of different causes, including liver congestion, hypoxemia or low cardiac output. Fortunately, most CHD show a good long-term outcome from a cardiac perspective, but great attention should be paid on non-cardiac health problems that develop frequently in patients suffering from CHD. The treatment of liver dysfunction in CHD requires a close multidisciplinary management in a vulnerable patient collective. Unfortunately, structured recommendations on the management of liver dysfunction in patients with CHD are scarce. The objective of this review is to provide insights on the pathophysiology and etiologies of liver dysfunction as one of the most relevant non-cardiac problems related to CHD. Furthermore, we advise here on the management of liver disease in CHD with special attention on assessment of liver dysfunction, management of portal hypertension as well as on surveillance and management of hepatocellular carcinoma (HCC). A multidisciplinary perspective may help to optimize morbidity and mortality in the long-term course in these patients. However, as evidence is low in many aspects, we encourage the scientific community to perform prospective studies to gain more insights in the treatment of liver dysfunction in patients with CHD. CA extern

BACKGROUND: Marfan syndrome (MFS) is a genetically determined multiorgan disease that leads to severe physiological and psychological impairments in adult life. Little consensus exists regarding quality of life (QOL) in individuals with MFS. The present study sought to investigate QOL in a representative cohort of adults with MFS. METHODS: Patient-reported outcome measures from a representative sample of 102 adults with MFS (39.3±13.1 years of age; 40.2% female) were retrospectively analyzed and compared with those from adults with different congenital heart defects (CHD), at the German Heart Center Munich. QOL was assessed using the updated five-level version of the EQ-5D. RESULTS: Differences between both populations were analyzed. Subjects affected by MFS reported an overall reduced QOL. Compared to CHD patients, individuals with MFS scored significantly lower in the dimensions of pain/discomfort, anxiety/depression, mobility and usual activities (P

BACKGROUND: The number of adults with congenital heart disease (ACHD) is steadily increasing. Over their life-time, many of the affected patients require medical rehabilitation after interventional or surgical treatment of residua, sequels or complications of their congenital heart defect (CHD). However, up to now only scarce data exist about indication, performance and outcomes of cardiac rehabilitation in ACHD. METHODS: The course and outcome of rehabilitation after previous interventional or surgical treatment in ACHD was analyzed in a retrospective cohort study. RESULTS: Two hundred and five ACHD {54% female; mean age 34±12 [16–68] years} with mild (23.9%), moderate (35.1%) or severe CHD (41.0%), of whom 32% had complex CHD, 21% right-ventricular outflow tract obstructions, 20% pre-tricuspid shunts, 18% left heart or aortic anomalies, 9% post-tricuspid shunts and 2% other congenital cardiac anomalies were included into analysis. The main indications for rehabilitation were a preceding surgical (92%) or interventional (3%) treatment of the underlying CHD immediately before rehabilitation. During rehabilitation, no severe complications occurred. The number of patients in function class I/II increased from 189 to 200 and decreased in class III/IV from 16 to 5. Cardiac medication could be reduced or stopped after rehabilitation in 194 patients, with the exception of ACE-inhibitors. There was an improvement in cardiovascular risk factors. While before medical treatment 77% (n=157) patients were capable of working, the number increased to 82% [168] at the end of rehabilitation. Throughout a follow-up 9.3% (n=19) of patients needed further cardiac interventions. CONCLUSIONS: The current study provided for the first time comprehensive data on the course of rehabilitation in a large cohort of ACHD after surgical or interventional treatment. The overall outcome of ACHD after rehabilitation was uneventful and favorable. Further research is required to assess the clinical long-term outcome, the impact of rehabilitation on the quality of life, disease coping and employment. The results of this study can serve as a benchmark for the development of specific rehabilitation programs in ACHD.

