Medizinische Leitlinie zur Behandlung von Erwachsenen mit angeborenen Herzfehlern (EMAH)

Schmaltz, A. A.; Bauer, Ulrike; Baumgartner, Helmut; Cesnjevar, Robert; Haan, Fokko de; Franke, C.; Gabriel, Harald; Gohlke-Bärwolf, Ch.; Hagl, S.; Hess, John; Hofbeck, Michael; Kaemmerer, Harald; Kallfelz, H. C.; Lange, Peter; Nock, Hermine; Oechslin, Erwin; Schirmer, K; Tebbe, Ulrich; Trindade, Pedro T.; Weyand, Michael; Breithardt, Günter

doi:10.1007/s00392-008-0639-8

Cited by 36 publications

(11 citation statements)

References 38 publications

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“…Adequate, life-long, specialized health care, provided by experienced ACHD specialists, is one of the most important determinants of well-being and long-term survival in ACHD [6,17,23]. This survey was the first to examine the actual medical care of ACHD in Germany, where nationwide specialized care is available from ACHD-accredited cardiologists and pediatric cardiologists in individual practices, clinics, and centers.…”

Section: Discussionmentioning

confidence: 99%

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center

Seidel

Nebel

Achenbach

et al. 2020

JCM

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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’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’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 “very good” or “good”. 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.

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Section: Discussionmentioning

confidence: 99%

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center

Seidel

Nebel

Achenbach

et al. 2020

JCM

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“…Therefore, adequate management of GUCH patients require sophisticated medical treatment from specialized paediatric and/or adult cardiologists. Concerns of sufficient medical treatment were met by scientific statements from cardiovascular societies [63] and by the establishment of international guidelines [21, 72]. …”

Section: Introductionmentioning

confidence: 99%

Molecular genetics of congenital atrial septal defects

et al. 2009

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Congenital heart defects (CHD) are the most common developmental errors in humans, affecting 8 out of 1,000 newborns. Clinical diagnosis and treatment of CHD has dramatically improved in the last decades. Hence, the majority of CHD patients are now reaching reproductive age. While the risk of familial recurrence has been evaluated in various population studies, little is known about the genetic pathogenesis of CHD. In recent years significant progress has been made in uncovering genetic processes during cardiac development. Data from human genetic studies in CHD patients indicate that the genetic aetiology was presumably underestimated in the past. Inherited mutations in genes encoding cardiac transcription factors and sarcomeric proteins were found as an underlying cause for familial recurrence of non-syndromic CHD in humans, in particular cardiac septal defects. Notably, the cardiac phenotypes most frequently seen in mutation carriers are ostium secundum atrial septal defects (ASDII). This review outlines experimental approaches employed for the detection of CHD-related genes in humans and summarizes recent findings in molecular genetics of congenital cardiac septal defects with an emphasis on ASDII.

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“…However, it is now recommended that only patients with a defined high risk of endocarditis are to receive endocarditis preventive antibiotic treatment, which is a drastic change from the recommendations from the American Heart Association in 2007 [29]. Table 2 shows an example of endocarditis protective antibiotic treatment for non-cardiac intervention [30].…”

Section: Prevention Of Endocarditismentioning

confidence: 99%

Anaesthesia management for non-cardiac surgery in children with congenital heart disease

Yamamoto¹,

Schindler²

2016

Anaesthesiol Intensive Ther

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Congenital heart disease (CHD) is the most common form of congenital abnormality and occurs in over 1% of newborns. Approximately 30% of children with CHD have other extra-cardiac anomalies, which significantly increases mortality in CHD patients. It is expected that the number of CHD patients who consult non-specialized hospitals for non-cardiac surgery after palliative or corrective operations will increase because of the extraordinary progression of treatments, such as surgical procedures, interventional procedures, and intensive care medicine, as well as diagnosis. The aim of this article is to enable anaesthesiologists who are not usually engaged in the anaesthesia management of CHD patients to provide perioperative management for CHD patients safely and with confidence by having basic and advanced knowledge about CHD patients and their pathophysiological characteristics. Anestezjologia Intensywna Terapia 2016, tom 48, nr 5, 320-328Key words: anaesthesia, children, congenital heart diseases, extra-cardiac anomalies, Fontan and hemi-Fontan circulation, Ohm's law, pulmonary vascular resistance Congenital heart disease (CHD) is the most common form of congenital abnormality and occurs in over 1% of newborns [1]. This frequency is much higher than expected and is still constant regardless of the continuous progression of prenatal diagnosis [2,3]. In addition to the diagnosis, treatments such as surgical procedures [4−7], interventional procedures [8,9], and intensive care medicine [10] have made extraordinary progress in the past several decades. CHD patients tend to have palliative or corrective operations younger than before [11], and the prognoses, especially in the patients with more complicated CHD, have improved markedly [12,13]. Therefore, it is expected that the number of CHD patients who consult non-specialized hospitals for non-cardiac surgery after palliative or corrective operations will increase. Indeed, it has been reported that the risk of perioperative cardiac arrest is higher in CHD children [14]. Although the frequency of perioperative complications for non-cardiac surgery in CHD patients who have no preoperative complications such as pulmonary hypertension, congestive heart failure, or cyanosis, has been reported to be as low as the frequency in non-CHD patients [15], recent cohort studies have reported that CHD patients who were undergoing non-cardiac surgery exhibited increased perioperative morbidity and mortality [16,17]. The aim of this article is to enable anaesthesiologists who are not usually engaged in anaesthesia management for CHD patients to provide perioperative management for CHD patients safely and with confidence by having basic and advanced knowledge about CHD patients and their pathophysiological characteristics [18].

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Medizinische Leitlinie zur Behandlung von Erwachsenen mit angeborenen Herzfehlern (EMAH)

Cited by 36 publications

References 38 publications

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center

Molecular genetics of congenital atrial septal defects

Anaesthesia management for non-cardiac surgery in children with congenital heart disease

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