Heart Failure in Adult Congenital Heart Disease
P re f a c e Evolving Perspectives on H e a r t F a i l u re i n A d u l t s w i t h Congenital Heart Disease
Alexander R. Opotowsky, MD, MPH
Michael J. Landzberg, MD
Editors
Fig. 1. Number of publications listed on PubMed (www.pubmed.org) using MESH terms for both “heart failure” and “congenital heart disease,” 1965 to 2013. Data for 2013 extrapolated from publications listed through August 2013.
systematic scientific investigation can be challenging. The presence of unique, some might say unnatural, arrangements, however, provides opportunities for rich exploration. This issue of Heart Failure Clinics includes articles on a broad range of topics related to the syndrome of heart failure in adults with
Heart Failure Clin 10 (2014) xv–xvi http://dx.doi.org/10.1016/j.hfc.2013.10.005 1551-7136/14/$ – see front matter Ó 2014 Published by Elsevier Inc.
heartfailure.theclinics.com
It may be argued that a sizable subset, even the majority, of adults living with congenital heart disease (CHD) have heart failure as defined by Zipes and Braunwald, “the pathophysiologic state in which the heart is unable to pump blood at a rate commensurate with the requirements of the metabolizing tissues or can do so only from an elevated filling pressure.”1 A proposed alternative definition, “heart failure is the label for a cardiovascular syndrome that is lacking uniform criteria for definition,” would perhaps best align with heart failure among adults with CHD.2 This topic is of increasing interest and importance, as reflected in the number of publications relating to both CHD and heart failure published annually (Fig. 1), and not only because adults with CHD comprise a growing aging population with more clinically apparent heart failure.3,4 As importantly, heart failure syndromes in adults with CHD are vastly heterogeneous, are often sustained or in flux over a lifetime, and highlight the frequently overlooked role of multisystem dysfunction and complex systems biology integral to understanding clinical presentation. Research may require identifying physiologically consistent subsets from the diverse universe of CHD, resulting in small populations to study. As a result,
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Preface CHD. How is it that patients with no subpulmonary ventricle,5 or with a morphologic right ventricle ejecting into the systemic circulation, or with systemic pulmonary artery pressures and chronic cyanosis may live seemingly normal lives without subjective limitation well into adulthood?6 How do these patients successfully adapt to intrinsic cardiovascular dysfunction and associated psychosocial stress and strain?7 As more women with previously fatal forms of CHD survive to adulthood, pregnancy has become increasingly common8–10; how do the hemodynamic and neurohormonal changes of pregnancy interact with underlying CHD?11 Can we extrapolate the prognostic value of specific biomarkers or standard imaging assessment or effectiveness of medical therapies for the systemic left ventricle to the systemic right ventricle, or to patients with a single ventricle?12–14 This issue of Heart Failure Clinics reflects the efforts of clinicians and investigators across the globe with a shared goal to answer these and other questions to improve the lives of patients with CHD and better understand how what superficially appears to be physiologically applicable only to a few patients may help