Adults With Congenital Heart Disease: Not Just Big Babies* Steve Davis, MD Department of Pediatric Critical Care Medicine Cleveland Clinic Childrens Hospital Cleveland, OH
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n this issue of Pediatric Critical Care Medicine, May et al (1) describe the use of clinical decision support tools to improve compliance with adult Surgical Care Improvement Project (SCIP) measures. This is an important step in improving the care of a complex and growing population of patients without a clearly identified “home.” Many of these patients are cared for in Children’s hospitals that have not been required to submit SCIP data. This is just one of many challenges in caring for these patients in a pediatric setting. As previously noted, the population of adults with congenital heart disease (ACHD) now exceeds that of children with congenital heart disease (CHD). In addition, admissions to the hospital for ACHD patients have almost doubled in the last decade (2, 3). This has put a strain on both pediatric and adult hospitals and ICUs in particular to be able to safely care for a population of patients that is both resource intensive and complex in nature. Advances in all aspects of the care of children with CHD have resulted in survival to adulthood increasing from 10% to more than 90%. Gilboa et al (4) reviewed death certificates from 1999 to 2006 and were able to show a 24% decline in mortality due to CHD and that 76% of all CHD deaths now occur in adults. This has led to a new population within ACHD patients, geriatric patients, who now make up more than 11% of all ACHD patients. Geriatric ACHD patients admitted to tertiary care centers in the European Union increased 10-fold from 2000 to 2012 (5). This growth has spurred a healthy debate over whom and in what setting to care for these patients. Economic issues have frequently been a factor and will remain a consideration during the coming years as pressure to maintain revenue streams has never been greater. Although adult cardiology was initially concerned with structural defects, it has evolved into one primarily concerned with acquired defects such as coronary artery disease, heart failure, and arrhythmias. The ACHD population has created a need for specialists with expertise in CHD, knowledge of
*See also p. 428. Key Words: adult congenital heart disease; adult congenital heart disease cardiology; quality indicators; Surgical Care Improvement Project The author has disclosed that he does not have any potential conflicts of interest. Copyright © 2014 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies DOI: 10.1097/PCC.0000000000000137
490
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adult medicine, and a desire to care for these challenging patients. There is a considerable gap between the skills of the typical pediatric or adult cardiologist and the needs of these patients. Because early long-term survivors were primarily young adults, this challenge was not as profound in the past. Many of these patients were cared for in pediatric cardiac centers and their issues were not that different from their pediatric counterparts. As the population has aged, however, new issues such as monitoring during pregnancy, coronary artery disease, chronic obstructive pulmonary disease, diabetes, peripheral vascular disease, and chronic tobacco, drug, and alcohol use have become commonplace. These issues challenge those of us who spend the vast majority of our time caring for children. Similarly, most adult cardiac programs have significant gaps in knowledge around the care of patients with complex CHD. This has led to the development of the subspecialty of ACHD cardiology. Trainees can be either adult or pediatric cardiologists (6). There are an increasing number interested in pursuing this field, but the situation is similar to the development of dual-trained pediatric cardiologist/intensivists in that the demand far outweighs the supply and most patients will be cared for by someone without this level of training. This situation is more challenging, however, in that having a large supply of ACHD cardiologists would not alleviate the challenges these patients bring to all the other members of a multidisciplinary care team. These issues are particularly prominent in the perioperative period, where anesthesiologists, intensivists, nephrologists, pulmonologists, nurses, respiratory therapists, and many other providers will need to become well versed in the management of complex issues only rarely encountered in their training or usual practice. Webb and Veldtman (7) recently published their thoughts on setting up ACHD programs with recommendations for composition and training