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

Abstract

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.

Figure 1

Data compiled from the statistics of the Office of Population Consensus and Surveys for England and Wales showing reported deaths from congenital heart disease in the various age groups. In 1958 death was most common in infancy and by 1986 deaths were more common over age 20 years.

Figure 2

Number and ages of grown-up patients with congenital heart disease admitted to the National Heart Hospital and Royal Brompton Hospital 1975 to 1997. In the last decade there has been an increase in adults over age 30 years. From JS, GUCH Unit database.

Figure 3

The principal reasons for 373 medical admissions to the Royal Brompton Hospital GUCH unit in 1997. Abbreviations: assess, assessment; BE, bacterial endocarditis; med, medical; MRI, magnetic resonance imaging; TOE, transoesophageal echocardiography. From JS, GUCH Unit database.

Figure 4

Operations performed in 1997 in the Royal Brompton GUCH specialist unit. (A) First operation. *Atrial septal defects (ovale type) now mostly closed by non-surgical intervention, profoundly reducing surgical treatment for this lesion. (B) Reoperation. Abbreviations: AoV, aortic valve; ASD, atrial septal defect; AVR, aortic valve replacement; clos, closure; Co Ao, coarctation of the aorta; Lig, ligation; LV, left ventricle; MV, mitral valve; OP, ostium primum; PA, pulmonary artery; PDA, patent ductus arteriosus; prev, previous; Reop, reoperation; Rep, repair; repl, replacement; Res, resection; RVOTR, right ventricular outflow tract reconstruction; Sec, secundum; sub AS, subaortic stenosis; TC, total correction; TP, transplant; valvot, valvotomy; VSD, ventricular septal defect. From JS, GUCH Unit database.