Respiratory distress

When a newborn or a young infant is in respiratory distress, do not assume that the underlying problem is primarily respiratory. A child whose problem is primarily cardiac may present with pulmonary symptoms or secondary pulmonary complications, such as an infection.

For our purposes, two types of respiratory distress can be defined: (1) tachypnea, or abnormally rapid respirations, and (2) dyspnea, or difficulty breathing.

Cyanotic heart lesions or lesions involving low cardiac output may be associated with a compensatory rapid respiratory rate, particularly on exertion, because of diminished peripheral oxygenation. Children with cyanotic lesions often are tachypneic without displaying increased work of breathing (i.e., dyspnea).

Left ventricular (LV) failure from any cause results in a high end-diastolic pressure in the left ventricle and elevated pulmonary venous pressure. This in turn causes a higher back pressure in the pulmonary vessels and transudation of fluid into the pulmonary interstitial spaces, making the lungs less compliant. The child then works harder to breathe and becomes dyspneic. To assist with the increased work required for breathing, the accessory muscles come into use, and subcostal indrawing is observed. Respirations become rapid. The child’s wet, stiff lungs are very susceptible to secondary infection, which may be the reason medical attention initially is sought for the patient.

Fatigue, excessive perspiration, and poor weight gain

In young infants, metabolic demands are usually greatest during feedings. Thus the infant with poor peripheral oxygenation as a result of low cardiac output tires easily during feeding (the equivalent of exercise in older children). Because of fatigue, the infant is unable to take a full feeding. In addition, rapid respiration diminishes the time available for swallowing. This combination of factors results in failure to gain weight. In the baby with a large left-to-right shunt, such as a VSD, the process is exaggerated by the higher caloric needs of an overworked myocardium. Increased sympathetic activity causes excessive perspiration, which often is a valuable diagnostic feature.

It also is important to ask about the duration and quantity of feedings. Feeding usually is accomplished within 20 minutes, which means that the mother’s breast usually should feel empty by then or the bottle should be finished. In a bottle-fed baby, the amount of formula taken per feed and how many times per day the baby feeds are important details that may help you understand the baby’s difficulties and can be used in guiding future treatment.

Squatting

Parents of older children with certain cyanotic heart defects, especially tetralogy of Fallot, may offer the observation that when their youngster tires, he or she assumes a squatting position. Squatting helps increase systemic oxygen saturation by decreasing the amount of right-to-left shunting. These days squatting is a very uncommon symptom/sign in the developed world because most children with conditions for which squatting helps have their lesions fully repaired long before they are able to walk.

Cyanosis

Parents frequently report that their children turn blue. On further inquiry, this phenomenon turns out to be blueness of the hands and feet only, or blueness around the lips. If the lips themselves remain pink, all of these events represent peripheral cyanosis or acrocyanosis. In some disease states, this phenomenon can represent a condition in which there is decreased peripheral circulation as a result of poor ventricular function, although children with that type of problem generally look unwell in other ways. Much more commonly, acrocyanosis represents immaturity of the autonomic nervous system, causing peripheral vasoconstriction and slower peripheral circulation as a result. Consequently, acrocyanosis in a child who is well should be considered benign.

In contrast, central cyanosis occurs when more than 5 g/dL of deoxygenated hemoglobin is present in the blood. This condition is most commonly manifested as blueness or sometimes as excessive redness or as a purple tinge of the lips and tongue. Although central cyanosis can come from a variety of cardiac, respiratory, neurologic (hypoventilation), or hematologic causes, its presence is always abnormal.

Hypercyanotic spells

In some forms of congenital heart disease, the degree of cyanosis worsens periodically and can be quite profound, to the point of causing loss of consciousness. These episodes are known as hypercyanotic spells and occur classically in children with cyanotic congenital heart disease that involves narrowing of the infundibulum (the subpulmonary outflow tract) and a VSD, classically tetralogy of Fallot. This clinical phenomenon is caused by either: (1) an increase in pulmonary resistance (resulting from either a subpulmonary infundibular muscle spasm or an increase in peripheral pulmonary vascular resistance) or (2) a fall in systemic vascular resistance. Both mechanisms result in a relaive increase in the restriction of blood flow out of the right ventricular outflow and a subsequent increase in right-to-left shunting across the VSD. Spells often are precipitated by crying. After patients with hypercyanotic spells have been stabilized, their condition should always be discussed with a pediatric cardiologist before they are discharged from the hospital or emergency department.

Angina

Angina is rare but not unknown in infants and children; it can occur in persons with severe aortic stenosis or possibly pulmonary stenosis because of associated myocardial ischemia. It also may occur in persons with certain forms of congenital heart disease after surgical repair that involved manipulation of the coronary arteries. Angina may be seen in patients with Kawasaki disease in whom coronary stenoses or thromboses develop within coronary aneurysms. Angina has been recognized in infants with very rapid paroxysmal tachycardias and in those with an aberrant left coronary artery. Rarely, children with homozygous forms of familial hypercholesterolemia can have angina as a result of atherosclerotic coronary narrowing. Chest pain in this rare group of patients must always be taken seriously.

The manifestations of angina in young children are varied but may include classic chest pain or simply periodic irritability associated with sweating or pallor. When chest pain in children is invariably associated with exercise, angina should be a consideration.

Peripheral edema

Infants and young children differ strikingly from adults regarding the development of peripheral edema in the presence of congestive heart failure. Pretibial and presacral edema are late developments in a child with congestive circulatory failure. This phenomenon is believed to be due to a difference in tissue turgor. When peripheral edema resulting from heart failure does develop in an infant, it first appears periorbitally and usually is preceded by other manifestations, such as tachypnea, tachycardia, dyspnea, and liver enlargement.

Orthopnea

Unlike in adults, orthopnea is not obvious in the infant with heart failure, even when tachypnea, dyspnea, hepatomegaly, and the radiographic findings of pulmonary edema are present. In the adult, orthopnea is a symptom; in the infant, it is a sign.

Age of onset

Family history

Genetic factors are increasingly recognized to be important in the etiology of congenital heart disease. Reports exist of as many as three children of the same parents being affected with VSD, PDA, and aortic stenosis. These are exceptions, however, because the tendency for congenital heart disease to occur in a child when one parent has a congenital heart lesion is only mildly increased (2% to 4% in most cases, compared with about 1% in the general population). The genetic tendency for a congenital heart lesion also exists in siblings, particularly for left-sided lesions such as bicuspid aortic valve. In certain syndromes or hereditary disorders with cardiac implications, however, the genetic tendency for recurrence is high. For example, Marfan syndrome is inherited as an autosomal dominant gene, and thus there is a 50% chance of recurrence. The same is true in 22q.11 deletion syndromes, which are highly associated with conotruncal cardiac defects. Consanguinity is a significant causative factor in congenital heart disease, but the tendency varies between families and with the type of heart lesion.

In summary, a search for evidence of congenital heart disease in the family is important. However, when there is a history of congenital heart disease in one parent or in a previous child, counseling regarding the risk of recurrence is best done by a geneticist.

Prenatal history

Because the cause of congenital heart disease is multifactorial, known contributory factors should be sought in the prenatal history, including:

In most instances, however, no specific contributory factors can be identified.

History of delivery

An important but infrequent cardiovascular problem in newborns is persistent pulmonary hypertension, which may cause central cyanosis, myocardial dysfunction, or both. This condition often is preceded by a difficult delivery and meconium aspiration. It is unlikely to occur after an uncomplicated delivery. Clinical differentiation of pulmonary hypertension from congenital heart disease may be difficult and usually requires echocardiography.

