Heart Failure in Children: Etiology and Treatment Joseph W. Rossano, MD, and Robert E. Shaddy, MD
A
lthough much of the care of pediatric patients with heart disease has focused on the treatment and palliation of various forms of congenital heart disease, there is increasing recognition of the importance of heart failure in child health.1 Many children with heart disease are at risk for developing heart failure, and heart failure can occur in the context of other diseases such as sepsis, cancer, and inflammatory diseases. The diagnosis and treatment of heart failure in children has emerged during the past decade as a subspecialty field within cardiology, with heart failure specialists being trained at many centers throughout the world. As the field has expanded rapidly over time, this review will focus on the epidemiology of pediatric heart failure, the diagnosis and risk-stratification of patients with heart failure, advanced therapies for heart failure, and new paradigms for management.
Epidemiology Heart failure is defined as a clinical and pathophysiologic syndrome that results from any structural or functional impairment of ventricular filling or ejection of blood.2 There is no single diagnostic test that establishes the diagnosis of heart failure; rather, it remains a clinical diagnosis with characteristic signs and symptoms resulting from a combination of circulatory, neurohormonal, and molecular abnormalities.1 Even though it is estimated that there are >5 million adults with heart failure, the prevalence of heart failure in children is not known.3 This is in part due to the diverse diseases that lead to heart failure including simple and complex forms of congenital heart diseases, cardiomyopathies, rhythm abnormalities, and acquired conditions such as myocarditis and Kawasaki disease.4-6 In the US, there are currently >14 000 pediatric heart failure–related hospitalizations annually, which corresponds to 14-18 hospitalizations per 100 000 children.4 The majority of these children have some form of congenital heart disease, although a significant number have a form of cardiomyopathy or myocarditis. This is a disease state associated with significant morbidity and mortality.7 The overall hospital mortality is 7%, and because the hospital mortality for children is very low, children whose hospitalization is complicated by heart failure have a >20-fold increase in the risk of death.4 Patients hospitalized with heart failure are at risk for multiple morbidities including respiratory failure, renal
ACE BNP ECMO VAD
Angiotensin-converting enzyme B-type natriuretic peptide Extracorporeal membrane oxygenation Ventricular assist device
failure, and sepsis.4,7,8 Many of these morbidities are associated with a striking increase in the risk of death (Figure 1). It is unknown how many children have heart failure outside of the hospital. The annual incidence of cardiomyopathies has been reported as 1.1-1.2 per 100 000 children, with the peak incidence occurring in infancy (7.8-8.3 per 100 000 per year).9,10 Although these patients are at risk for heart failure, not all will develop heart failure. Andrews et al11 reported that new-onset heart failure due to heart muscle disease occurred at a rate of 0.87 per 100 000 children aged
A
lthough much of the care of pediatric patients with heart disease has focused on the treatment and palliation of various forms of congenital heart disease, there is increasing recognition of the importance of heart failure in child health.1 Many children with heart disease are at risk for developing heart failure, and heart failure can occur in the context of other diseases such as sepsis, cancer, and inflammatory diseases. The diagnosis and treatment of heart failure in children has emerged during the past decade as a subspecialty field within cardiology, with heart failure specialists being trained at many centers throughout the world. As the field has expanded rapidly over time, this review will focus on the epidemiology of pediatric heart failure, the diagnosis and risk-stratification of patients with heart failure, advanced therapies for heart failure, and new paradigms for management.
Epidemiology Heart failure is defined as a clinical and pathophysiologic syndrome that results from any structural or functional impairment of ventricular filling or ejection of blood.2 There is no single diagnostic test that establishes the diagnosis of heart failure; rather, it remains a clinical diagnosis with characteristic signs and symptoms resulting from a combination of circulatory, neurohormonal, and molecular abnormalities.1 Even though it is estimated that there are >5 million adults with heart failure, the prevalence of heart failure in children is not known.3 This is in part due to the diverse diseases that lead to heart failure including simple and complex forms of congenital heart diseases, cardiomyopathies, rhythm abnormalities, and acquired conditions such as myocarditis and Kawasaki disease.4-6 In the US, there are currently >14 000 pediatric heart failure–related hospitalizations annually, which corresponds to 14-18 hospitalizations per 100 000 children.4 The majority of these children have some form of congenital heart disease, although a significant number have a form of cardiomyopathy or myocarditis. This is a disease state associated with significant morbidity and mortality.7 The overall hospital mortality is 7%, and because the hospital mortality for children is very low, children whose hospitalization is complicated by heart failure have a >20-fold increase in the risk of death.4 Patients hospitalized with heart failure are at risk for multiple morbidities including respiratory failure, renal
ACE BNP ECMO VAD
Angiotensin-converting enzyme B-type natriuretic peptide Extracorporeal membrane oxygenation Ventricular assist device
failure, and sepsis.4,7,8 Many of these morbidities are associated with a striking increase in the risk of death (Figure 1). It is unknown how many children have heart failure outside of the hospital. The annual incidence of cardiomyopathies has been reported as 1.1-1.2 per 100 000 children, with the peak incidence occurring in infancy (7.8-8.3 per 100 000 per year).9,10 Although these patients are at risk for heart failure, not all will develop heart failure. Andrews et al11 reported that new-onset heart failure due to heart muscle disease occurred at a rate of 0.87 per 100 000 children aged
