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This chapter reviews congenital and acquired heart disease in children. The section on congenital heart defects begins with a review of fetal and neonatal cardiac physiology, followed by a discussion of specific lesions and their diagnosis and management organized by clinical presentation. A brief discussion of pediatric murmurs follows, and the section concludes with a discussion of common surgical procedures for repairing congenital heart defects and associated complications.

The section on acquired heart disease in children reviews inflammatory and infectious disorders and cardiomyopathies, of which the most important is hypertrophic cardiomyopathy.

Fetal Circulation

Fetal circulation involves a number of shunts to bypass the liquid-filled lungs, which are incapable of providing oxygen to circulating blood. Blood oxygenated by the maternal lungs passes through the placenta via the umbilical vein to the fetus. Roughly half of the blood flow passes through the liver and half through the ductus venosus to the inferior vena cava, where it mixes with deoxygenated fetal blood returning from the lower body. The inferior vena cava enters the right atrium, where deoxygenated blood returning from the upper body and head via the superior vena cava mixes with that from the inferior vena cava. Blood from the right atrium travels in one of three routes: A portion passes through the foramen ovale into the left atrium, where it subsequently travels to the left ventricle, through the aorta, and into the vessels supplying the fetal head and upper extremities; most of the remainder enters the right ventricle and passes into the pulmonary artery, where the majority is shunted away from the lungs through the ductus arteriosus connecting the pulmonary artery with the aorta; and a small amount of blood travels to the lungs to provide oxygen and nutrients to support fetal lung growth.1

Transitional Circulation

The newborn's lungs expand and become air filled with gradual reabsorption of fetal lung fluid. This increases the partial pressure of arterial oxygen (PaO2) of blood flowing through the newborn lung, which in turn mediates a cascade of events that completes the transition to adult circulatory patterns. Flow through the umbilical arteries ceases, and the venous flow through the cord slows and then stops. Pulmonary vascular resistance falls, and pulmonary blood flow increases (pulmonary vascular resistance continues to fall with increases in blood flow over the first 30 to 45 days of extrauterine life). The ductus venosus and ductus arteriosus close, and decreased pulmonary arterial resistance coupled with increased systemic resistance create increased blood flow through the atria. Left atrial pressure exceeds right atrial pressure, which leads to closure of the foramen ovale.

Neonatal Cardiac Physiology

Because neonates and small children have relatively noncompliant ventricular walls, they cannot increase stroke volume but rely on changes in heart rate to adjust cardiac output. Thus, sinus tachycardia is usually the first response to stress in infants ...

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