Determining the gestational age of a newborn also is very important because persistent patency of the ductus arteriosus is common in premature infants.

Initial assessment

When you start the examination, don’t undress the baby right away. Begin with something relatively non-threatening, such as an examination of the hands. The baby usually allows you to examine the palmar creases and to check the nail beds and muscle tone without much protest. Then feel the brachial pulses for rate, rhythm, and volume, the last being the most important. Feel this pulse in every baby you examine to learn the difference between a normal and abnormal brachial pulse. An abnormally “full” pulse suggests a PDA or aortic insufficiency. In premature infants, palpable palmar pulses (from the palmar arch) indicate the same thing. A shallow, slow-rising brachial pulse suggests LV outflow tract obstruction. Do not feel for the femoral pulses yet.

Looking for central cyanosis

Because the most pressing clinical problems are congestive heart failure and cyanosis, decide early in the examination whether central cyanosis is present. Because this determination is not always easy, you may find an experienced nurse’s opinion invaluable. Many normal newborns have a deep plethoric appearance as a result of a transiently high hemoglobin concentration, particularly if there was a delay in clamping the umbilical cord. Plethora is not as obvious in the mucous membranes, so look carefully in the baby’s mouth. Deep pressure on the skin may help, because the blanched area does not pink up as quickly in infants with central cyanosis. Many normal infants exhibit a generalized mottling, particularly after being bathed (see Fig. 4-2); this condition is called cutis marmorata (literally, marbled skin) and is not pathologic. It also is common in children with Down syndrome. Observe the effect of crying. Invariably, central cyanosis resulting from cardiac disease increases during crying, but do not make the baby cry until after you have listened to the heart.

It is important to be certain of the presence of cyanosis; this determination may require a second examination and often is not at all obvious. When you are examining a child in the hospital, measuring the blood oxygen saturation will help greatly, as may observing the effect of having the child breathe 100% oxygen. Finally, remember that cyanosis may occur in only one part of the body; for example, the lower part of the body may show cyanosis while the upper part remains pink. This condition can occur with an aortic coarctation or persistent pulmonary hypertension when there is associated right-to-left shunting through a PDA.

Clinical manifestations of heart failure

When low cardiac output and high pulmonary venous pressure cause sufficient hemodynamic disturbance to produce clinical manifestations, cardiac enlargement is invariably present. Whether the disturbance involves primarily the left or the right ventricle, the left side of the thorax becomes prominent anteriorly (Fig. 10–1). Although this prominence may not be evident in the first month of life, it certainly will be evident by the age of 3 months. When respiratory distress due to heart failure has been present for 2 months or longer, the greater diaphragmatic contractions during respiration may produce a sulcus in the lower thorax, with outward flaring of the inferior rib cage edge. Therefore, look for a sulcus, left-sided chest prominence, abnormal chest or abdominal movement, an increased respiratory rate, and subcostal indrawing.

FIGURE 10–1 Prominence of the left side of the chest in a 3-year-old with ventricular septal defect and moderate left-to-right shunt that is causing enlargement of both left and right ventricles.

Palpation

Now lay your prewarmed hand very gently on the infant’s chest, remembering that the heart may not be in its normal position. With the tips of the first and second fingers of your right hand, depress the thorax just left of the xiphoid process (Fig. 10–2). Your fingertips are now lying on the right ventricle. A faint impulse is allowable, but if the heart is enlarged, a definite forceful movement will be present. When you perform this maneuver repeatedly in normal infants, you soon will be able to tell the difference between normal and abnormal findings. This distinction will help you make a quick decision about whether the 6-week-old baby who presents with respiratory distress has a cardiac or a respiratory problem.

FIGURE 10–2 Press on the precordium to the left of the xiphoid process with the first and second digits of your right hand to detect enlargement of the right ventricle.

Now depress the thorax in the apical area. Prominence of the apical impulse is diagnostically less helpful in infants than in older children, except in rare instances, such as in tricuspid atresia in which the right ventricle is hypoplastic. Then palpate in the second interspace at the left sternal border, where a prominent pulmonary artery pulsation may be elicited. Finally, place one index finger carefully in the suprasternal notch (Fig. 10–3), searching first for an abnormal pulsation and then for a thrill. Then work in the opposite direction, searching for thrills and palpable sounds. At this point, you should have made a reasonable appraisal of the child’s cardiac dynamics.

FIGURE 10–3 Insert the index finger of your right hand deep in the suprasternal notch, searching first for pulsation and then for a thrill.

Liver size and position

Whether you are right- or left-handed, stand or sit on the baby’s right side to palpate for the liver. Use the tip of your right thumb and begin well down in the right lower quadrant of the abdomen, pressing inward and upward (Fig. 10–4). If the baby has just been fed, do not press very deeply. If the edge of the liver is soft, its margin may be difficult to detect; nevertheless, if the liver is enlarged, you should appreciate a sense of resistance as your thumb tip moves superiorly. If the edge is difficult to feel, use soft percussion, tapping the second digit of your left hand with the second digit of your right hand, beginning low in the right lower quadrant and placing the second digit of your left hand parallel to the liver edge (Fig. 10–5). You should be able to sense the change in the percussion note signifying the liver’s edge. Except in the presence of pulmonary hyperinflation, the edge of the liver normally should not be more than 1 to 2 cm below the costal margin.

FIGURE 10–4 Palpate the liver with movement of the tip of the thumb inward and cephalad, beginning low in the right lower quadrant of the abdomen.

FIGURE 10–5 Percuss for the liver edge, using soft percussion with the second digit of your right hand on the second digit of your left hand, which has been positioned parallel to the liver edge.

Finally, remember that the liver can be ectopic (i.e., on the left side or up in the thorax).

Auscultation

You need all the acoustic help you can get during cardiac auscultation, so be sure to turn off radios and televisions, close the door, and have everything and everyone be as quiet as possible. Cardiac auscultation is not easy, even in older cooperative patients, but coping with a restless baby with rapid cardiac and respiratory rates in a noisy nursery is a real trial. Having the child eat or giving him or her a pacifier may help. Hospital stethoscopes frequently are of poor quality, so find and use a good one.

Remember that the two main determinants of auscultatory proficiency are the fit of the earpieces and the quality (or at least the education!) of the gray matter between them.

Recognition of normal splitting of the second heart sound often is impossible when the heart rate is rapid. However, you should be able to assess the intensity or loudness of the second sound. Its intensity increases in the presence of pulmonary hypertension or when the aorta is anteriorly placed, as in transposition of the great vessels. Occasionally an ejection sound can be appreciated, which is an abnormal finding. Listen over the back for the murmur of coarctation and to both sides of the skull for the bruit of an intracranial arteriovenous malformation.

A detailed description of cardiac auscultation and the types of findings possible is contained in a subsequent section of this chapter. The following list contains a few dogmatic but valuable generalizations concerning auscultatory findings in young infants:

Palpating the femoral pulses

Palpation of the pulses calls for gentleness, persistence, and patience, so make yourself comfortable before you begin. First, remove the baby’s diaper and palpate for femoral pulsations. Many babies do not appreciate having people poke around in their groins and may cry, urinate, or both. Femoral pulses are particularly difficult to appreciate in obese babies; thus, do not rush into a diagnosis of coarctation of the aorta if you have difficulty feeling them. If the femoral pulses are not palpable in an asthenic baby, however, there is cause for concern (see Fig. 4-20). Now palpate both brachial pulses again. If you can detect good brachial pulses and are certain that the femoral pulses are absent or greatly depressed, listen to the heart before measuring the blood pressure and upsetting the baby. Listen particularly for a high-pitched blowing systolic murmur, which is best heard anteriorly below the left clavicle and can be heard clearly in the left axilla and back, medial to the scapula.

