[Care of adults with congenital heart diseases in Germany-Leading role by internal medicine specialists and general practitioners]. / Versorgung von Erwachsenen mit angeborenen Herzfehlern in Deutschland ­ tragende Rolle von Internisten und Hausärzten.

BACKGROUND: 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). METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018.

In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article.

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia.

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy.

Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group.

Recommendations for adult and paediatric cardiologists on obtaining additional qualification in "Adults with Congenital Heart Disease" (ACHD).

BACKGROUND: The number of adult congenital heart disease (ACHD) patients will be larger in the medium to long term than that of children and adolescents with congenital heart disease. The present structures for the medical care of ACHD patients are not sufficient and need to be improved. Therefore the Task Force aimed at developing recommendations for adult and paediatric cardiologists to acquire the additional qualification "Adults with Congenital Heart Disease" (ACDH). METHODS: The members of the interdisciplinary Task Force were selected on the basis of their special clinical, scientific and organisational expertise. The leading author submitted a draft version, which was revised by a sub-group of the interdisciplinary Task Force. It was subsequently agreed upon and re-circulated by all the members of the Task Force. The recommendations were then presented to the relevant committees of all participating associations and groups and approved following detailed discussion. RESULTS: A training programme for acquiring an additional qualification in the treatment of adults with congenital heart disease was created successfully. CONCLUSIONS: The medical care of adults with congenital heart disease is a sub-speciality in the border area between adult cardiology and paediatric cardiology. ACHD cardiologists are to be specially trained experts with appropriate knowledge and special skills and experience in the diagnosis and therapy of congenital heart disease in adults. ACHD cardiologists should be able to recognise and treat problems that occur in adulthood in connection with congenital heart disease.

Recommendations for improving the quality of the interdisciplinary medical care of grown-ups with congenital heart disease (GUCH).

BACKGROUND/OBJECTIVES: The number of adult congenital heart disease (ACHD) patients will be larger in the medium to long term than that of children and adolescents with congenital heart disease. The present structures for the medical care of ACHD patients are not sufficient and need to be improved. Therefore the task force's assignment and objective was to develop recommendations for the structure of the interdisciplinary medical care of adults with congenital heart disease (GUCH). METHODS: The members of the interdisciplinary task force were selected on the basis of their special clinical, scientific and organizational expertise. Initially, a sub-group of the interdisciplinary task force compiled a draft version of these recommendations, with reference to international recommendations. It was circulated and then agreed with all task force members in two joint meetings. The recommendations were then submitted to the relevant committees of all participating societies and groups and approved following detailed discussion. RESULTS: With the publication of this document the interdisciplinary task force considers its first task as completed. CONCLUSIONS: The compiled recommendations for the structure of the interdisciplinary medical care of adults with congenital heart disease (GUCH) should ensure that the structural and medical pre-conditions for comprehensive GUCH medical care are created.

Pulmonary hypertension due to left heart disease: updated Recommendations of the Cologne Consensus Conference 2011.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more frequent forms of PH including PH owing to left heart disease. The guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) have not been sufficiently investigated in other forms of PH. However, despite the lack of respective efficacy data an uncritical use of targeted PAH drugs in patients with PH associated with left heart disease is currently observed at an increasing rate. This development is a matter of concern. On the other hand, PH is a frequent problem that is highly relevant for morbidity and mortality in patients with left heart disease. It that sense, the practical implementation of the European Guidelines in Germany requires the consideration of several specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH due to left heart disease. This commentary describes in detail the results and recommendations of the working group which were last updated in October 2011.

Follow-up of cardiovascular risk markers in hypertensive patients treated with irbesartan: results of the i-SEARCH Plus Registry.

