Year : 2008  |  Volume : 11  |  Issue : 1  |  Page : 3--5

Grown-up congenital heart (GUCH) disease: An evolving global challenge

James A DiNardo 
 Senior Associate in Cardiac Anaesthesia, Children's Hospital Boston and Associate Professor of Anaesthesia, Harvard Medical School, USA

Correspondence Address:
James A DiNardo
Senior Associate in Cardiac Anaesthesia, Children«SQ»s Hospital Boston and Associate Professor of Anaesthesia, Harvard Medical School

How to cite this article:
DiNardo JA. Grown-up congenital heart (GUCH) disease: An evolving global challenge.Ann Card Anaesth 2008;11:3-5

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DiNardo JA. Grown-up congenital heart (GUCH) disease: An evolving global challenge. Ann Card Anaesth [serial online] 2008 [cited 2020 Sep 29 ];11:3-5
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Over the last 30 years, owing to worldwide advances in paediatric cardiology, cardiac surgery, cardiac anaesthesia and cardiac intensive care, the survival of children suffering with congenital heart disease (CHD) into adulthood has increased to approximately 95%. [1] The number of adults with CHD is rapidly increasing to a level where adults with CHD will outnumber the children with CHD in the very near future. The lack of comprehensive databases in most parts of the world causes difficulty in precise quantification of this evolving population but some data is available. A conservative estimate of the number of adult patients with CHD in the United States in the year 2000 was 787,800; of these, approximately 300,000 suffer with moderate CHD and approximately 117,000 suffer with truly complex disease. [2] [Table 1],[Table 2] delineate the moderate and complex diseases as defined in this analysis. The data obtained from Quebec, Canada, indicates that 49% of the patients alive in the year 2000 with severe CHD (defined as teratology of Fallot, complete atrioventricular canal defect, transposition complex, truncus arteriosus, univentricular heart or hypoplastic left heart syndrome) were adults. [3]

Six subsets of patients comprise the GUCH population are the following: [4] (1) CHD patients with simple lesions or survivable complex lesions presenting for the initial treatment, (2) CHD patients with previous palliative procedures presenting for further palliation or correction, (3) CHD patients presenting for anticipated reoperative procedures, (4) CHD patients presenting for the repair of residual lesions, (5) CHD patients presenting for thoracic organ transplantation, (6) CHD patients presenting for the treatment of acquired heart disease. The variation in the relative proportions of these groups forming the GUCH population in a particular country or region can be estimated. In many developing nations, late presentation of CHD patients is common due to the economic and cultural restraints. [5] In addition, for neonates and infants, palliative procedures with or without later definitive repair may result in lower mortality and better resource allocation compared to that in the definitive early repair. [6] Thus, while the composition of the GUCH population may be different in the Indian subcontinent compared to that in North America and Europe, there is every reason to believe that a rapidly growing GUCH population worldwide will require care.

The medical issues that should be addressed in the GUCH population can rightfully be characterized as residues (lesions for the most part intentionally left behind at the time of reparative surgery) or sequelae (necessary consequences of reparative operations or the natural history of the lesions). [7] These issues have comprehensively been reviewed elsewhere. [1] The requirements of these patients however are more extensive than the management of complex medical problems and include psychosocial, vocational, reproductive and exercise and rehabilitation issues. Comprehensive care provided by an appropriately educated multidisciplinary team is necessary. The regional centers will be required to coordinate care for such patients. Guaranteeing access to care will require identification of patients, creation of advocacy and patient education groups and creative solutions to overcome financial and organisational obstacles to delivery of care. These issues have been extensively addressed in a 5-part Task Force statement generated by the American College of Cardiology in 2001 [2],[8],[9],[10],[11] and by the National Heart, Lung and Blood Institute in 2006. [12] Similar consensus documents have recently been generated by the Canadian Society of Cardiology [13],[14],[15] and the European Society of Cardiology. [16]

Despite the implementation of these guidelines and creation of designated centers to care for GUCH patients in some regions of the world, a majority of population remains underserved. Based on the number of GUCH patients treated at six specialised tertiary care centers in North America and Europe, a significant shortfall in the provision of tertiary care to this patient population has been reported. [17] It has recently been concluded that GUCH patients often receive no care or suboptimal care, which is perhaps the worst service provided by any cardiovascular subspecialty. [1] As an integral part of the GUCH care team, it is incumbent upon the anaesthesiologists to be involved in the planning and implementation of the comprehensive and multidisciplinary programs that will be necessary to adequately and compassionately care for these patients at present and in the foreseeable future. Obviously, one organizational scheme will not be appropriate for all patients. A viable system that is sensitive to the belief systems and the traditions and customs of the population it serves is required. In addition, the constraints imposed by the local and national health care policies and limitations to resource allocation will also have to be considered.


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