Blood pressure

Although measuring the systemic blood pressure can be a difficult task, you should try to do so. The normal systolic blood pressure of an infant is between 60 and 80 mm Hg in both the arm and the leg (Table 10–1). The four methods of measuring blood pressure are (1) auscultatory, (2) palpatory, (3) visual (flush), and (4) Doppler.

All methods require the use of an inflatable cuff. The first decision is to choose the size of the cuff; the size is of great importance because the measuring the pressure involves occlusion of the arterial pulse (or the brachial arterial pulse when the arm is used). If the cuff used is too small, greater pressure must be used to obliterate the pulse, and the blood pressure measured will be artificially high. Use a cuff that covers almost the full extent of the upper arm, with the elbow bent. Always have a full selection of cuff sizes available. The right arm should be chosen when possible because of the proximity of the LSCA to a coarctation.

For all methods of measuring blood pressure, first supinate the child’s arm to make the radial artery easily accessible. Apply the cuff, elevate the arm, and then inflate the cuff. Prior elevation of the arm (or opening and closing the hand) prevents the auscultatory gap phenomenon (Fig. 10–6). If this procedure is not followed, when you inflate the cuff and listen for Korotkoff sounds, as you decrease the pressure in the cuff you may hear a sound appear, then disappear, and then reappear as the pressure is further decreased. This phenomenon, known as the auscultatory gap, occurs because of increased vascular resistance distal to the cuff.

FIGURE 10–6 Supinate the patient’s hand and elevate the arm before expanding the sphygmomanometer cuff. This maneuver positions the radial artery properly and eliminates the auscultatory gap.

The conventional method of measuring blood pressure is the auscultatory method. The edge of the diaphragm of a warm stethoscope is placed under the inferior edge of the cuff, and the cuff is inflated. Listen for the Korotkoff sounds as the cuff pressure is decreased, watching the sphygmomanometer needle sphygmomanometer as you listen. The first sound you hear denotes the systolic level. As the cuff pressure is decreased further, an abrupt change in the intensity of the sound may be heard. If this change is detected, it is recorded as the diastolic level. Continue decreasing the cuff pressure, recording the disappearance of Korotkoff sounds, which is recorded as the diastolic level if no intensity change has been detected. Usually two or three recordings are made, elevating the arm before each attempt.

It may be impossible to measure the blood pressure by the auscultatory method, particularly when a baby does not cooperate. The palpatory method of measurement should then be attempted. Prior elevation of the arm is not required. The radial artery pulse is palpated, and the cuff is elevated until the pulse disappears. The cuff pressure is then decreased, and the level of systolic pressure is estimated by the time of the reappearance of the pulse. Only the systolic pressure can be measured by the palpatory method. A variation of this method involves detection of the appearance of the pulse with a manual Doppler probe held on the patient’s radial or brachial pulse. This method is the one we prefer using for infants, in whom cooperation for auscultation is so frequently limited.

The flush method also measures systolic pressure only, and unfortunately, it requires two persons. Using both hands on the child’s upraised arm, express most of the blood from the arm. This maneuver sometimes can be accomplished by wrapping the arm tightly from the fingers to the cuff level with a tensor bandage. The second person should then inflate the cuff and unwrap the bandage. The arm should look relatively pale. One person watches the arm, and the other watches the sphygmomanometer. As the cuff pressure is decreased, the person watching the arm indicates verbally the moment at which the flush occurs while the other person records the manometer level.

Blood pressure measurement with automatic Doppler equipment is much easier than with the other methods, because no auscultation is required. Unfortunately, the equipment is not always available, blood pressures obtained are sometimes questionable, and the choice of cuffs may be limited.

One way or another, a reliable blood pressure measurement must be obtained. If you suspect an aortic coarctation (i.e., if there is high pressure in the arm, an absence of femoral pulses, and a murmur), repeat the procedure in the thigh, using a blood pressure cuff of an appropriately larger size. Again, the manual Doppler method with the probe held on the posterior tibial or popliteal pulse is our preferred method of measuring blood pressure. The normal range of systolic and diastolic blood pressures for older children is shown in Table 10–2.

In our experience, the so-called radial-femoral pulse delay is a useless sign in infants with a rapid heart rate. It is easy to say that such a delay is present when one knows in advance that the diagnosis is aortic coarctation. It is better to rely on a comparison of pulse volumes and blood pressure measurements.

Additional maneuvers

After listening to the infant’s back and finishing the general examination, you may have to make the baby cry, so advise the parents of your intention. Gently flicking the bottom of the foot usually does the trick, but at times a surreptitious pinch or two of the big toe (the dermal compression test) is required. While the baby cries, look for central cyanosis. Remember that central cyanosis occurs in any baby who holds his or her breath for a prolonged period. If the mucous membranes inside the mouth exhibit a purplish discoloration while the baby is crying vigorously, the infant probably has a serious problem. Discoloration of the buccal mucous membranes may be the one and only clinical finding in transposition of the great arteries, a disorder that is potentially lethal if not diagnosed early. If you have any doubts about this diagnosis, consult a cardiologist as soon as possible.

History-taking

Although many of the symptoms sought in infants and older children are similar, the cardiovascular history of the older child differs somewhat from that of the infant, because the diseases seen are different and children are becoming more active. It is therefore important to modify the cardiac functional enquiry accordingly for older children. For example, respiratory symptoms that previously were precipitated by feeding may be brought on with exercise or play. These modifications are discussed in the following sections.

Respiratory symptoms and exercise intolerance

Respiratory difficulty that occurs in older children as opposed to infants usually is precipitated by activity rather than by feeding. Respiratory symptoms are closely related to exercise intolerance, which is the primary manner in which respiratory symptoms manifest in this group. Eliciting the exact nature of the respiratory symptoms that are being experienced is important. For example, stridor on exertion has a different differential diagnosis than does dyspnea on exertion. In the former, a vascular ring would be an important diagnosis to exclude, particularly if the child has concomitant swallowing difficulties. Shortness of breath on exertion is a common finding in older children with heart disease, but it usually is not described that way. Instead, children may complain that they are not able to keep up with their same-age peers in gym class. They may stop before other children at play when participating in activities such as bike riding or running. You can inquire about this phenomenon in a very concrete way by asking about common childhood games, such as tag. For example, you can ask children if they are always “it” when playing tag or if they often finish last in races. Try to quantify exercise tolerance by asking the number of stairs the patient can go up without becoming tired or whether it is necessary to pause halfway up the stairs to take a rest before continuing. Ask children who cannot climb any stairs how far they are able to walk on a level surface. Other (usually older) children have a feeling of fatigue in the absence of activity.

Poor weight gain

Like babies with heart disease, older children with serious heart disease often do not grow very well. This finding is most pronounced in children with congestive heart failure and is caused by the increased energy needs of the body created by the heart failure. These increased metabolic demands use up the energy that otherwise would be used for growth. Compounding the problem, these children often have poor food intake, either because they tire easily when eating or because they cannot tolerate food due to intestinal edema related to the heart failure. A former teacher used to enjoy asking students and cardiology fellows about this phenomenon at rounds. He would say, “If you were trapped on a desert island with only one instrument to look after children with heart disease, what would it be?” Almost invariably, the uninitiated would say, “A stethoscope.” However, the answer he was looking for was “a scale,” because children with serious heart disease just do not grow properly.

