The need for specialized care for patients with Grown-up Congenital Heart Disease

The pressing need for subspecialisation and specialized units for the care of Grown-up Congenital Heart Disease (GUCH) patients is discussed by Günter Breithardt with illustration from the system initiated in Germany GUCH patients, also called Adults with Congenital Heart Disease (ACHD), has become an area of ever increasing demand because, due to major advances in paediatric cardiology, cardiology, anaesthesiology and particularly in cardiac surgery, more than 85% of patients with congenital heart disease (CHD) now reach adulthood.1 The number of ACHD has already exceeded that of the paediatric population with CHD.2 Until the early 1970s, cardiology as a sub discipline of internal medicine in many countries, was mostly concerned with acquired or CHD. However, specialized paediatric cardiology units were developing rather fast during that decade. On the other hand, with the advent of invasive coronary angiography the focus in most cardiology units moved away from CHD, with coronary artery disease, heart failure, and arrhythmias becoming more and more in the forefront. The expertize in CHD became the domain of paediatric cardiologists and their collaborating cardiac surgeons. Jane Somerville, London, had opened the first dedicated ward for children and adolescents with CHD already in 1975.3 As of 1991, she successfully created the new Working Group on GUCH Disease of the European Society of Cardiology. To our knowledge, Canada was the first country to propose a comprehensive network for the medical care of adult patients with CHD through national and regional centres.4 Patients with CHD have a great variety of often complex anatomical abnormalities of the heart and circulation with ensuing haemodynamic consequences. Most ACHD after surgical or interventional treatment have anatomical and functional residuals and conditions sometimes requiring additional interventions. Heart failure, arrhythmias, sudden cardiac death, infectious endocarditis, acquired heart diseases, (progressive) pulmonary hypertension, neurological complications, emotional and intellectual impairments, and haematological and rheological abnormalities are prevalent. Due to the often persistent impairment, there is also a need for specific advice about socio-legal questions such as insurance, provision for old age, types of education (school, study, occupation), employability, physical resilience (e.g. performance ability, sporting activity), acquisition of a driver's licence, suitability to fly, and often also issues concerning pregnancy and inheritance of heart defects.5,6 The vast spectrum of unoperated or operated CHD has made it very demanding, complicated, and time-consuming to take care of and advise ACHD. Whereas CHD in children and adolescents is well cared for by paediatric cardiologists, they are mostly unfamiliar with the problems of CHD in adulthood and of acquired heart disease. Adult cardiologists, on the other hand, were not trained to take over full responsibility for ACHD. The care for such patients demands a broad knowledge of and experience with the various anatomy malformations and their hemodynamic consequences, the different surgical procedures and their late complications and eventual sequelae. Decisions on therapy are hardly evidence-based, as no large clinical trial data are available. Therapeutic approaches developed in adult or paediatric cardiology are simply transferred to adult patients with CHD.5,6 This clearly demands more evidenced-based recommendations based on sound clinical research data which, due to the diversity of CHD diagnoses and the rare occurrence of specific entities, can best be generated by intensive international collaboration.7 Although there has been a widespread network of care for paediatric patients with CHD, major problems arise when a patient with CHD reaches adulthood. This transition has often been described as falling into a void of medical care. The adult patient is then often confronted with lack of knowledge and experience with CHD among the general adult cardiologists. Recent data show that the existing ACHD programmes in Europe may have only 7.1% of the afflicted patients in active follow-up.8 Thus, many patients get lost, mostly during the transition phase. Adult patients with CHD should be treated by cardiologists who have a profound knowledge of the pathological anatomy and haemodynamics of the treatment methods used, of possible delayed consequences of prior interventions and of new interventional and surgical techniques but who at the same time are also familiar with the problems of acquired adult diseases. Apart from a few internationally recognized centres for paediatric patients, that are also taking care of adult patients with CHD, it was difficult for patients to identify the right doctor and the right centre, as there was no certification for cardiologists or paediatric cardiologists as specialists for CHD in adults. There were only a few paediatric cardiologists and adult cardiologists, both in our country and elsewhere, with sufficient specialist knowledge in the field of CHD in adults. This has been and is often still a problem in most health care systems. Also the qualification of nurses and other health care personnel is frequently inadequate to cope with the increasing number of patients. Fortunately, the scene is changing with more dedicated personnel now being available in many