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GUEST EDITORIAL Table of Contents   
Year : 2008  |  Volume : 11  |  Issue : 1  |  Page : 3-5
Grown-up congenital heart (GUCH) disease: An evolving global challenge

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

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

How to cite this URL:
DiNardo JA. Grown-up congenital heart (GUCH) disease: An evolving global challenge. Ann Card Anaesth [serial online] 2008 [cited 2022 May 21];11:3-5. Available from:

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.

   References Top

1.Warnes CA. The adult with congenital heart disease: Born to be bad? J Am Coll Cardiol 2005;46:1-8.  Back to cited text no. 1    
2.Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, et al. Task force 1: The changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37:1170-5.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation 2007;115:163-72.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Daebritz SH. Update in adult congenital cardiac surgery. Pediatr Cardiol 2007;28:96-104.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Saxena A. Congenital heart disease in India: A status report. Indian J Pediatr 2005;72:595-8.  Back to cited text no. 5    
6.Rao SG. Pediatric cardiac surgery in developing countries. Pediatr Cardiol 2007;28:144-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Perloff JK, Warnes CA. Challenges posed by adults with repaired congenital heart disease. Circulation 2001;103:2637-43.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Foster E, Graham TP Jr, Driscoll DJ, Reid GJ, Reiss JG, Russell IA, et al. Task force 2: Special health care needs of adults with congenital heart disease. J Am Coll Cardiol 2001;37:1176-83.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Child JS, Collins-Nakai RL, Alpert JS, Deanfield JE, Harris L, McLaughlin P, et al. Task force 3: Workforce description and educational requirements for the care of adults with congenital heart disease. J Am Coll Cardiol 2001;37:1183-7.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Landzberg MJ, Murphy DJ Jr, Davidson WR Jr, Jarcho JA, Krumholz HM, Mayer JE Jr, et al. Task force 4: Organization of delivery systems for adults with congenital heart disease. J Am Coll Cardiol 2001;37:1187-93  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Skorton DJ, Garson A Jr, Allen HD, Fox JM, Truesdell SC, Webb GD, et al. Task force 5: Adults with congenital heart disease: Access to care. J Am Coll Cardiol 2001;37:1193-8.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Williams RG, Pearson GD, Barst RJ, Child JS, del Nido P, Gersony WM, et al. Report of the National Heart, Lung and Blood Institute Working Group on research in adult congenital heart disease. J Am Coll Cardiol 2006;47:701-7.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Therrien J, Dore A, Gersony W, Iserin L, Liberthson R, Meijboom F, et al. CCS Consensus Conference 2001 update: Recommendations for the management of adults with congenital heart disease. Part I. Can J Cardiol 2001;17:940-59.  Back to cited text no. 13    
14.Therrien J, Gatzoulis M, Graham T, Bink-Boelkens M, Connelly M, Niwa K, et al. Canadian Cardiovascular Society Consensus Conference 2001 update: Recommendations for the Management of Adults with Congenital Heart Disease--Part II. Can J Cardiol 2001;17:1029-50.  Back to cited text no. 14  [PUBMED]  
15.Therrien J, Warnes C, Daliento L, Hess J, Hoffmann A, Marelli A, et al. Canadian Cardiovascular Society Consensus Conference 2001 update: Recommendations for the management of adults with congenital heart disease part III. Can J Cardiol 2001;17:1135-58  Back to cited text no. 15  [PUBMED]  
16.Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A, et al. Management of grown up congenital heart disease. Eur Heart J 2003;24:1035-84  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Niwa K, Perloff JK, Webb GD, Murphy D, Liberthson R, Warnes CA, et al. Survey of specialized tertiary care facilities for adults with congenital heart disease. Int J Cardiol 2004;96:211-6.  Back to cited text no. 17  [PUBMED]  [FULLTEXT]

Correspondence Address:
James A DiNardo
Senior Associate in Cardiac Anaesthesia, Children's Hospital Boston and Associate Professor of Anaesthesia, Harvard Medical School
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.38442

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