Cyanosis

Cyanosis is distinctly uncommon in older children presenting for the first time with possible heart disease because most cyanotic defects have been repaired or palliated by the time children reach 5 years of age. It is still worth asking about, though, because occasionally a patient with pulmonary hypertension or an undiagnosed cyanotic lesion turns up, and the presence of cyanosis may be an important clue to the diagnosis.

Palpitations

Palpitations are uncommon, but they can occur in very young children. Palpitations are discussed in detail in the section on teenagers (pages 156-158). In young children, it is important to remember that palpitations may be reported as “chest pain” or simply as an uncomfortable feeling in the throat, the latter being particularly common in the presence of supraventricular tachycardia (SVT).

Syncope

Although true cardiac syncope is uncommon in young children, the practice of holding one’s breath is not uncommon. As is described for older patients later in this chapter, the history is the key to the diagnosis. Breath-holding typically is practiced by children between the ages of 6 months and 6 years and rarely occurs beyond 8 years of age. Episodes may be associated with cyanosis or pallor. In contrast to most cases of cardiac syncope (which usually occur out of the blue), breath-holding episodes are almost always triggered by an injury or anger-inducing event, though such triggers may be relatively minor. As with older children, exploration of the family history is important (see the section on teenagers), with consideration of a wider differential when there is a history of sudden or unexplained death. When the description of the events suggests cardiac syncope, patients should be referred to a pediatric cardiologist. A more detailed description of syncope is included in the section on teenagers.

A summary of points to include in the cardiac functional inquiry at different ages is provided in Table 10–3.

TABLE 10–3 Symptoms to Include in Cardiac Functional Enquiry at Different Ages

Past medical history and medication use

The past medical history also is important in older children because the presence of certain syndromes, which may have been undiagnosed in younger children, may lead you to suspect a cardiac problem. For example, a child known to have a history of tracheal-esophageal fistula repair might have VACTERL syndrome (a mnemonic for Vertebral, analatresia, tracheo-esophageal fistula, Renal and Radial Limb anomalies), a condition commonly associated with congenital heart defects such as VSDs. Similarly, in a girl complaining of short stature, subsequent examination might reveal findings of Turner syndrome. Some children, such as recent immigrants or patients who recently have moved from other cities, may have a previously unknown cardiac condition or have had previous cardiac surgery. Eliciting this information is important, because it clearly would affect your interpretation of other symptoms.

Medication use, including the dose, frequency, and duration of use, should always be explored carefully. Ask specifically about over-the-counter medications and herbal remedies, because they often are overlooked but may have cardiac effects. Medication use may be solely or largely responsible for some symptoms, notably palpitations. In other cases, a medication may be contraindicated in a particular heart condition, such as erythromycin in patients with long QT syndrome. Other aspects of the history are similar to those described for younger and older children.

General examination, inspection, and palpation

The general examination, inspection, and palpation portions of the examination of a 3- to 5- year-old are similar to those for the infant and toddler. The decision to examine the child while he or she is on an examination table or in the parent’s lap must be individualized; often it is useful to keep the patient in the parent’s lap. Both the parent and child should face you and sit with the child facing forward. As with younger children, you should start by examining the hands and brachial pulses. The transition to a supine position is easy with a child who is facing forward—just raise the legs and pull forward slightly. This position, with the majority of the child’s body still on the parent’s lap, keeps the child comfortable while giving you easy access to the precordium for palpation and auscultation and to the legs for checking the femoral pulses. For children inclined to kick, you can maintain control of the legs by holding them close to your body underneath your arms. These positions are pictured in Figure 10–7.

FIGURE 10–7 Patients hesitant to lie on the examination table can be examined on the parent’s lap, in both the sitting (A) and supine (B) positions. In the supine position, the examiner holds the patient’s legs against his or her sides both to help support the patient and to avoid what could otherwise be an unfortunate kick from a worried preschooler.

By the time a child is 3 to 5 years old, lesions causing cyanosis or congestive heart failure will have been revealed. The spectrum of disease in toddlers and young children includes congenital lesions that have been overlooked or cause few symptoms, such as atrial septal defects (ASDs), small VSDs, bicuspid aortic valve, and acquired cardiac disorders, such as pericarditis, myocarditis, cardiac manifestations of hereditary muscular and neuromuscular diseases, rhythm disturbances, and other rare disorders. By far, the most common problem clinicians face in this age group is the interpretation of heart sounds and murmurs, especially the systolic murmur. Consequently, we will concentrate on this issue.

Heart sounds and murmurs

Heart Sounds

The heart sounds are conveniently numbered S₁ through S₄. S₁ and S₂ are sounds of valve closure, with S₁ caused by mitral and tricuspid closure and S₂ caused by aortic and pulmonary valve closure. Each heart valve makes a sound on both closing and opening. The sound that occurs upon opening is not heard in healthy people. Given that there are four valves along with systole and diastole, the concept is confusing. You must remember that in people of good health, blood does not move during the period when pressure is building or falling; these are the periods of isovolumic contraction and relaxation. In the systolic events of left ventricle contraction, mitral regurgitation (if present) begins immediately with closure of the mitral valve as LV pressure exceeds left atrial pressure. Regurgitation begins immediately (with S₁), and an early systolic regurgitant murmur is heard. Meanwhile, as pressure builds in the left ventricle, the aortic valve opens and ejection begins. In the presence of underlying aortic valve pathology, an ejection murmur takes place, beginning a short period after S₁, and a midsystolic ejection murmur evolves. Therefore, the regurgitant murmur begins with the preceding sound, but the murmur of obstruction or excessive flow begins after the preceding sound. If you can comprehend this process, you are well on the way to understanding cardiac auscultation.

Memorize these two facts:

1. The left-sided valves close before the right-sided valves.

2. Left-sided valve closures are much louder. The right-sided valve can be heard closing only when the stethoscope is positioned directly over it on the chest.

The mechanism of production of the S₃ and S₄ heart sounds is in question. They probably are a result of the deceleration of blood at the end of early (S₃) and late (S₄) rapid filling phases of the ventricles. Although the exact mechanism of the third and fourth heart sounds is poorly understood, S₃ usually is related to high flows and S₄ reflects a poorly compliant ventricle. S₃ sounds are normal in children with hyperdynamic circulations and thin chest walls but are usually abnormal in patients older than 30 years, when the effects of age have lowered stroke volume and increased body mass. Audible S₄ sounds are always abnormal. S₃ and S₄ sounds occur in the ventricles and are low-pitched. They are heard loudest over the ventricle in which they occur and are best heard with the bell of the stethoscope.

Use of the terms clicks and snaps is a continual source of confusion. Valve opening is quiet in healthy persons and signals the end of the period of isovolemic contraction, or relaxation.

Key Point

A problem exists when a sound is heard at the time of the opening of any heart valve.

A sound heard at the time of opening of the pulmonary or aortic valve is called an ejection click; when mitral or tricuspid opening is heard, the term opening snap is used. The clicks signal the beginning of ejection into a dilated great vessel; the snaps signal the commencement of diastolic flow into the ventricle. Both are always high-pitched and are loudest over their respective valves, except the aortic click, which is usually heard clearly at the apex. The pulmonary ejection click is unique in that it is loudest or sometimes only heard during expiration. The only hope of identifying these sounds is a thorough working knowledge of what is normal and of what you would expect to hear in a normal infant or child when you place a stethoscope on a particular area of the chest. Normal and abnormal sounds for each listening area are shown in Figure 10–8.

FIGURE 10–8 Normal and abnormal sounds in the four conventional auscultation areas. A, aortic; P, pulmonary; T, tricuspid; M, mitral; EC, ejection click; OS, opening snap.