centres.8 In 2003, the German Cardiac Society, together with the German Paediatric Cardiology Society, the German Society for Cardio-Thoracic Surgery, and professional organisations of out-of-hospital cardiologists and paediatric cardiologists, patient organizations, and the German Heart Foundation, formed an interdisciplinary Task Force EMAH (Erwachsene mit Angeborenen Herzfehlern, Adults with Congenital Heart Disease) with the remit to consent (i) on the structure and quality of care for ACHD in Germany,5,6 (ii) on the additional qualifications needed by adult or paediatric cardiologists to take care of these patients,10 and (iii) to prepare guidelines for the care of ACHD in German speaking countries based on the ESC and other guidelines.11 Until then, there had been no structured subspecialization with certification for cardiologists who look after these patients, although experienced individuals had emerged from a few centres in the past in our country. It was agreed5,6 that, similar to the Canadian system, there should be three levels of care which are closely interlocked and which allow the movement of patients and close communication from one level to the other when needed. Care on a given level is based on the type, the severity, and the stage of the heart defect. Basic care by the general practitioner is mostly for common diseases such as colds, etc. whereas the first level of specific care for patients with CHD should be provided by certified adult or paediatric cardiologists in specialized medical practices (outside hospitals) or in regional hospitals. National centres for ACHD should represent the third level. Their physicians must be highly qualified and the centres must be equipped for the treatment of complex, serious and rare CHDs, and must therefore, offer a broad spectrum of services.5,6 In addition, training programmes for subspecialization in this field should be incorporated into these tertiary centres. These three levels of care are different from the initial recommendations of the 2003 ESC Guidelines12 which categorized patients into three subgroups based on complexity and severity of the disorder. Instead, the 2010 ESC Guidelines,13 recommended that GUCH patients should be seen at least once in a specialist centre and the GUCH specialist will then make recommendations for the level of care and follow-up intervals on an individual patient basis. The background for this recommendation was that even simple defects may require specialist care under certain circumstances (e.g. atrial septal defect with pulmonary arterial hypertension). Most important was the proposal to establish a network between specialist centres, local general adult cardiology departments, cardiologists in private practice (where available), and general practitioners. When the programme was started in our country, the chance to add subspecialization in GUCH/ACHD to the existing curricula for board-certification by the Chambers of Physicians [the National Authority according to the European Union of Medical Specialists (Union Européenne des Médecins Spécialistes) (UEMS) terminology14] in the various states of Germany was considered as low.9,10 Therefore, based on the proposal of the committee for postgraduate training of the Federal Chamber of Physicians, it was decided to issue this type of qualification as a programme of the professional and scientific societies, hoping that this degree would in the long run be accepted by the Chambers of Physicians and later incorporated into the Board certification process. After more than 10 years, the chances are now better that our regulations will be incorporated but the necessary amendment to our national and local state regulations is still under elaboration. Our requirements for training in GUCH/ACHD are applicable for both, adult and paediatric cardiologists where each group has to demonstrate special expertize in the main categories of the other field. Cardiologists specialized in adult CHD need to have appropriate knowledge, experience and special skills in the management of CHD in adults. Paediatric cardiologists have to be able to recognize the diseases which are typical for adults, e.g. coronary heart disease or hypertension. Further diagnosis and treatment of diseases typical of adulthood is reserved for adult cardiologists.9,10 These specialists should be familiar with out-patient and in-patient medical care of adult patients with CHD throughout their illness including the treatment of complications caused by the illness and preventive, rehabilitative, socio-medical, and psychological measures. This knowledge and experience is acquired during the course of a formal programme of at least 18 months of post-graduate training added to the usual training in adult or paediatric cardiology (Box 1 below). The German ACHD sub-specialization training programme for adult cardiologists includes (Hess 2007, 2011) 6 months working in a clinic for paediatric cardiology, 6 months working in a national ACHD centre accredited for this additional qualification, and the remaining 6 months can be completed in a paediatric cardiology centre, an accredited national ACHD centre, or an accredited practice or clinic specialising in ACHD. These requirements that were finally published in 20079,10 deviate from the recommendations in a subsequent position paper published in 2014 of the Working Group on Grown-up Congenital Heart Disease of the ESC15 and the 2015 ACGME Program Requirements for Graduate