GUCH

About: GUCH is a research topic. Over the lifetime, 137 publications have been published within this topic receiving 4106 citations.

MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE: An Increasing Problem

Abstract: A small new population of patients in cardiology, the grown-up (adult) congenital hearts (GUCH), need superspecialist care and expertise, particularly those patients with complex defects previously operated on in childhood or who were not operated on. Arrhythmias are the most common cardiologic reason for admission to the hospital, and atrial flutter is the most frequent disorder of rhythm, usually related to hemodynamic disturbances. The GUCHs need solutions to problems of transition of care, ideally an adolescent area for the younger patients, knowledge of which and when to investigate each anomaly, expertise in cardiac catheterization and interventions, cardiac surgeons experienced in congenital as well as adult disease since one in five admissions are for cardiac surgery, at-risk pregnancy service, counseling on life's problems, and most of all a few dedicated centers with cardiologists and surgeons trained in this subspecialty. The profession needs to achieve optimal care for these precious patients, particularly those with complicated lesions and complex surgery, particularly with the valve replacements. Although small in number and high in expense, GUCH patients should be centralized so expertise and experience can be concentrated despite the need to cross barriers of finance and bureaucracy. Currently, with the exception of a few expert centers, these patients receive suboptimal medical management.

Pregnancy and congenital heart disease

Abstract: Congenital heart disease occurs in 0.8% of newborn infants around the world. Advances in medical and surgical treatments over the past decades has led to more than 85% of these infants surviving to adulthood.1 2 Most interventions, however, have not been curative and about half of adults with congenital heart disease face the prospect of further surgery, arrhythmia, heart failure, and—if managed inappropriately—premature death. The burden of pregnancy represents a new challenge in women with congenital heart disease. In the United Kingdom about 250 000 adults have congenital heart disease (also known as “grown up congenital heart disease (GUCH)” patients), and this number is growing.3 Half of these patients are women, most of reproductive age. After suicide, cardiac disease is now the leading cause of maternal death in the UK, with most of these casualties having had congenital heart disease.4 The medical profession should therefore be aware of the risks that women with congenital heart disease face during pregnancy so that they can be given adequate preconception counselling and optimal care during pregnancy, delivery, and the postpartum period.5–7 Discussions about future pregnancies, family planning, and contraception should begin in adolescence to prevent accidental and potentially dangerous pregnancies in women with congenital heart disease. The impact of heart disease on childbearing potential needs to be explained clearly and sympathetically. Counselling has to address how pregnancy may affect not just the mother but also the fetus and the rest of the family (box 1). This allows women to make an informed choice whether they wish to accept the risks associated with pregnancy. The counselling should ideally be provided in a joint clinic by an obstetrician with expertise in heart disease and a cardiologist with special training in adult congenital heart disease. ### The risk for the mother The risk for pregnant women …

Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK

Abstract: Executive summary: This report addresses the needs and problems of grown-up congenital heart (GUCH) patients and makes recommendations on organisation of national medical care, training of specialists, and education of the profession. The size of the national population of patients with grown-up congenital heart disease (GUCH) is uncertain, but since 80–85% of patients born with congenital heart disease now survive to adulthood (age 16 years), an annual increase of 2500 can be anticipated according to birth rate. Organisation of medical care is haphazard with only three of 18 cardiac surgical centres operating on over 30 cases per annum and only two established specialised units fully equipped and staffed. Not all grown-ups with congenital heart disease require the same level of expertise; 20–25% are complex, rare, etc, and require life long expert supervision and/or intervention; a further 35–40% require access to expert consultation. The rest, about 40%, have simple or cured diseases and need little or no specialist expertise. The size of the population needing expertise is small in comparison to coronary and hypertensive disease, aging, and increasing in complexity. It requires expert cardiac surgery and specialised medical cardiology, intensive care, electrophysiology, imaging and interventions, “at risk” pregnancy services, connection to transplant services familiar with their basic problem, clinical nurse specialist advisors, and trained nurses. An integrated national service is described with 4–6 specialist units established within adult cardiology, ideally in relation or proximity to university hospital/departments in appropriate geographic location, based in association with established paediatric cardiac surgical centres with designated inpatient and outpatient facilities for grown-up patients with congenital heart disease. Specialist units should accept responsibility for educating the profession, training the specialists, cooperative research, receiving patients “out of region”, sharing particular skills between each other, and they must liaise with other services and trusts in the health service, particularly specified outpatient clinics in district and regional centres. Not every regional cardiac centre requires a full GUCH specialised service since there are too few patients. Complex patients need to be concentrated for expertise, experience, and optimal management. Transition of care from paediatric to adult supervision should be routine, around age 16 years, flexibly managed, smooth, and explained to patient and family. Each patient should be entered into a local database and a national registry needs to be established. The Department of Health should accept responsibility of dissemination of information on special needs of such patients. The GUCH Patients’ Association is active in helping with lifestyle and social problems. Easy access to specialised care for those with complex heart disease is crucial if the nation accepts, as it should, continued medical responsibility to provide optimal medical care for GUCH patients.

Infective endocarditis in the grown-up congenital heart (GUCH) population

Abstract: Aims Infective endocarditis accounts for 4% of admissions to a specialized unit for grown-up congenital heart patients. This study defines lesions susceptible to infection, antecedent events, organisms, outcome and surgical treatment in a group of such patients. Methods and results The grown-up congenital heart disease database was searched for all patients aged 13 years and above with adequate documentation of infective endocarditis retrospectively between 1983–1993 and thereafter between 1993–1996. There were 185 patients (214 episodes) divided into Group I: 128 patients unoperated or palliated and Group II: 57 patients after definitive repair and/or valve repair/replacement. In Group I, the commonest affected sites were ventricular septal defect in 31 (24%), left ventricular outflow tract in 22 (17%) and mitral valve in 17 (13%) and in Group II, left ventricular outflow tract in 20 (35%), repaired Fallot in 11 (19%), and atrioventricular defects in eight (14%). Infective endocarditis was not seen in secundum atrial septal defects before or after closure; in closed ventricular septal defects and ducts without left-sided valve abnormality; in isolated pulmonary stenosis; in unrepaired Ebstein; or after Fontan-type or Mustard operations. Surgery was performed in 39 patients: as an emergency in 17, and for failed medical therapy in 22. Only 87 (41%) of patients had a predisposing event: dental procedure or sepsis were the commonest events in Group I (33%) and cardiac surgery in Group II (50%). Streptococci species were found in 54% of Group I patients and in 45% of Group II. Staphylococci aureus was commoner in Group II (25%) compared to Group I (14%). Mean time from the onset of symptoms to diagnosis was 60 and 29 days in Groups I and II, respectively. Eight (4%) patients died as a result of septicaemia related to emergency or repeated surgery and Staphylococcus aureus infection. Recurrent attacks occurred in 21 (11%) patients. Conclusion Reparative surgery does not prevent endo-carditis except for closure of a ventricular septal defect and duct. Delay in diagnosis is serious since it contributes to mortality, although the overall mortality % is not high. Specific lesions are not affected so prophylaxis is probably unnecessary in those anomalies.