Key Point

It is essential to follow a constant, systematic procedure for listening to heart sounds and murmurs in all children. Never auscultate the heart through clothing. The examination is difficult enough without such interference.

First, listen exclusively to the individual heart sounds (understanding in advance what is normal). Then listen, equally systematically, to the murmurs. When listening to the heart, begin at the apex and work your way toward the left sternal border and up to the base. Start with the diaphragm going one way and then switch to the bell and work your way back.

Here is a brief summary of normal sounds as heard when the child is supine. If sounds other than these are heard, the child may well have a cardiac problem.

• At the apex, you should hear a single first, a single second, and possibly a third sound. The first and second sounds are high-pitched, and the first sound is usually louder. The third sound is heard best with the bell of the stethoscope.

• At the tricuspid area, the first sound may be closely split, and the second sound is single. You may hear a third heart sound.

• In the pulmonary area, the first sound is usually single. The second sound is split in inspiration and should be closely split or single in expiration.

• At the aortic area, both the first and second sounds are single, and aortic closure is usually louder.

Remember that these are the findings when the patient is supine. The intensity of the first heart sound varies with AV conduction time (the interval between the onset of P wave and R wave [PR interval]). When the PR interval is prolonged, the valve leaflets may have almost closed when the ventricle contracts. Accordingly, the first sound is faint or absent; this pattern can occur in normal persons who have a long PR interval. Usually, if the patient stands up, the PR interval shortens, and the first sound increases to fairly normal intensity. Occasions exist in which there is beat-to-beat variation in intensity of the first sound. This variation occurs in AV dissociation, as in complete heart block, in which there is beat-to-beat variation in the PR interval—a useful sign in differentiating complete AV block from sinus bradycardia.

Gallop Rhythm

Gallop rhythm is a misleading term, because gallop generally is thought to signify a problem, which it does not. It is better to speak of a triple rhythm and of the sound that causes the tripling. Several types of tripling exist, only some of which signify a problem. If the triple rhythm is rapid, there is a gallop cadence, which should still be described as tripling. A first sound, second sound, and prominent third sound would constitute tripling, as would a fourth sound, first sound, and second sound, or a first sound, ejection click, and second sound. The type of tripling commonly called gallop rhythm occurs when the third and fourth heart sounds sum in the presence of tachycardia, which may or may not be pathologic. In infants with a physiologically long PR interval and tachycardia, summation tripling can be normal; presence of a pathologic third sound or fourth sound and tachycardia, however, would be abnormal. Because tripling can be normal or abnormal, you must try to identify the sound that causes the tripling.

Midsystolic Click

The sound that does not fit any of the preceding descriptions and is usually best heard in the midcardiac area is the sound of mitral valve prolapse—the midsystolic click. Usually heard in midsystole, this sound may be a single click or a series of clicks. It is caused by prolapse of the mitral valve or portions of it into the left atrium. Frequently associated with a deficiency of tone in connective tissue, these midsystolic clicks occur most often in tall, asthenic, more commonly female individuals. It is best heard with the patient standing and leaning forward. A midsystolic click also can cause a triple rhythm; such a click may be confusing until you have heard it two or three times. Variation in intensity from moment to moment also is characteristic.

Murmurs

Heart murmurs are caused either by turbulence in the blood or by tissue vibration. Conventionally, they are classified according to their timing as:

• Systolic (occurring between the first and second sounds)

• Diastolic (occurring between the second sound and the first sound)

• Continuous (present continuously throughout the cardiac cycle)

The last term also includes the murmur that begins in systole, passes through the second sound, and ends in diastole.

Systolic Murmurs

Systolic murmurs also are classified according to their dynamic mechanism, of which there are four types:

1. Regurgitation (backward flow of blood)

2. Obstruction to forward flow

3. Vibration of tissue, which occurs in the normal heart when forceful contraction causes the tissue to vibrate and in an abnormal heart when a substance such as calcium is present and is made to vibrate even by normal blood flow

4. Excessive flow, which implies that the volume of blood is too large for a normal orifice or vessel

Ask yourself which mechanism is operating whenever you hear a systolic murmur.

Regurgitant Murmurs

There is a general tendency to use the terms regurgitation and insufficiency synonymously. Insufficiency is a poor term. For example, the valve may be insufficient in its ability to open properly; thus, valve stenosis also could be classified as insufficient.

Backward blood flow through a valve is regurgitation. Blood that regurgitates does not have to wait for the aortic or pulmonary valve to open; thus, turbulence may begin during the period of isovolemic contraction, commencing with the first heart sound, continuing through systole, and concluding with the second heart sound. Typically, these murmurs are pansystolic. The pitch of a murmur is directly proportional to the pressure gradient between the place where the blood flow that is causing the murmur begins and the place where it ends. In each of the three conditions associated with systolic regurgitation, the pressure gradient between the two chambers is high. A high-pressure gradient is associated with a high-velocity jet, which causes shedding of small vortices or eddies. Although the murmur traditionally is described as high-pitched, it is in fact of medium pitch, in the middle range of our hearing (400 to 550 Hz), but it is relatively high-pitched as most murmurs go. It sounds like a breath sound and may be blowing or harsh, like tracheal breathing. Neophyte auscultators invariably mistake breath sounds as low-pitched because of their soft quality, but they are not low-pitched.

Key Point

When a murmur sounds like a breath sound, it is not an innocent murmur.

The three hemodynamic disturbances associated with systolic regurgitant murmurs are (1)VSD; (2) mitral regurgitation; and (3) tricuspid regurgitation.

These abnormalities share a common hemodynamic feature: Each is associated with a high systolic pressure gradient. For example, in mitral regurgitation, LV pressure is 100 mm Hg and left atrial pressure is only 5 mm Hg. In small VSDs, the regurgitant murmur may be cut off in late systole as the septum contracts; therefore, the murmur begins with the first sound but ends before the second sound and thus is early systolic to midsystolic in timing.

Generally, regurgitant murmurs are heard loudest over the chamber in which they originate. Thus the murmur of mitral regurgitation is heard loudest at the apex and radiates toward the axilla. The murmur of VSD is heard best along the left sternal border, over the right ventricular area. The murmur of tricuspid regurgitation is unique in that it is more intense during inspiration because of increased right ventricular filling. It is best heard at the left lower sternal border. It is helpful to emphasize that there is no innocent murmur that sounds like a regurgitant murmur. If it sounds like a breath sound—harsh or blowing, of any degree of intensity—it is organic and signifies regurgitation of blood.

Obstructive Murmurs

All obstructive murmurs are organic. The turbulence caused by obstruction produces eddies of large but varying size, and the vortex shedding that results is a dissipation of a large amount of energy. Therefore, obstructive murmurs are coarse and loud. Because the turbulence occurs during forward flow, it must wait for the aortic and pulmonary valves to open, and there is a pause between the first heart sound and the beginning of the murmur. The velocity and volume of blood passing through the valve is greatest toward the center of systole, so the murmur will be loudest at this time, creating a crescendo-decrescendo, diamond-shaped or kite-shaped type of murmur.

These loud, coarse murmurs generally occur over the pulmonary or aortic valve. Unfortunately, there is the occasional exception. The murmur of aortic coarctation tends to be higher in pitch but is heard in a different area, being maximal high in the precordium, in the left axilla, and over the left side of the back. Occasionally, obstructions occur in the mid ventricle, in which case the murmur also tends to be of higher pitch and may be difficult to differentiate from a regurgitant murmur. Generally speaking, obstructive murmurs are recognized easily as being organic from their intensity and coarseness, and they tend to radiate in the direction of blood flow, where the vortex shedding process is occurring. Hence, the murmur caused by aortic stenosis is heard clearly over the carotid arteries, whereas the murmur of pulmonary stenosis frequently radiates to the back.