of members as well as who and what should be available. In the current economic environment, it will be challenging for many programs to meet these recommendations. In the absence of specific accreditation or clear guidelines from governmental bodies, it may be difficult to justify the necessary level of resource allocation. The American Congenital Heart Association is in the process of developing criteria for accreditation, which will be a welcome step toward driving rational growth of ACHD programs. Canada and the United Kingdom have already formalized to a limited extent the resources needed to provide care for this population. A recent Joint Working group of the British Congenital Cardiac Association, Intervention Society, and Cardiovascular Society published their recommendations for provision of services and quality auditing (8). Many of their recommendations are beyond the current ability of centers caring for ACHD patients. June 2014 • Volume 15 • Number 5
Editorials
Of note, they close with the statement that interventions for ACHD patients are an evolving discipline and new data are rapidly emerging; therefore, they will review these recommendations in 3 years. A working group of ACHD experts from the United States, Canada, and European Union recently developed a set of 55 quality indicators around six ACHD conditions (9). This is an important step as there have not been specific quality metrics for this population and they will hopefully be useful tools to compare ACHD programs in the future. May et al (1) were able to show excellent compliance with an established set of SCIP measures, which are largely unfamiliar to most pediatric caregivers. This is a necessary step toward improving the care of ACHD patients. This is, however, not an issue unique to pediatric cardiovascular disease. Appropriate transitions of care for children with cancer, pulmonary disease, mental health disorders, and many other conditions are also in evolution. Many centers have established “transition” clinics to more smoothly hand over young adults with chronic disease. We must continue to push collaborative efforts that are not based on revenue generating prospects. This collaboration is essential to the improvement of care as it is currently estimated that up to 70% of children are lost to follow-up or experience lapses in care after leaving pediatric cardiology practices (10, 11). This may be easier as we begin to focus more efforts on population health and move away from the episodic care of individuals made profitable by a fee-for-service base.
REFERENCES
1. May LJ, Longhurst CA, Pageler NM, et al: Optimizing Care of Adults with Congenital Heart Disease in a Pediatric Cardiovascular ICU Using Electronic Clinical Decision Support. Pediatr Crit Care Med 2014; 15:428–434
Pediatric Critical Care Medicine
2. O’Leary JM, Siddiqi OK, de Ferranti S, et al: The changing demographics of congenital heart disease hospitalizations in the United States, 1998 through 2010. JAMA 2013; 309:984–986 3. Opotowsky AR, Siddiqi OK, Webb GD: Trends in hospitalizations for adults with congenital heart disease in adult life. J Am Coll Cardiol 2009; 54:460–467 4. Gilboa SM, Salemi JL, Nembhard WN, et al: Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006. Circulation 2010; 122:2254–2263 5. Tutarel O, Kempny A, Alonso-Gonzalez R, et al: Congenital heart disease beyond the age of 60: Emergence of a new population with high resource utilization, high morbidity, and high mortality. Eur Heart J 2014; 35:725–732 6. Hess J, Bauer U, de Haan F, et al: Recommendations for adult and paediatric cardiologists on obtaining additional qualification in “Adults with Congenital Heart Disease” (ACHD). Int J Cardiol 2011; 149:186–191 7. Webb GD, Veldtman GR: Setting up an adult congenital heart programme. SA Heart 2013; 10:586–591 8. Qureshi SA, Hildick-Smith D, de Giovanni J, et al; British Congenital Cardiac Association; British Cardiovascular Intervention Society; British Cardiovascular Society: Adult congenital heart disease interventions: Recommendations from a joint working group of the British Congenital Cardiac Association, British Cardiovascular Intervention Society, and the British Cardiovascular Society. Cardiol Young 2013; 23:68–74 9. Gurvitz M, Marelli A, Mangione-Smith R, et al: Building quality indicators to improve care for adults with congenital heart disease. J Am Coll Cardiol 2013; 62:2244–2253 10. Goossens E, Stephani I, Hilderson D, et al; SWITCH(2) Investigators: Transfer of adolescents with congenital heart disease from pediatric cardiology to adult health care: An analysis of transfer destinations. J Am Coll Cardiol 2011; 57:2368–2374 11. Winter MM, Mulder BJ, van der Velde ET: Children and adults with congenital heart disease lost to follow-up: Who and when? Circulation 2010; 121:e252