us understand the wider syndrome of heart failure, however defined, in the general population. Alexander R. Opotowsky, MD, MPH Boston Adult Congenital Heart and Pulmonary Hypertension Service Boston Children’s Hospital Brigham and Women’s Hospital 300 Longwood Avenue, Bader 209 Boston, MA 02115, USA Michael J. Landzberg, MD Boston Adult Congenital Heart and Pulmonary Hypertension Service Boston Children’s Hospital Brigham and Women’s Hospital 300 Longwood Avenue, Bader 209 Boston, MA 02115, USA E-mail addresses: [email protected] (A.R. Opotowsky) [email protected] (M.J. Landzberg)
REFERENCES 1. Zipes DP, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine. Philadelphia: W.B. Saunders; 2005. 2. Coronel R, de Groot JR, van Lieshout JJ. Defining heart failure. Cardiovasc Res 2001;50:419–22. 3. Rodriguez FH 3rd, Moodie DS, Parekh DR, et al. Outcomes of heart failure-related hospitalization in adults with congenital heart disease in the United States. Congenit Heart Dis 2012. 4. Rodriguez FH, Marelli AJ. The Epidemiology of heart failure in adults with congenital heart disease. Heart Fail Clin 2013;10(1):1–7. 5. Gewillig M, Goldberg DJ. Failure of the Fontan circulation. Heart Fail Clin 2013;10(1):105–16. 6. Opotowsky AR, Landzberg MJ, Beghetti M. The exceptional and far-flung manifestations of heart failure in Eisenmenger syndrome. Heart Fail Clin 2013; 10(1):91–104. 7. Kovacs AH, Moons P. Psychosocial functioning and quality of life in adults with congenital heart disease and heart failure. Heart Fail Clin 2013;10(1):35–42. 8. Opotowsky AR, Siddiqi OK, D’Souza B, et al. Maternal cardiovascular events during childbirth among women with congenital heart disease. Heart 2012;98:145–51. 9. Tobler D, Fernandes SM, Wald RM, et al. Pregnancy outcomes in women with transposition of the great arteries and arterial switch operation. Am J Cardiol 2010;106:417–20. 10. Said SM, Dearani JA, Silversides CK, et al. Longerterm issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation. Cardiol Young 2011;21(Suppl 2):93–100. 11. Grewal J, Silversides CK, Colman JM. Pregnancy in women with heart disease: risk assessment and management of heart failure. Heart Fail Clin 2013; 10(1):117–30. 12. Ohuchi H, Diller GP. Biomarkers in adult congenital heart disease heart failure. Heart Fail Clin 2013; 10(1):43–56. 13. Burchill LJ, Mertens L, Broberg CS. Imaging for the assessment of heart failure in congenital heart disease: ventricular function and beyond. Heart Fail Clin 2013;10(1):9–22. 14. Book WM, Shaddy RE. Medical therapy in adults with congenital heart disease. Heart Fail Clin 2013; 10(1):167–78.
P re f a c e Evolving Perspectives on H e a r t F a i l u re i n A d u l t s w i t h Congenital Heart Disease
Alexander R. Opotowsky, MD, MPH
Michael J. Landzberg, MD
Editors
Fig. 1. Number of publications listed on PubMed (www.pubmed.org) using MESH terms for both “heart failure” and “congenital heart disease,” 1965 to 2013. Data for 2013 extrapolated from publications listed through August 2013.
systematic scientific investigation can be challenging. The presence of unique, some might say unnatural, arrangements, however, provides opportunities for rich exploration. This issue of Heart Failure Clinics includes articles on a broad range of topics related to the syndrome of heart failure in adults with
Heart Failure Clin 10 (2014) xv–xvi http://dx.doi.org/10.1016/j.hfc.2013.10.005 1551-7136/14/$ – see front matter Ó 2014 Published by Elsevier Inc.