Vibratory Murmurs

Vibratory murmurs are murmurs of musical quality. They have harmonics or frequencies of sound that are integer multiples of the fundamental frequency. The innocent vibratory murmur found in children was described first in 1909 by Still, who likened it to the twanging of string. Vibratory murmurs arise in tissue, and because tissue vibrates in harmonics, these murmurs are unlike any others. Nevertheless, the musical quality is difficult for some examiners to appreciate. A medium-pitched musical murmur would sound like a hum, whereas one with high-pitched components would sound like a seagull’s cry. Because vibration occurs in tissue, it often transmits in the same tissue plane. Thus a vibratory murmur arising in the LV outflow tract will transmit through the LV tissue toward the apex or through the aortic wall up toward the aortic listening area.

The innocent vibratory systolic murmur heard commonly in children probably results from a high stroke volume being ejected forcefully, causing tissue in the left ventricle to vibrate. It is heard maximally in expiration and usually is best heard midway between the left sternal border and the apex. Merely detecting a musical quality of the murmur in children means that the chances are high that the murmur is innocent (Fig. 10–9). When calcium is deposited in a heart valve, the resultant murmur is not just musical; it has high-pitched components. Occasionally, in children who have a perimembranous VSD, the murmur may have a similar high-pitched component, possibly caused by vibration of the membranous portion of the septum.

FIGURE 10–9 Systolic murmurs. ASD, atrial septal defect; VSD, ventricular septal defect.

Key Point

A musical murmur in a child is almost invariably innocent. If it hums, it is not organic.

Flow Murmurs

Flow murmurs are generated by the turbulence associated with increased stroke volume. Systolic flow murmurs occur in the outflow tract of either the left or right ventricle and accordingly are usually heard maximally at the left or right sternal border in the second interspace. A flow murmur at the left sternal border is probably occurring in the pulmonary artery and is almost never loud enough to be associated with a thrill. Flow murmurs heard at the right sternal border may be associated with a short, coarse, low-pitched sound over the carotid artery. Flow murmurs usually are associated with other evidence of a high stroke volume. Invariably, when the patient with such a murmur is examined in a standing position, the systolic flow murmur greatly diminishes in intensity or totally disappears because of the decrease in stroke volume that occurs in the standing position.

Flow Murmurs and the Second Heart Sound

The characteristics of the second sound become extremely important in the interpretation of the significance of flow murmurs. Unfortunately, the mechanism of an ASD murmur, which is actually a flow murmur arising in the right ventricular outflow tract, is similar to that of the innocent functional flow murmur heard in normal individuals, and the two murmurs may be indistinguishable on auscultation. The key distinguishing feature is the characteristic fixed splitting of the second sound that occurs with most ASDs. When listening for the second heart sound, apply the diaphragm of the stethoscope over the second interspace at the left sternal border (with the patient supine). In the normal child, the split of the second sound widens with inspiration as a result of increasing right ventricular stroke volume and longer ventricular contraction. With expiration, the split narrows but may not close entirely. In the common form of ASD, blood ejected from the right ventricle is constant in volume in both inspiration and expiration; hence, splitting of the second sound is fixed, meaning that it does not change with the respiratory phase. If you have difficulty hearing normal movement of the split of the second sound, have the child sit up; movement of the split may be sluggish in the supine position. Other features, such as an easily palpable right ventricular impulse and a mid-diastolic murmur in the tricuspid area, may help in the diagnosis of ASD.

Key Point

A nonmusical ejection systolic murmur in the pulmonary listening area and fixed splitting of the second sound signify ASD. A systolic murmur cannot be ignored until you are certain that the components of the second sound are moving normally.

Innocent murmurs are common. Such murmurs may be heard on careful auscultation in as many as 40% of 3- to 4-year-old children and are estimated to be audible in at least 60% of children over the course of their lifetime. Innocent murmurs include the vibratory murmur, the flow murmur with a normal second sound, the carotid bruit, and the venous hum (described later in this chapter). It is important to know these murmurs well, because any health care system can tolerate only a limited number of cardiology consultations for innocent murmurs.

Diastolic Murmurs

Diastolic murmurs are organic, with rare exceptions, such as the mid-diastolic flow murmur that may occur with marked sinus bradycardia. Velocity of flow in diastole differs from systole; it is maximum early in diastole with the opening of the AV valves and then late in diastole with atrial contraction. These velocities influence the timing of diastolic murmurs, but generally speaking, diastolic murmurs are classified much like systolic murmurs, by their timing. They may be early, beginning with the second sound, mid-, or mid-to-late. Yet, when a murmur is only late diastolic in timing, we term it presystolic. The term pandiastolic is never used. The mechanisms are the same, and the murmurs produced are therefore regurgitant, obstructive, flow, or vibratory.

Regurgitant diastolic murmurs imply either aortic or pulmonary valve regurgitation. As with regurgitant systolic murmurs, the regurgitant diastolic murmur begins with that portion of the second heart sound caused by the closure of either the pulmonary or aortic valve. The murmur of aortic regurgitation is high-pitched because of the high pressure gradient between the aorta and the left ventricle in diastole, and it is heard maximally along the left sternal border, where the turbulence is occurring. The murmur of pulmonary regurgitation with normal pulmonary artery pressure is low-pitched because of the low pressure gradient; it is heard in the same area as the aortic regurgitation murmur. When the child has pulmonary hypertension, the murmur, known as the Graham-Steell murmur, is high-pitched because of the high pressure gradient between the pulmonary artery and the right ventricle in diastole.

Obstructive murmurs are caused by mitral or tricuspid stenosis, which are uncommon congenital heart lesions. In areas where rheumatic fever is still endemic, this murmur may be encountered when an older child has mitral stenosis associated with chronic rheumatic carditis. Decrescendo-crescendo in shape related to flow velocity, the murmur is low-pitched, and it does not begin until the mitral valve opens; therefore, there is a pause between the second sound and the start of the murmur.

With mitral valve stenosis, the murmur occurs in the left ventricle and is loudest at the apex. Frequently, only the late diastolic portion of the murmur is present; in which case it is presystolic in timing. Students commonly identify this murmur improperly, believing it to be systolic. There is no systolic murmur maximal at the apex that is low-pitched and rumbling.

A diastolic flow murmur occurs with lesions such as VSD, ASD, and mitral or tricuspid regurgitation. Its presence indicates that flow volume across the AV valve is at least twice normal. Mid-diastolic in timing, of short duration, and medium in pitch, this flow murmur is heard maximally in either the apical or tricuspid area, depending on which valve generates the turbulence. As noted previously, the same murmur is heard in the presence of marked bradycardia, and it is invariably present in complete AV block before the implantation of a pacemaker.

Occasionally, a vibratory or musical diastolic murmur may be heard. One example is the cooing, early diastolic murmur of aortic regurgitation that occurs when the regurgitant jet causes bacterial endocarditis vegetations on the aortic valve to vibrate.

Many noncardiologists have difficulty eliciting diastolic murmurs because of inexperience and the relative rarity of diastolic murmurs.