Microalbuminuria (MAU), high-sensitivity C-reactive protein (hsCRP), and N-terminal pro-brain natriuretic peptide (NT-proBNP) are risk markers used to predict the prognosis of hypertensive patients; however, they have not been prospectively evaluated in primary care. An investigation was conducted using i-SEARCH Plus, a registry documenting 1649 patients with hypertension who received irbesartan at office-based cardiologists over 12 months. Mean age at baseline was 61.4±11.3 years, 43.2% were women, and blood pressure was 159.8±20.1/93.4±11.9mm Hg. Median albumin/creatinine ratio (ACR) at baseline was 9.90 (interquartile range [IQR], 5.76--25.52) mg/g, hsCRP 2.46 (IQR, 1.16--5.14) mg/L, and NT-proBNP 89.28 (IQR, 38.63-203.40) pg/mL. In patients with MAU (ACR ≥20mg/g), the age-adjusted risk of a combined end point of newly diagnosed coronary artery disease (CAD), myocardial infarction, stroke/transitory ischemic attack, and death at 12-month follow-up was increased (odds ratio [OR], 2.67; 95% confidence interval [CI], 1.49-4.76), as was the incidence of CAD (OR, 3.27; 95%CI, 1.39-7.68) and death (OR, 4.63; 95%CI, 1.44-14.94). No correlations with end points were found for hsCRP or NT-proBNP after adjusting for age and the presence of MAU. MAU is an independent predictor of cardiovascular events in hypertensive patients. These findings confirm previous reports on the prognostic value of MAU and establish its incremental value over hsCRP and NT-proBNP.

[Long-term follow-up of cardiovascular risk markers in patients with hypertension. Rationale, design, and baseline characteristics of the i-Search Plus Registry]. / Langzeitverlauf kardiovaskulärer Risikomarker bei Patienten mit Hypertonie. Rationale, Design und Ausgangscharakteristika des i-SEARCH Plus Registers.

BACKGROUND: Cardiovascular risk markers like microalbuminuria (MAU), highly sensitive C-reactive protein (hsCRP) and brain natriuretic peptide (BNP) currently gain importance to estimate risk in trials and clinical practice. Blockade of the renin-angiotensin system (RAS) has been shown to reduce some of these risk markers in clinical trials, but validation of their time course and role in clinical practice is still pending. DESIGN: To fill this gap, the design of a nationwide registry study was chosen in which patients attending their cardiologist were observed for 12 months and the effect of blocking the RAS with the angiotensin II receptor blocker irbesartan was documented. Primary question: risk for mortality and the incidence of cardiovascular events in relation to baseline values of MAU, hsCRP, and BNP. Secondary questions: correlations between cardiovascular risk markers (1) amongst each other with respect to cardiovascular events, (2) with clinical findings (echocardiography, electrocardiogram), (3) with the heart rate, (4) with further metabolic parameters (blood sugar, HbA(1c), etc.), and (5) with blood pressure control. RESULTS: Until April 1, 2006, 2,149 patients were recruited in 305 centers in Germany. Patients had a mean age of 61.4 (+/- 11.3) years. Waist circumference was 103.6 (+/- 13.5) cm. 95.1% of all patients had arterial hypertension at inclusion (> or = 140/90 mmHg). The mean value for albumin/creatinine was 68.9 (+/- 307.5) mg/g (n = 2,100), for hsCRP 4.6 (+/- 8.3) mg/l (n = 2,136), and for proBNP 236.5 (+/- 557.3) pg/ml (n = 2,138). CONCLUSION: The present register will elucidate the time course and the interdependence of the cardiovascular risk markers MAU, hsCRP and proBNP as well as their prediction of cardiovascular endpoints in hypertensive individuals. In addition, the role of RAS-blocking agents will be evaluated. A valuable contribution to estimate risk and to optimize care for cardiovascular high-risk patients in clinical practice can be expected.

[Management of patients with prosthetic heart valves]. / Nachsorge von Patienten mit künstlichen Herzklappen.

More than 15,000 valve replacements are annually performed in Germany resulting in a significant improvement of function, symptoms and prognosis. Nevertheless, prosthetic heart valves bear a small risk of serious complications and these patients remain cardiac patients all their life. Therefore, cardiologic checkups are recommended at regular intervals and, if needed, also on a short-term appointment. Despite the presence of unspecific symptoms most patients do not reveal problems concerning their prosthetic valves, which can be quickly determined using the standard examination techniques of a cardiologist. The routine "basic examination" should be extended to an "advanced examination" in case of abnormal findings (in the "basic examination"). Apart from history taking, physical examination, ECG and laboratory tests, echocardiography (either transthoracic or, if necessary, transesophageal) is the most important method for evaluation of prosthetic valves. Occasionally, complementary stress tests, right/left heart catheterization or MRI are needed. Common complications include valve thrombosis with or without embolization, endocarditis, structural failure, hemolysis and paravalvular regurgitation which can be reliably detected and treated by the cardiologist. Furthermore, it is the cardiologist's responsibility to define the patients' antithrombotic treatment regimen and to guide the patients through special situations like gravidity or before and after an operation. With some restrictions, patients with prosthetic heart valves can be fully rehabilitated in respect of professional life and sports.