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Pediatric heart failure: current state and future possibilities.

Rossano JW, Jang GY - Korean Circ J (2015)

Bottom Line: The condition is associated with a high rate of morbidity and mortality and places a significant burden on families of affected children and to society as a whole.Current medical therapy is taken largely from the management of heart failure in adults, though clear survival benefit of these medications are lacking.There is an ongoing investigation to improve outcomes in high-risk populations, such as small infants and those with complex congenital heart disease, including patients with functionally univentricular hearts.

View Article: PubMed Central - PubMed

Affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

ABSTRACT
Heart failure is a complex pathophysiological syndrome that can occur in children from a variety of diseases, including cardiomyopathies, myocarditis, and congenital heart disease. The condition is associated with a high rate of morbidity and mortality and places a significant burden on families of affected children and to society as a whole. Current medical therapy is taken largely from the management of heart failure in adults, though clear survival benefit of these medications are lacking. Ventricular assist devices (VADs) have taken an increasingly important role in the management of advanced heart failure in children. The predominant role of these devices has been as a bridge to heart transplantation, and excellent results are currently achieved for most children with cardiomyopathies. There is an ongoing investigation to improve outcomes in high-risk populations, such as small infants and those with complex congenital heart disease, including patients with functionally univentricular hearts. Additionally, there is an active investigation and interest in expansion of VADs beyond the predominant utilization as a bridge to a heart transplant into ventricular recovery, device explant without a heart transplantation (bridge to recovery), and placement of devices without the expectation of recovery or transplantation (destination therapy).

No MeSH data available.


Related in: MedlinePlus

Freedom from death or transplantation for patients with pure dilated cardiomyopathy. Retrospective cohort diagnosed 1990-1995; Prospective cohort diagnosed 1996-2002. Adopted from Towbin JA, et al. with permission from the publisher.17)
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Figure 2: Freedom from death or transplantation for patients with pure dilated cardiomyopathy. Retrospective cohort diagnosed 1990-1995; Prospective cohort diagnosed 1996-2002. Adopted from Towbin JA, et al. with permission from the publisher.17)

Mentions: The number of children with chronic heart failure is difficult to ascertain, in part due to the diverse nature of diseases that can lead to heart failure. There are many patients at risk for heart failure from a number of disorders, including congenital heart disease, myocarditis, cardiomyopathy, metabolic disorders, and effects of medications (e.g., anthracyclines). However, not all patients at risk of heart failure will develop heart failure. The incidence of dilated cardiomyopathy form large population-based studies in the United States and Australia range from 0.57 to 0.73 per 100000 children per year.13)14) Not all of these patients will have heart failure at the time of diagnosis, and this number likely underestimates the true incidence as there are likely those with the disease that have not yet been identified. In a prospective study from the United Kingdom, the annual incidence of new-onset heart failure from heart muscle disease was found to be 0.87 per 100000 children who were less sixteen years old.15) Only 66% of these patients were alive or had no transplant one year later.15) This high risk of death or transplant has been confirmed in multiple single center and multi-center reports, with the five-year transplant free survival ranging from 50 to 65% (Fig. 2).16)17)18) The risk for death and transplant is greatest after the initial diagnosis. After one-year, the risk drops significantly with reported events in the range of 1% per year.19) However, not all patients deteriorate, and a significant minority of patients will have a meaningful recovery of ventricular function.20)21) Clearly, further study is needed to understand the worldwide incidence and prevalence of heart failure to understand the true burden of disease and the impact on society.


Pediatric heart failure: current state and future possibilities.

Rossano JW, Jang GY - Korean Circ J (2015)

Freedom from death or transplantation for patients with pure dilated cardiomyopathy. Retrospective cohort diagnosed 1990-1995; Prospective cohort diagnosed 1996-2002. Adopted from Towbin JA, et al. with permission from the publisher.17)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4310974&req=5

Figure 2: Freedom from death or transplantation for patients with pure dilated cardiomyopathy. Retrospective cohort diagnosed 1990-1995; Prospective cohort diagnosed 1996-2002. Adopted from Towbin JA, et al. with permission from the publisher.17)
Mentions: The number of children with chronic heart failure is difficult to ascertain, in part due to the diverse nature of diseases that can lead to heart failure. There are many patients at risk for heart failure from a number of disorders, including congenital heart disease, myocarditis, cardiomyopathy, metabolic disorders, and effects of medications (e.g., anthracyclines). However, not all patients at risk of heart failure will develop heart failure. The incidence of dilated cardiomyopathy form large population-based studies in the United States and Australia range from 0.57 to 0.73 per 100000 children per year.13)14) Not all of these patients will have heart failure at the time of diagnosis, and this number likely underestimates the true incidence as there are likely those with the disease that have not yet been identified. In a prospective study from the United Kingdom, the annual incidence of new-onset heart failure from heart muscle disease was found to be 0.87 per 100000 children who were less sixteen years old.15) Only 66% of these patients were alive or had no transplant one year later.15) This high risk of death or transplant has been confirmed in multiple single center and multi-center reports, with the five-year transplant free survival ranging from 50 to 65% (Fig. 2).16)17)18) The risk for death and transplant is greatest after the initial diagnosis. After one-year, the risk drops significantly with reported events in the range of 1% per year.19) However, not all patients deteriorate, and a significant minority of patients will have a meaningful recovery of ventricular function.20)21) Clearly, further study is needed to understand the worldwide incidence and prevalence of heart failure to understand the true burden of disease and the impact on society.

Bottom Line: The condition is associated with a high rate of morbidity and mortality and places a significant burden on families of affected children and to society as a whole.Current medical therapy is taken largely from the management of heart failure in adults, though clear survival benefit of these medications are lacking.There is an ongoing investigation to improve outcomes in high-risk populations, such as small infants and those with complex congenital heart disease, including patients with functionally univentricular hearts.

View Article: PubMed Central - PubMed

Affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

ABSTRACT
Heart failure is a complex pathophysiological syndrome that can occur in children from a variety of diseases, including cardiomyopathies, myocarditis, and congenital heart disease. The condition is associated with a high rate of morbidity and mortality and places a significant burden on families of affected children and to society as a whole. Current medical therapy is taken largely from the management of heart failure in adults, though clear survival benefit of these medications are lacking. Ventricular assist devices (VADs) have taken an increasingly important role in the management of advanced heart failure in children. The predominant role of these devices has been as a bridge to heart transplantation, and excellent results are currently achieved for most children with cardiomyopathies. There is an ongoing investigation to improve outcomes in high-risk populations, such as small infants and those with complex congenital heart disease, including patients with functionally univentricular hearts. Additionally, there is an active investigation and interest in expansion of VADs beyond the predominant utilization as a bridge to a heart transplant into ventricular recovery, device explant without a heart transplantation (bridge to recovery), and placement of devices without the expectation of recovery or transplantation (destination therapy).

No MeSH data available.


Related in: MedlinePlus