Medical Management of Chronic Heart Failure in Children

Abstract

Chronic heart failure (CHF) in children occurs mostly as a result of systolic dysfunction of the systemic ventricle or of congenital defects leading to large left-to-right shunts and pulmonary overcirculation. The ensuing symptoms and signs are similar in both cases, and include respiratory distress, poor feeding and growth, and hepatic congestion. Grading the severity of the symptoms accurately and reproducibly is important for studying CHF and the response to therapy. The Ross classification for young children and the New York Heart Association classification for older children are frequently utilized for such grading. The standard therapy for CHF in children consists of diuretics, to reduce cardiac preload and improve symptoms, and the maximization of nutritional support. The role of digoxin in treating CHF in children is controversial, especially regarding those children with pulmonary overcirculation where the function of the systemic ventricle is usually well preserved. As the importance of neurohormonal changes in the pathogenesis of worsening CHF is elucidated, newer medications aimed at counteracting such changes are becoming more important in the medical therapy of CHF in children. ACE inhibitors improve function and survival in adults with CHF, and they probably do the same in children with systemic ventricular dysfunction. It is less clear how effective they are in pulmonary overcirculation, but patients with high flow and low pulmonary resistance are most likely to benefit. In infants receiving treatment with ACE inhibitors, it is necessary to monitor for renal insufficiency or renal failure. beta-Adrenoceptor blockade has also been established as an effective therapy for adults with CHF with beneficial effects on survival and left ventricular function. While data for the pediatric population are limited, early studies suggest that beta-adrenoceptor antagonists (beta-blockers) may work well in infants and children with CHF. Caution must be used by starting treatment with very low dosages of beta-blockers and gradually increasing to the desired goals with close monitoring of blood pressure and heart rate. It is clear that larger multicenter trials are crucial to our ability to provide the most appropriate treatment for children with CHF. The demand for effective medical treatment will increase as more patients with palliated single ventricles survive surgery and then develop CHF from dysfunction of a hypertrophic and dilated single ventricle.

Indications for Heart Transplantation in Pediatric Heart Disease

“…[143][144][145] Delayed growth with retarded bone age is commonly observed in pediatric heart transplantation candidates. 146,147 Pediatric transplant recipients have generally exhibited normal growth rates after transplantation, but their absolute heights and weights tend to be in the lower range of normal.…”

Association of Digoxin With Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use Dataset

“…Over the last 2 decades, however, digoxin has lost favor with many pediatric cardiologists, with only 28% of patients in the National Pediatric Cardiology Quality Improvement Collaborative (NPC‐QIC) discharged home on the medicine . Reasons for the decreased use of digoxin include question about its mechanism of action, concerns about toxicity, and availability of other pharmacological options . However, it has recently been suggested that digoxin might play a role in reducing interstage mortality …”

“…14 Reasons for the decreased use of digoxin include question about its mechanism of action, concerns about toxicity, and availability of other pharmacological options. 15 However, it has recently been suggested that digoxin might play a role in reducing interstage mortality. 16 The objective of our study was to evaluate the association of outpatient digoxin use with interstage mortality in infants with single ventricle congenital heart disease in a multicenter study using data from the Pediatric Heart Network Single Ventricle Reconstruction (SVR) trial.…”

Nutrition Implications of Heart Failure and Heart Transplantation in Children With Dilated Cardiomyopathy

“…16 Tube feeding is often necessary to provide adequate energy for growth. The Ross classification of HF uses growth and responses to feeding as criteria to describe degree of HF 12 (Table 1).…”