Heart Failure in Adult Congenital Heart Disease
Fore w ord Adults with Congenital Heart D i s e a s e : G ro w i n g P a i n s
Mandeep R. Mehra, MD
Javed Butler, MD, MPH
Adults with congenital heart disease now outnumber children with an estimated prevalence of 3 per 1000. Terrific advances in surgical correction of anatomic defects, widespread recognition of avoiding teratogenicity, improved rates of maternal vaccination, prevention of illnesses during pregnancy, and improvements in more accurate clinical recognition of congenital heart defects are some principal reasons for this growing but welcome imbalance. Indeed, this group of patients exhibits a unique complexity coupled with the layering of manifestations of more traditional riskmarker-driven and metabolically sustained cardiac perturbations such as hypertension, hyperlipidemia, obesity, coronary heart disease, and unique arrhythmias. This has led to the resurgence of a specialty in its own right, one that requires a skill set that spans pediatric and adult medicine, singular experience in disease identification, appreciation of altered anatomical-physiological interactions, and knowledge of therapeutics often found only in select teams based at large academically oriented institutions. The adult with congenital heart disease may represent a daunting task to the general clinician but epitomizes a versatile opportunity for mechanistic understanding of disease guided by incisive appreciation for unique therapeutic challenges. Take the right ventricle in congenital heart disease, for example. This chamber can be anatomically a systemic ventricle or a single ventricle, or it
can be subject to uniquely abnormal loads or electrical stimulation. These facets all allow the physiologist and clinician to learn more about the pathophysiology of the ventricle, allowing for possible evolution of therapeutic stances in areas where the right heart is diseased due to noncongenital cause. The Fontan circulation, which represents a hemodynamic compromise, is interesting to observe in its natural history. As it fails with age and loses hemodynamic efficiency, a model of right heart failure emerges that challenges the clinician critically to find ways to support the circulation despite the absence of a contractile right-sided pump. An arrhythmia, which may be well tolerated in structurally normal hearts, can be hemodynamically devastating and prognostic. Such rhythms may challenge the clinician to develop and employ advanced techniques in catheter ablation or robotic magnetic navigation to treat the substrate. More recently, the subcutaneous defibrillator has become available in selected patients with congenital heart disease in whom transvenous lead implantation is unachievable or contraindicated. Finally, the development of pulmonary arterial hypertension is a common complication of congenital heart disease and at extremes can manifest as the Eisenmenger syndrome. Observations in the natural history of survival of Eisenmenger syndrome compared with common varieties of pulmonary arterial hypertension have taught us that prognosis is vastly determined by the expression of right heart
Heart Failure Clin 10 (2014) xiii–xiv http://dx.doi.org/10.1016/j.hfc.2013.11.001 1551-7136/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
heartfailure.theclinics.com
Consulting Editors
xiv
Foreword failure and right ventricular adaptation rather than the hemodynamic load itself. In this issue of Heart Failure Clinics, we see an extraordinary constellation of well-constructed topics that include the breadth and depth of knowledge in adults with congenital heart disease. These articles take us on an extensive journey ranging from the pathophysiology, to the epidemiology and the anatomic, physiological, and electrical aberrations that signal progressive disease. Furthermore, the state of the art in surgical and interventional techniques is discussed, including the role of end-stage therapy as with mechanical circulatory support and transplantation. Yet, the editors do not ignore the importance of psychological and sociodynamic factors that can influence adherence and access to as well as outcomes from treatment in this unique subset of patients.