Background: Due to the increase in survival rates for congenital heart disease (CHD) in the last decades, over 90% of patients today reach adulthood. Currently, there are more than 300,000 adults with CHD (ACHD) living in Germany. They have an increased need for specialized medical care, since almost all ACHD have chronic heart disease and suffer from specific chronic symptoms, risks, and sequelae. Primary care physicians (PCPs) play a crucial role in referring patients to ACHD specialists or specialized institutions. This cross-sectional study is intended to clarify the real-world care of ACHD from the PCP&rsquo, s perspective. Methods: This analysis, initiated by the German Heart Centre Munich, was based on a 27-item questionnaire on actual ACHD health care practice in Germany from the PCP&rsquo, s perspective. Results: In total, 767 questionnaires were considered valid for inclusion. The majority of the PCPs were general practitioners (95.9%), and 84.1% had cared for ACHD during the past year. A majority (69.2%) of the PCPs had cared for patients with simple CHD, while 50.6% and 33.4% had cared for patients with moderate and severe CHD, respectively, in all age groups. PCPs treated almost all typical residual symptoms and sequelae, and advised patients regarding difficult questions, including exercise capacity, pregnancy, genetics, and insurance matters. However, 33.8% of the PCPs did not even know about the existence of certified ACHD specialists or centers. Only 23.9% involved an ACHD-specialized physician in their treatment. In cases of severe cardiac issues, 70.8% of the PCPs referred patients to ACHD-certified centers. Although 52.5% of the PCPs were not sufficiently informed about existing structures, 64.2% rated the current care situation as either &ldquo, very good&rdquo, or &ldquo, good&rdquo, Only 26.3% (n = 190) of the responding physicians were aware of patient organizations for ACHD. Conclusions: The present study showed that the majority of PCPs are not informed about the ACHD care structures available in Germany. The need for specialized ACHD follow-up care is largely underestimated, with an urgent need for optimization to reduce morbidity and mortality. For the future, solutions must be developed to integrate PCPs more intensively into the ACHD care network.

Background: As a result of improved surgical and therapeutical management, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, the natural course of CHD is complicated by noncardiac medical problems. Aim of the study was to evaluate noncardiac comorbidities in a contemporary cohort of adults with CHD (ACHD). Methods: In a tertiary care center for ACHD, 821 consecutive patients, admitted to the outpatient clinic, were evaluated for clinically relevant noncardiac comorbidities. Results: The consecutively included patients (age: range, 15–80 years; 56% female) represent all types and severity grades of acyanotic and cyanotic CHD. A considerable proportion of ACHD had significant noncardiac comorbidities, which have the potential to profoundly influence the natural course of the underlying disease. In 95.5%, relevant non-cardiac comorbidities were apparent, that could be related to 16 special medical fields as endocrinologic/metabolic disease, gastroenterology/hepatology, gynecology/obstetrics, angiology, orthopedics, neurology/psychiatry and others. Most frequently seen comorbidities were endocrine and metabolic disorders (43.97%). Conclusions: Non-cardiac comorbidities are increasingly common in ACHD. The data revealed non-cardiac comorbidities as they were presented in the cohort of ACHD seen in a tertiary center. The study proves that ACHD with significant non-cardiac comorbidities need multidisciplinary care by medical organ specialists, aside the congenital cardiologist, with a deep knowledge about congenital heart defects, the special effects of the organ disease on the particular heart defect and, how the heart defect may affect the course of the particular organ disease. The study may create the basis for the development of screening programs for comorbidities in ACHD as well as a multidisciplinary concept for diagnosis and treatment of concomitant disorders or for disease prevention. Particularly disease prevention may improve quality of life as well as the further fate of the affected patients.

Immer mehr Patienten mit angeborenem Herzfehler (AHF) erreichen durch verbesserte Therapiestrategien das Erwachsenenalter. Trotz einer primar erfolgreichen Behandlung finden sich bei fast allen AHF im Verlauf Rest- und Folgezustande, die fur die Morbiditat und Mortalitat der Patienten entscheidend sind. Wegen dieser Residuen ist fast ausnahmslos eine lebenslange Nachsorge erforderlich.