Medical Education in Adult Congenital Heart Disease that both now recommend 24 months of subspecialty training.16 Also the contents of the training requirements have become stricter from 2007 to 2014. As many of the applicants to our scheme far exceeded our minimum requirements for time of subspecialty training and number of procedures performed are concerned, mutual recognition of qualifications may still become difficult when the proposed ESC Working Group scheme will become active. It will be up to the UEMS to propose a unified scheme of subspecialty training in the future. A report by members of the Working Group GUCH of the ESC8 on the structure and activities of adult CHD programmes in Europe concluded that ‘important avenues for future developments in ACHD are to establish formal training for ACHD cardiologists, to increase the number of ACHD programmes, to take sufficient nurse specialists on staff to provide psychosocial and transitional care, and to reduce unnecessary variability in time spent on ACHD care’. Our own efforts in Germany have now resulted in a high number of trained and certified paediatric and adult cardiologists with the added qualification in GUCH or ACHD17: to date 223 paediatric cardiologists and 87 cardiologists. Nearly 100 certified colleagues work in office-based practices (i.e. outside hospitals). Most of the awarded certifications based on the regulations during the transition phase. This high number is mostly due to the fact that there were more paediatric cardiologists than expected, the majority of whom had been at one of the traditional large centres in Berlin and Munich, and few adult cardiologists who met the requirements and could apply during a transition phase. This process required the documentation of previous activities analogous to the requirements of training (Box 2 above), and to successfully pass a verbal examination with five examiners from adult and paediatric cardiology (two from each group), and from congenital heart surgery. At the end of the transition period, the full curriculum had to be passed, now with additional documentation of knowledge, experience, and procedural skills in a log book. In addition, 16 supra-regional EMAH-centres, three hospitals and six out-of-hospital practices have now been recognized as having a main emphasis on EMAH (EMAH-Schwerpunktklinik or—Schwerpunktpraxis). Recertification after 5 years has just started. The ACHD additional training programme for paediatric cardiologists includes 6 months working in a cardiology clinic accredited by the relevant State Chamber of Physicians for the training of internists specializing in cardiology, or, a clinic authorized to provide training in internal medicine, 6 months working in a national ACHD centre accredited for this additional qualification, and the remaining 6 months can be completed in an accredited national ACHD centre or an accredited practice or clinic specializing in ACHD. The overall GUCH landscape has changed in many countries. Kempny et al.7 assessed the evolving situation of adult CHD research and of centres, based on publications of original research in the field between 1995 and 2011. Overall, there was a dramatic increase in ACHD publications, accompanied by a matching increase in impact factors and an over proportional rise in ACHD contributions relative to the general academic field. This correlated with self-reported patient volume and the number of identified ACHD centres in Europe and North America. This clearly shows that the global interest in ACHD is increasing. One major question regarding the structure of care is whether there should be a wide network of specialized centres or whether there should be a concentration of a very few centres in a country. Diller et al.18 hypothesized that supra-regional centres may profit from larger patient and procedure numbers, improved provider expertize, as well as the effect of economies of scale and potentially more investment in technology and human capital. After studying 10 major centres in England, their results suggest that higher competition (i.e. more centres within a 2-h car ride for patients) may be associated with better clinical results and superior research output. In contrast, no such association was found when assessing centre volume, a variable that is conventionally thought to be the major driver of clinical quality and innovation in the field. The field of CHD in adults is rapidly developing and represents a great challenge for physicians and other health care personal with regard to knowledge, experiences and skills as well as for the creation of optimal network-like structures of care. Recent developments in this field are encouraging, showing that the demand for high-level care at least in some countries and regions is increasingly met. Increasing research will ultimately lead to more robust recommendations for the management of this rapidly growing patient population. Günter Breithardt, chair, Ulrike Bauer, Helmut Baumgartner, Hans Carlo Kallfelz, Harald Kaemmerer, Achim Schmaltz Other members of the Interdisciplinary EMAH Task Force: Hashim 'Abdul-Khaliq, Felix Berger, Jens Bahlmann, Ingo Dähnert, Andreas Franke, Fokko De Haan, Torben Geier, John Hess, Michael Hofbeck, Dieter Horstkotte, Hermine Nock, Thomas Paul, Christian Schlensak, Karl Robert Schirmer, Udo Sechtem, Ulrich Tebbe, Jochen Weil, and Michael Weyand. References are available as supplementary material at European Heart Journal online.