Continuous Murmurs

Of the many causes of continuous murmurs, only two are of major importance. The most common continuous murmur is a normal finding known as the venous hum. In children whose circulation is hyperkinetic, continuous turbulence is audible over the jugular veins and usually is loudest in the right supraclavicular fossa. This murmur, usually heard only in the sitting or upright position, varies considerably in intensity with movement of the child’s head, and its intensity may be influenced by light pressure on either jugular vein (Fig. 10–10). The turbulence also may be palpated with light pressure on the jugular vein (Fig. 10–11). With light finger pressure, a thrill may be palpable. Occasionally, a venous hum is audible when the child is supine with the head only slightly elevated. This murmur, as common as it is, frequently confounds the examiner, who usually has forgotten the basic rule of first listening with the patient in the supine position.

FIGURE 10–10 The intensity of a venous hum may be enhanced by rotating the head laterally and extending the neck slightly.

FIGURE 10–11 Pressure on the jugular vein influences the intensity of a venous hum, and with very light pressure, a thrill may be detected.

The other important continuous murmur is that of a PDA. It is heard maximally on the left side of the thorax, usually just below the clavicle or between the left sternal border and the midclavicular line in the second interspace. In the child older than 1 month whose pulmonary artery pressure is not elevated, the patent ductus murmur has the same continuous timing as the venous hum but peaks in intensity earlier, at the time of the second heart sound, when the pressure gradient between the aorta and pulmonary artery is the greatest. In contrast to the venous hum, it is heard clearly in the supine position. Flow through a PDA of average size increases aortic runoff and LV stroke volume. Accordingly, the pulse is bounding, and LV activity (only the left) is readily palpable. If either of these findings is present, be sure to search particularly for a patent ductus murmur. This murmur has been variously described as having a machinery or train-in-a-tunnel quality. An inexperienced examiner hears only the loudest part of the murmur, often missing its decrescendo diastolic component.

Rarely, a continuous murmur with the characteristics of a PDA murmur is heard in another location over the precordium (e.g., in a patient with a coronary artery fistula, in which it is best heard along the lower left sternal border). A continuous murmur from anything other than a venous hum is pathologic, and the patient should be referred to a specialist.

Other Systolic Murmurs

Two murmurs that deserve individual attention are the cardiorespiratory murmur and the murmur of mitral valve prolapse.

The cardiorespiratory murmur is frequently missed because it generally does not occur in the conventional listening stations. It tends to be loudest in the midclavicular line in the third interspace on either side of the chest, more often on the right. Occasionally, it also is heard in the back. Characteristically, three successive systolic blowing murmurs occur in the middle and late inspiration phases. They are entirely absent during early inspiration and expiration. When heard loudest at the apex, the cardiorespiratory murmur may be confused with the murmur of mitral regurgitation. The cardiorespiratory murmur has no clinical significance. It is thought to be generated in a portion of lung that is trapped and compressed during inspiration. If the patient is cooperative and can hold his or her breath, the murmur, of course, disappears.

The whoop that sometimes occurs with mitral valve prolapse is best heard with the patient standing. It occupies the mid to late portion of systole and may be exceedingly loud, sometimes being audible without a stethoscope. Whoops are usually evanescent, being loud at one time and absent at another. When a colleague performing cardiac auscultation says excitedly, “You just have to come and hear this,” it usually turns out to be this whoop. The patient is usually tall and asthenic and frequently has a thoracic bony abnormality, such as pectus excavatum. No other murmur sounds like it.

You may find this clinical tidbit interesting: Mitral valve prolapse may be familial and due to a congenital connective tissue defect. There was a case in which two sisters had mitral valve prolapse and, at times, either sister could have a whoop loud enough that it could be heard by the other sister across the room. When one sister had the noise, the other sister would say, “You’re whooping!”

The mitral regurgitation that may accompany mitral valve prolapse may not have a whooping quality. Instead, it may have only the blowing quality of mitral regurgitation from any cause. It will be mid to late systolic in timing, however, at least in its mild form.

Pericarditis and mediastinal emphysema

Two other auscultatory findings of significance are the pericardial friction rub of pericarditis and the mediastinal crunch of mediastinal emphysema.

Pericardial friction occurs most frequently in patients who have undergone cardiac surgery. When detected without relation to cardiac surgery, it may be associated with pericarditis from any cause, but usually viral. Most clinicians identify a friction rub easily because of its characteristic scratchy quality. Pericardial rubs may be heard anywhere on the left side of the chest but usually are heard most clearly along the sternal border. Contrary to earlier teaching, their presence bears little or no relation to the amount of effusion within the pericardium. Pericardial rubs generally have three phases related to atrial filling, ventricular ejection, and the rapid phase of ventricular filling, giving them a characteristic cha-cha-cha cadence.

The mediastinal crunch, once heard, is also characteristic. It indicates air present within the pericardial space. It has much the same quality as pericardial friction, but although it may have a to-and-fro rhythm, it does not have a three-phase cadence. More often, the rhythm of mediastinal crunch is chaotic—it is sometimes systolic and sometimes phasic with respiration. It may occur spontaneously or after a chest injury, including iatrogenic lung injury from mechanical ventilation.

Auscultation technique for murmurs

As with the examination of individual heart sounds, to ensure that you fully understand a particular murmur, it is important to be systematic in its assessment. Start at the apex with the patient supine. Begin with the stethoscope diaphragm, because most troubling murmurs are in the medium- to high-pitched range. Listen to systole. A murmur in systole at the apex is not necessarily loudest in this area, so track the murmur with the stethoscope to its point of maximal intensity. While listening, attempt to answer the following questions:

Let us suppose that the murmur has been described as pansystolic (regurgitant); of grade 3/6 intensity; high-pitched with a harsh blowing quality; and heard maximally at the fifth left interspace in the anterior axillary line. This is the description of mitral regurgitation. Remember that if any apical systolic murmur is pansystolic in timing, it should be identified automatically as organic. Then listen to diastole at the apex. Listen carefully to the nothings—the areas initially perceived to be silent.

Then move the stethoscope to the tricuspid area and listen first to systole and then again to diastole. Suppose that you hear a murmur in systole, but as you track it to its point of maximum intensity, it is loudest at the left sternal border in the second interspace. Is it regurgitant or ejection? If it is not pansystolic (regurgitant) and if it does not sound like a breath sound, it is probably ejection. Is it caused by obstruction, flow, or vibration? If it is not low-pitched and coarse, it is not obstructive. Listen for a musical component. If there is no musical component, the murmur is not vibratory. Thus, by exclusion, it is a flow murmur that is either innocent or due to ASD.

You should now listen to the second heart sound again. If splitting is fixed, the child probably has ASD. Do not be satisfied with this conclusion, however; listen also to diastole. If the child has ASD, a mid-diastolic flow murmur is probably present at the left sternal border in the fourth space. If the split in the second sound moves nicely, ASD is not present. Have the child stand up; if the murmur disappears, you can conclude that the murmur is innocent. In this situation, other signs of a high-output state are probably present. In this manner, proceed with auscultation at each listening area, listening to both systole and diastole.

Auscultation of the heart is a difficult skill to acquire. To become proficient requires many hours of listening to hearts, so take advantage of every opportunity you have to do so. Despite their best intentions, most students do not listen to enough hearts to acquire a high level of auscultatory skill. Thus you are thus encouraged to use alternate methods to improve your skills, such as CD ROMs, DVDs, Web-based learning tools, and podcasts (e.g., review EarsOn! at http://earson.ca). Substantial research in cardiac auscultation supports the fact that repetition is the key to success in this area.

Observation

Observe the child’s chest. Is the left side abnormally prominent? Are there abnormal pulsations? Do you see any scars? (Remember to check front and back!) A safe way to begin the hands-on part of the examination is by gently picking up the child’s hand. Are the palmar creases normal? Is there clubbing?