Background Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. Methods and results 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23–62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and cirrhosis and 1/10 patients. Conclusions Adult Eisenmenger patients are at increased risk of hepatic impairment. Non-invasive screening could be helpful in detecting liver alterations. In our small series, however, TE could not detect fibrosis or cirrhosis in any affected patient, while ARFI was very reliable. Patients should be transferred to centres, where a multidisciplinary expert knowledge is available and a close collaboration between cardiologists and hepatologists exists.

Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy.Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast.Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (

Background: In patients with congenital heart disease (CHD), there is little data on the diagnostic and prognostic relevance of hand grip strength (HGS) for clinical assignment, while in the general population the loss of muscle strength and mass is an important risk factor in cardiovascular disease which is conversely associated with morbidity and all-cause mortality. This study aimed to assess the degree of muscle dysfunction using HGS as a biomarker in a large group of patients with CHD who often develop muscle dysfunction. Methods: In total, 385 patients (27.6±13.1 years, 43% female) were included and assigned to 5 diagnostic groups: complex anomalies (n=131), left heart anomalies/aortopathies (n=107), right heart/pulmonary artery anomalies (n=92), primary left to-right-shunts (n=42) and miscellaneous CHD (n=13). Patients with Fontan circulation, chronic cyanosis, morphologic right systemic ventricle, arterial switch operation, or Ebstein’s anomaly were analyzed separately. A control group (CG) consisted of 124 healthy individuals (30.1±12.1 years, 42% female). HGS was measured with a Jamar Hydraulic Hand Dynamometer. Results: HGS was reduced in CHD patients compared to controls (35.2±14.6 versus 43.7±14.4 kg). Most impairments were present in females (26.1±7.6 kg). Patients with cyanosis had lower HGS values compared to acyanotic CHD patients (P=0.03). Patients with left heart lesions had the highest HSG values (40.7±14.7 kg), while patients with primary left-to-right shunt lesions had the lowest HSG values (30.9±11.3 kg). Within specific groups of cardiac anomalies, patients with Fontan circulation showed the lowest (P=0.033) and patients with a morphologic right system ventricle showed higher results (P=0.004). The late mid-term survival was favorable, and 7 patients (1.8%) died in a median interval of 422 days (range, 206–1,824 days) after HGS-testing. Conclusions: This study provides the most comprehensive data on the use of HGS in CHD to date. Grip strength is an easily applicable, repeatedly usable and a cost-effective diagnostic tool to gain a quick, quantifiable assessment of the patient’s current muscle function as an expression of cardiac fitness. Considering the low number of patients who died in the observation period, HGS may not be a suitable tool for survival assessment or identification of patients at risk. However, HGS is well suited to determine muscle function and strength and thereby to identify and to follow-up patients who have an increased cardiovascular risk.

Congenital heart diseases (CHD) are a leading cause of morbidity in children with a high impact on the psychological health of parents. Possible short-term and long-term psychological problems among parents are addressed in the current paper. The diagnosis of CHD paired with subsequent surgical and interventional treatment and prolonged hospital stays cause acute psychological distress and can lead to posttraumatic stress disorders (PTSD). As the disease course progresses, the impact on parents' health tends to decrease, but the risk of developing long-term psychological issues remains high. Studies have focused mainly on stress and other distressing symptoms without explicitly addressing the effects of a CHD diagnosis on the family system. Since the social environment may play an important role in parent's life, it may be useful to conduct studies to address these issues. In particular, the psychological situation of the father and the impact of the child's disease on the different dimensions of the father's life, such as parenting skills and influences on the parental relationship, have been largely neglected. Recent research has also disregarded the impact of CHD on siblings of the affected child. Research on chronic diseases in general has shown that the children's age and severity of the disease are related to an increased level of stress. Given the severity of CHD, anxiety and depression were higher in parents with children with more severe conditions. In addition, the results suggest that a positive construction of the parent-child relationship (attachment and bonding) is impaired, especially in mothers. Mothers reported worries and concerns about the challenging tasks they would face after learning about their child's CHD and how they can deal with their child's needs. It has also been shown that the child's illness has a negative impact on the whole family system, including the parent's relationship. Impairments on the parental relationship were perceived differently among mothers and fathers. Thus, there is high need for major changes to be identified, developed and implemented in the psychological care of parents with chronically ill children. So far, research has focused more on the psychological status of parents with chronically ill children, but less research has closely examined the effects of a child's CHD on its parent's mental health even though there is a high demand in additional support. A holistic treatment approach should include professional parental support, especially during children's hospitalization, information on the home care resources and services (especially respite services) and psychological support for parents.