heartfailure.theclinics.com
It may be argued that a sizable subset, even the majority, of adults living with congenital heart disease (CHD) have heart failure as defined by Zipes and Braunwald, “the pathophysiologic state in which the heart is unable to pump blood at a rate commensurate with the requirements of the metabolizing tissues or can do so only from an elevated filling pressure.”1 A proposed alternative definition, “heart failure is the label for a cardiovascular syndrome that is lacking uniform criteria for definition,” would perhaps best align with heart failure among adults with CHD.2 This topic is of increasing interest and importance, as reflected in the number of publications relating to both CHD and heart failure published annually (Fig. 1), and not only because adults with CHD comprise a growing aging population with more clinically apparent heart failure.3,4 As importantly, heart failure syndromes in adults with CHD are vastly heterogeneous, are often sustained or in flux over a lifetime, and highlight the frequently overlooked role of multisystem dysfunction and complex systems biology integral to understanding clinical presentation. Research may require identifying physiologically consistent subsets from the diverse universe of CHD, resulting in small populations to study. As a result,
xvi
Preface CHD. How is it that patients with no subpulmonary ventricle,5 or with a morphologic right ventricle ejecting into the systemic circulation, or with systemic pulmonary artery pressures and chronic cyanosis may live seemingly normal lives without subjective limitation well into adulthood?6 How do these patients successfully adapt to intrinsic cardiovascular dysfunction and associated psychosocial stress and strain?7 As more women with previously fatal forms of CHD survive to adulthood, pregnancy has become increasingly common8–10; how do the hemodynamic and neurohormonal changes of pregnancy interact with underlying CHD?11 Can we extrapolate the prognostic value of specific biomarkers or standard imaging assessment or effectiveness of medical therapies for the systemic left ventricle to the systemic right ventricle, or to patients with a single ventricle?12–14 This issue of Heart Failure Clinics reflects the efforts of clinicians and investigators across the globe with a shared goal to answer these and other questions to improve the lives of patients with CHD and better understand how what superficially appears to be physiologically applicable only to a few patients may help us understand the wider syndrome of heart failure, however defined, in the general population. Alexander R. Opotowsky, MD, MPH Boston Adult Congenital Heart and Pulmonary Hypertension Service Boston Children’s Hospital Brigham and Women’s Hospital 300 Longwood Avenue, Bader 209 Boston, MA 02115, USA Michael J. Landzberg, MD Boston Adult Congenital Heart and Pulmonary Hypertension Service Boston Children’s Hospital Brigham and Women’s Hospital 300 Longwood Avenue, Bader 209 Boston, MA 02115, USA E-mail addresses: [email protected] (A.R. Opotowsky) [email protected] (M.J. Landzberg)
REFERENCES 1. Zipes DP, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine. Philadelphia: W.B. Saunders; 2005. 2. Coronel R, de Groot JR, van Lieshout JJ. Defining heart failure. Cardiovasc Res 2001;50:419–22. 3. Rodriguez FH 3rd, Moodie DS, Parekh DR, et al. Outcomes of heart failure-related hospitalization in adults with congenital heart disease in the United States. Congenit Heart Dis 2012. 4. Rodriguez FH, Marelli AJ. The Epidemiology of heart failure in adults with congenital heart disease. Heart Fail Clin 2013;10(1):1–7. 5. Gewillig M, Goldberg DJ. Failure of the Fontan circulation. Heart Fail Clin 2013;10(1):105–16. 6. Opotowsky AR, Landzberg MJ, Beghetti M. The exceptional and far-flung manifestations of heart failure in Eisenmenger syndrome. Heart Fail Clin 2013; 10(1):91–104. 7. Kovacs AH, Moons P. Psychosocial functioning and quality of life in adults with congenital heart disease and heart failure. Heart Fail Clin 2013;10(1):35–42. 8. Opotowsky AR, Siddiqi OK, D’Souza B, et al. Maternal cardiovascular events during childbirth among women with congenital heart disease. Heart 2012;98:145–51. 9. Tobler D, Fernandes SM, Wald RM, et al. Pregnancy outcomes in women with transposition of the great arteries and arterial switch operation. Am J Cardiol 2010;106:417–20. 10. Said SM, Dearani JA, Silversides CK, et al. Longerterm issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation. Cardiol Young 2011;21(Suppl 2):93–100. 11. Grewal J, Silversides CK, Colman JM. Pregnancy in women with heart disease: risk assessment and management of heart failure. Heart Fail Clin 2013; 10(1):117–30. 12. Ohuchi H, Diller GP. Biomarkers in adult congenital heart disease heart failure. Heart Fail Clin 2013; 10(1):43–56. 13. Burchill LJ, Mertens L, Broberg CS. Imaging for the assessment of heart failure in congenital heart disease: ventricular function and beyond. Heart Fail Clin 2013;10(1):9–22. 14. Book WM, Shaddy RE. Medical therapy in adults with congenital heart disease. Heart Fail Clin 2013; 10(1):167–78.