www.pccmjournal.org
491
I
n this issue of Pediatric Critical Care Medicine, May et al (1) describe the use of clinical decision support tools to improve compliance with adult Surgical Care Improvement Project (SCIP) measures. This is an important step in improving the care of a complex and growing population of patients without a clearly identified “home.” Many of these patients are cared for in Children’s hospitals that have not been required to submit SCIP data. This is just one of many challenges in caring for these patients in a pediatric setting. As previously noted, the population of adults with congenital heart disease (ACHD) now exceeds that of children with congenital heart disease (CHD). In addition, admissions to the hospital for ACHD patients have almost doubled in the last decade (2, 3). This has put a strain on both pediatric and adult hospitals and ICUs in particular to be able to safely care for a population of patients that is both resource intensive and complex in nature. Advances in all aspects of the care of children with CHD have resulted in survival to adulthood increasing from 10% to more than 90%. Gilboa et al (4) reviewed death certificates from 1999 to 2006 and were able to show a 24% decline in mortality due to CHD and that 76% of all CHD deaths now occur in adults. This has led to a new population within ACHD patients, geriatric patients, who now make up more than 11% of all ACHD patients. Geriatric ACHD patients admitted to tertiary care centers in the European Union increased 10-fold from 2000 to 2012 (5). This growth has spurred a healthy debate over whom and in what setting to care for these patients. Economic issues have frequently been a factor and will remain a consideration during the coming years as pressure to maintain revenue streams has never been greater. Although adult cardiology was initially concerned with structural defects, it has evolved into one primarily concerned with acquired defects such as coronary artery disease, heart failure, and arrhythmias. The ACHD population has created a need for specialists with expertise in CHD, knowledge of
*See also p. 428. Key Words: adult congenital heart disease; adult congenital heart disease cardiology; quality indicators; Surgical Care Improvement Project The author has disclosed that he does not have any potential conflicts of interest. Copyright © 2014 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies DOI: 10.1097/PCC.0000000000000137
490
www.pccmjournal.org
adult medicine, and a desire to care for these challenging patients. There is a considerable gap between the skills of the typical pediatric or adult cardiologist and the needs of these patients. Because early long-term survivors were primarily young adults, this challenge was not as profound in the past. Many of these patients were cared for in pediatric cardiac centers and their issues were not that different from their pediatric counterparts. As the population has aged, however, new issues such as monitoring during pregnancy, coronary artery disease, chronic obstructive pulmonary disease, diabetes, peripheral vascular disease, and chronic tobacco, drug, and alcohol use have become commonplace. These issues challenge those of us who spend the vast majority of our time caring for children. Similarly, most adult cardiac programs have significant gaps in knowledge around the care of patients with complex CHD. This has led to the development of the subspecialty of ACHD cardiology. Trainees can be either adult or pediatric cardiologists (6). There are an increasing number interested in pursuing this field, but the situation is similar to the development of dual-trained pediatric cardiologist/intensivists in that the demand far outweighs the supply and most patients will be cared for by someone without this level of training. This situation is more challenging, however, in that having a large supply of ACHD cardiologists would not alleviate the challenges these patients bring to all the other members of a multidisciplinary care team. These issues are particularly prominent in the perioperative period, where anesthesiologists, intensivists, nephrologists, pulmonologists, nurses, respiratory therapists, and many other providers will need to become well versed in the management of complex issues only rarely encountered in their training or usual practice. Webb and Veldtman (7) recently published their thoughts on setting up ACHD programs with recommendations for composition and training of members as well as who and what should be available. In the current economic environment, it will be challenging for many