Die Zahl erwachsener Patienten mit angeborenen Herzfehlern (EMAH) wird in Deutschland auf ca. 280.000 geschatzt und ist damit groser als die der Kinder mit angeborenen Herzfehlern (AHF). Kardiovaskulare Rest- und Folgezustande bei fast allen EMAH erfordern die lebenslange Nachsorge. Trotzdem werden schatzungsweise mehr als 200.000 EMAH nicht von zertifizierten Spezialisten behandelt. Dies ist besonders kritisch, da viele Arzte der Basisversorgung keine spezifischen Kenntnisse uber die Probleme dieser teils sehr komplexen Anomalien haben. Auch konnen bei vorerst benignen Anomalien mit zunehmendem Patientenalter relevante Probleme auftreten, selbst nach fruhzeitig durchgefuhrter Therapie. Typisches Beispiel ist die pulmonalarterielle Hypertonie bei primaren Links-rechts-Shunt-Vitien. Unzureichende Verlaufskontrolle und Nachsorge konnen sich negativ auf Morbiditat und Mortalitat der Betroffenen auswirken. Fruhzeitige praventive Masnahmen werden moglicherweise nicht rechtzeitig eingeleitet. Kardiale Hauptprobleme der EMAH betreffen Herzinsuffizienz, -rhythmusstorungen, Endokarditiden und pulmonalvaskulare Erkrankungen. Mit zunehmendem Alter stellen kardiale oder nichtkardiale Komorbiditaten weitere Probleme dar. Diese manifestieren sich oft anders als bei erworbenen Herzerkrankungen. Dort etablierte Therapieregime lassen sich nicht unbedingt auf AHF ubertragen. Treten bei EMAH Probleme neu auf, wenden sie sich zumeist primar an ihren Haus- oder Allgemeinarzt. Trotz uberwiegend fehlender spezifischer Kenntnisse zu EMAH stellt dieser die Weichen fur die adaquate Diagnostik, Behandlung und prophylaktische Masnahmen. Daher muss der Hausarzt wissen, welcher (Kinder-) Kardiologe mit entsprechender EMAH-Erfahrung fur das gemeinsame Patientenmanagement infrage kommt.

Die Zahl der Frauen mit angeborenen Herzfehlern, die aufgrund guter Lebensqualitat eigene Kinder haben mochten, nimmt stetig zu. Dementsprechend besteht ein zunehmender Informationsbedarf hinsichtlich Beratung und Fuhrung dieser Patientengruppe. Eine Schwangerschaft mit ihren physiologischen Veranderungen kann fur die schwangere Patientin mit AHF ein erhebliches kardiales Risiko darstellen. Es lassen sich kaum allgemeingultige Aussagen treffen. Bei der Risikoabschatzung kommt es auf den individuellen Zustand der Patientin, die Art des Herzfehlers und die moglichen Residualdefekte an. Prakonzeptionell sollten therapiebedurftige Residuen sowie die korperliche Belastbarkeit der Patientin abgeklart werden. Optimierte kardiale Verhaltnisse vor einer Schwangerschaft erleichtern die Bewaltigung einer Schwangerschaft. Eine enge Kooperation zwischen Kardiologen und Geburtshelfern bez. der Beratung und Fuhrung in der Schwangerschaft ist ebenfalls unabdingbar.