All in all, this is a masterful compendium on heart failure in the setting of adults with congenital heart disease. Mandeep R. Mehra, MD BWH Heart and Vascular Center Brigham and Women’s Hospital 75 Francis Street A Building, 3rd Floor, Room AB324 Boston, MA 02115, USA Javed Butler, MD, MPH Emory Clinical Cardiovascular Research Institute 1462 Clifton Road NE, Suite 504 Atlanta, GA 30322, USA E-mail addresses: [email protected] (M.R. Mehra) [email protected] (J. Butler)
Fore w ord Adults with Congenital Heart D i s e a s e : G ro w i n g P a i n s
Mandeep R. Mehra, MD
Javed Butler, MD, MPH
Adults with congenital heart disease now outnumber children with an estimated prevalence of 3 per 1000. Terrific advances in surgical correction of anatomic defects, widespread recognition of avoiding teratogenicity, improved rates of maternal vaccination, prevention of illnesses during pregnancy, and improvements in more accurate clinical recognition of congenital heart defects are some principal reasons for this growing but welcome imbalance. Indeed, this group of patients exhibits a unique complexity coupled with the layering of manifestations of more traditional riskmarker-driven and metabolically sustained cardiac perturbations such as hypertension, hyperlipidemia, obesity, coronary heart disease, and unique arrhythmias. This has led to the resurgence of a specialty in its own right, one that requires a skill set that spans pediatric and adult medicine, singular experience in disease identification, appreciation of altered anatomical-physiological interactions, and knowledge of therapeutics often found only in select teams based at large academically oriented institutions. The adult with congenital heart disease may represent a daunting task to the general clinician but epitomizes a versatile opportunity for mechanistic understanding of disease guided by incisive appreciation for unique therapeutic challenges. Take the right ventricle in congenital heart disease, for example. This chamber can be anatomically a systemic ventricle or a single ventricle, or it
can be subject to uniquely abnormal loads or electrical stimulation. These facets all allow the physiologist and clinician to learn more about the pathophysiology of the ventricle, allowing for possible evolution of therapeutic stances in areas where the right heart is diseased due to noncongenital cause. The Fontan circulation, which represents a hemodynamic compromise, is interesting to observe in its natural history. As it fails with age and loses hemodynamic efficiency, a model of right heart failure emerges that challenges the clinician critically to find ways to support the circulation despite the absence of a contractile right-sided pump. An arrhythmia, which may be well tolerated in structurally normal hearts, can be hemodynamically devastating and prognostic. Such rhythms may challenge the clinician to develop and employ advanced techniques in catheter ablation or robotic magnetic navigation to treat the substrate. More recently, the subcutaneous defibrillator has become available in selected patients with congenital heart disease in whom transvenous lead implantation is unachievable or contraindicated. Finally, the development of pulmonary arterial hypertension is a common complication of congenital heart disease and at extremes can manifest as the Eisenmenger syndrome. Observations in the natural history of survival of Eisenmenger syndrome compared with common varieties of pulmonary arterial hypertension have taught us that prognosis is vastly determined by the expression of right heart
Heart Failure Clin 10 (2014) xiii–xiv http://dx.doi.org/10.1016/j.hfc.2013.11.001 1551-7136/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
heartfailure.theclinics.com
Consulting Editors
xiv
Foreword failure and right ventricular adaptation rather than the hemodynamic load itself. In this issue of Heart Failure Clinics, we see an extraordinary constellation of well-constructed topics that include the breadth and depth of knowledge in adults with congenital heart disease. These articles take us on an extensive journey ranging from the pathophysiology, to the epidemiology and the anatomic, physiological, and electrical aberrations that signal progressive disease. Furthermore, the state of the art in surgical and interventional techniques is discussed, including the role of end-stage therapy as with mechanical circulatory support and transplantation. Yet, the editors do not ignore the importance of psychological and sociodynamic factors that can influence adherence and access to as well as outcomes from treatment in this unique subset of patients.
All in all, this is a masterful compendium on heart failure in the setting of adults with congenital heart disease. Mandeep R. Mehra, MD BWH Heart and Vascular Center Brigham and Women’s Hospital 75 Francis Street A Building, 3rd Floor, Room AB324 Boston, MA 02115, USA Javed Butler, MD, MPH Emory Clinical Cardiovascular Research Institute 1462 Clifton Road NE, Suite 504 Atlanta, GA 30322, USA E-mail addresses: [email protected] (M.R. Mehra) [email protected] (J. Butler)
