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Providing medical care to a newborn with known or suspected heart disease can be daunting. Many practitioners are inclined to abdicate care to the pediatric cardiologist. This response is not only unnecessary, but also counterproductive to an integrated team approach to optimal care. An understanding of the pathophysiology of these conditions and the application of a few general principles will promote effective care and minimize the chance of iatrogenic misadventures. It is imperative that a concerted team approach is utilized, involving neonatology, cardiology, nursing, surgery, and anesthesiology. Effective and ongoing communication is essential for optimizing care and providing a uniform approach to the management of these complex medical patients.

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This chapter reviews the diagnosis and management of infants with heart failure, infective endocarditis, and intracardiac and intravascular thrombi. In addition, nutritional support of infants with heart disease, an important but often overlooked problem, is discussed. Initial evaluation and treatment of newborns with heart disease are discussed in Chapter 5. Although much of the focus in this chapter is on the heart failure syndrome in neonates, it should be noted that compared to the plethora of data available in adults, far less information is available regarding heart failure in newborn infants. The roles of physiologic and neurohormonal compensatory mechanisms have been studied largely in chronic compensated states in adult patients and mature animal models. The causes of heart failure in neonates differ from those in older age groups. Furthermore, it is likely that normal developmental changes during the neonatal period impact upon the nature and magnitude of the compensatory physiologic responses and the responses to therapy. Lastly, there are marked differences in the psychosocial aspects of chronic disease and the impact of heart failure on growth, development, and quality of life.

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Overview

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Heart failure in infancy is a syndrome that occurs as a consequence of the inability of the cardiovascular system to meet the metabolic and growth demands of the infant. It is a common feature of congenital heart disease presenting symptomatically in neonates. Heart failure in neonates and infants is most commonly caused by structural defects that result in decreased systemic output. The most common conditions associated with heart failure in infants are those in which there is a dominant left-to-right shunt with excessive pulmonary blood flow, reduced systemic blood flow, heart failure, and failure to thrive. Heart failure in neonates can also result from any structural defect that results in obstruction of systemic blood flow (eg, severe aortic stenosis) or by myocardial dysfunction (eg, dilated cardiomyopathy). Occasionally, heart failure occurs in situations in which the heart is structurally normal, but systemic output is very high and is associated with abnormal distribution of flow (eg, a large arteriovenous malformation), severe anemia, or excessive metabolic demands (eg, neonatal thyrotoxicosis).

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Pathophysiology

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The development and progression of heart failure results from a complex interplay of hemodynamic and neurohormonal factors. As illustrated in ...

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