programs to meet these recommendations. In the absence of specific accreditation or clear guidelines from governmental bodies, it may be difficult to justify the necessary level of resource allocation. The American Congenital Heart Association is in the process of developing criteria for accreditation, which will be a welcome step toward driving rational growth of ACHD programs. Canada and the United Kingdom have already formalized to a limited extent the resources needed to provide care for this population. A recent Joint Working group of the British Congenital Cardiac Association, Intervention Society, and Cardiovascular Society published their recommendations for provision of services and quality auditing (8). Many of their recommendations are beyond the current ability of centers caring for ACHD patients. June 2014 • Volume 15 • Number 5
Editorials
Of note, they close with the statement that interventions for ACHD patients are an evolving discipline and new data are rapidly emerging; therefore, they will review these recommendations in 3 years. A working group of ACHD experts from the United States, Canada, and European Union recently developed a set of 55 quality indicators around six ACHD conditions (9). This is an important step as there have not been specific quality metrics for this population and they will hopefully be useful tools to compare ACHD programs in the future. May et al (1) were able to show excellent compliance with an established set of SCIP measures, which are largely unfamiliar to most pediatric caregivers. This is a necessary step toward improving the care of ACHD patients. This is, however, not an issue unique to pediatric cardiovascular disease. Appropriate transitions of care for children with cancer, pulmonary disease, mental health disorders, and many other conditions are also in evolution. Many centers have established “transition” clinics to more smoothly hand over young adults with chronic disease. We must continue to push collaborative efforts that are not based on revenue generating prospects. This collaboration is essential to the improvement of care as it is currently estimated that up to 70% of children are lost to follow-up or experience lapses in care after leaving pediatric cardiology practices (10, 11). This may be easier as we begin to focus more efforts on population health and move away from the episodic care of individuals made profitable by a fee-for-service base.
REFERENCES
1. May LJ, Longhurst CA, Pageler NM, et al: Optimizing Care of Adults with Congenital Heart Disease in a Pediatric Cardiovascular ICU Using Electronic Clinical Decision Support. Pediatr Crit Care Med 2014; 15:428–434
Pediatric Critical Care Medicine
2. O’Leary JM, Siddiqi OK, de Ferranti S, et al: The changing demographics of congenital heart disease hospitalizations in the United States, 1998 through 2010. JAMA 2013; 309:984–986 3. Opotowsky AR, Siddiqi OK, Webb GD: Trends in hospitalizations for adults with congenital heart disease in adult life. J Am Coll Cardiol 2009; 54:460–467 4. Gilboa SM, Salemi JL, Nembhard WN, et al: Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006. Circulation 2010; 122:2254–2263 5. Tutarel O, Kempny A, Alonso-Gonzalez R, et al: Congenital heart disease beyond the age of 60: Emergence of a new population with high resource utilization, high morbidity, and high mortality. Eur Heart J 2014; 35:725–732 6. Hess J, Bauer U, de Haan F, et al: Recommendations for adult and paediatric cardiologists on obtaining additional qualification in “Adults with Congenital Heart Disease” (ACHD). Int J Cardiol 2011; 149:186–191 7. Webb GD, Veldtman GR: Setting up an adult congenital heart programme. SA Heart 2013; 10:586–591 8. Qureshi SA, Hildick-Smith D, de Giovanni J, et al; British Congenital Cardiac Association; British Cardiovascular Intervention Society; British Cardiovascular Society: Adult congenital heart disease interventions: Recommendations from a joint working group of the British Congenital Cardiac Association, British Cardiovascular Intervention Society, and the British Cardiovascular Society. Cardiol Young 2013; 23:68–74 9. Gurvitz M, Marelli A, Mangione-Smith R, et al: Building quality indicators to improve care for adults with congenital heart disease. J Am Coll Cardiol 2013; 62:2244–2253 10. Goossens E, Stephani I, Hilderson D, et al; SWITCH(2) Investigators: Transfer of adolescents with congenital heart disease from pediatric cardiology to adult health care: An analysis of transfer destinations. J Am Coll Cardiol 2011; 57:2368–2374 11. Winter MM, Mulder BJ, van der Velde ET: Children and adults with congenital heart disease lost to follow-up: Who and when? Circulation 2010; 121:e252
www.pccmjournal.org
491