Look at the fingertips from the side (Figs. 10–12 and 10–13). Clubbing occasionally can be normal and may occur in persons with noncardiovascular diseases. Are the fingers of normal length and number? Is there clinodactyly? Dorsiflex the fingers and wrist. Is the tone normal?

FIGURE 10–12 Clubbing is best seen if you view the digit from the side. The earliest sign is diminution of the angle between the nail root and the skin.

(From Constant J: Bedside cardiology, 5th ed. Hagerstown, MD, Lippincott Williams & Wilkins, 1999.)

FIGURE 10–13 Early clubbing in a 7-month-old child with cyanotic congenital heart disease. The nail root–skin angle has been flattened, and the tip of the finger is shiny.

Taking the pulses

Start with the brachial pulse, not the radial pulse. The closer to the heart the pulse is felt, the truer its quality. Using the first and second digits of your right hand, palpate the brachial artery, just above the antecubital fossa (Fig. 10–14). In the older child, it may be preferable to support the child’s right arm with your left, using your right thumb to palpate the pulse. The following questions are important:

FIGURE 10–14 The brachial pulse, which is used to assess the quality of the pulse, is palpated with the first two digits of the right hand.

If the pulse volume seems to be increased, check for a water-hammer pulse by elevating the child’s arm and encircling the upper arm with one hand (Fig. 10–15). A pulse of normal volume is not usually felt with this maneuver. Now dissect the pulse by analyzing the upstroke and downstroke.

FIGURE 10–15 To examine for a collapsing pulse, elevate the arm and encircle the upper arm with your examining hand. This sign may be present in patients with PDA, aortic regurgitation, or hyperkinetic circulation.

If the pulse volume is increased, the child has a hyperkinetic circulation, aortic insufficiency, or a PDA. In such a patient, when blood pressure is measured, the pulse pressure is increased, and when the chest is palpated, the heart action is increased. The pulse quality reflects the manner in which blood leaves the heart and the resistance it meets in the periphery. Now palpate the femoral pulse; if it is of good volume, aortic coarctation is not present. If it is absent or is distinctly smaller in volume than the brachial pulse, the blood pressures in the arms and legs must be carefully measured. As previously mentioned, radial-femoral pulse delay is difficult to elicit (Fig. 10–16), and if aortic regurgitation accompanies coarctation, such a delay is not present.

FIGURE 10–16 In coarctation of the aorta, the initial portion (the percussion wave) of the femoral pulse may be absent, causing so-called radiofemoral delay. Search for this sign with the radial and femoral pulses in juxtaposition.

The clinical conditions described in the following sections can be appreciated as alterations in pulse volume.

Palpation of the chest and abdomen

A 7-year-old child is unlikely to have heart failure; nevertheless, try to identify the edge of the liver. If the liver’s edge is 2 cm or more below the costal margin, look for clinical evidence of pulmonary air trapping (which may be pushing the diaphragm and liver downward), and percuss the top of the liver.

Gently lay a warm hand on the apical area and palpate the apical impulse. It is quick and diffuse, spilling over to the area left of the sternum in the fourth and fifth spaces. You are now palpating the left and right ventricles as they eject greater volumes of blood. If the child is quite active and pulse volume is increased, this finding is normal. If you palpate an apical impulse that is exclusively apical and is forceful and sustained, there is a problem. Palpate the area to the left of the sternum in the third and fourth spaces, searching for a right ventricular impulse. A diffuse quick impulse would be expected in a patient with ASD, for example.

When palpating the thorax for ventricular dynamics, remember the following facts:

Now palpate the second left interspace at the sternal border, using your first and second digits. If an impulse is present, an organic lesion is probably present, and there is pathologic dilatation of the pulmonary artery as a result of increased flow or pressure. Then palpate the suprasternal notch by inserting your index finger as deeply as possible. If the previous findings suggest a hyperkinetic circulation, an impulse normally can be palpated here. A marked, visible impulse at the suprasternal notch signifies increased flow in the aortic arch that is probably organic, and you should search specifically for PDA or aortic insufficiency during auscultation.

Now palpate in the reverse direction, searching for a thrill. Begin in the suprasternal notch. A thrill may be present here even in persons with minor degrees of obstruction in the LV outflow tract. Then palpate for a thrill in the conventional areas. Whatever you can feel you also will be able to hear, only better; however, appreciation of a thrill does help to classify murmurs. If you palpate a thrill, an organic process is certainly present, and you will hear a loud murmur, grade 4 to 6 in intensity.

Auscultation

After palpation of the chest and abdomen, it is time to auscultate. Organize your approach as discussed in detail in the section on examination of the 3- to 5-year-old (pages 146-153). Innocent murmurs are a major problem for the physician, partly because murmurs often are considered in isolation.

Hyperkinesis in a person with hyperkinetic circulation is not restricted to the cardiovascular system, and the child often is quite physically active. Unfortunately, innocent murmurs and organic murmurs can coexist. Even in the child with a hyperkinetic circulation, if the murmur sounds like a breath sound, it is abnormal. Ejection clicks are organic in any setting.

Palpitations

Palpitations represent the subjective feeling that one’s heart is beating in an unusual or abnormal manner. All children who are old enough to understand this phenomenon should be asked about palpitations, although the way in which one asks must be tailored to the child’s age and developmental abilities. Because many adults, let alone children, do not understand what palpitations are, it is important to ask about them in a way that can be easily understood. You can ask children if their heart sometimes beats in a funny way in their chest—for example, whether it skips a beat or seems to flip flop or do somersaults. Older children can be asked if they have noticed that their heart suddenly starts to race at any time. When this condition is present, the mode of onset and offset of these palpitations should be sought.

Palpitations resulting from arrhythmias often come on suddenly and resolve in the same way, although this phenomenon is not absolute. Palpitations that are associated with fainting or dizzy spells are significant and should be investigated more fully. The same is true for palpitations that are brought on by being startled or by exercise. Sometimes these palpitations are caused by ventricular dysrhythmias and can be a cause of sudden death, so take them seriously! Palpitations are sometimes perceived as chest pain. Children with SVT may complain of an unusual feeling in their throats instead of identifying their experience as palpitations. Young people with palpitations also should be asked about use of medications and recreational drugs, including the consumption of energy drinks and other products that contain caffeine.

Fainting

Fainting or syncope occurs when there is hypo-perfusion of the brain, which can arise because of peripheral vasodilation, bradycardia, or the presence of arrhythmias that result in decreased cardiac output. Some obstructive LV outflow tract lesions also can cause fainting. The most common cause of fainting in children is neurocardiogenic, which is also known as vasovagal syncope. This type of fainting spell arises when the brain is “tricked” into decreasing the heart rate and/or blood pressure inappropriately. Although potentially frightening, this type of syncope is largely benign. Fainting sometimes is confused with seizures, and some children who experience neurocardiogenic fainting can experience transient seizure activity during the period of relative brain hypoxia.

The key to determining the cause of fainting is the history. One must ask about the circumstances around the event, including details of exactly what the patient was doing and how he or she looked before, during, and after the event itself. Neurocardiogenic syncope often is preceded by symptoms such as weakness, sweating, and nausea that can last for a few seconds or 1 to 2 minutes. In contrast, cardiac syncope usually occurs without warning or out of the blue. In neurocardiogenic syncope the subsequent period of unconsciousness usually lasts no more than 2 or 3 minutes. On regaining consciousness, the child usually recognizes his or her environment almost immediately. Patients with neurocardiogenic syncope often are said to look pale and sweaty after an episode but otherwise are noted to be close to their normal selves. By contrast, patients who have seizures may remain in a post-ictal state for a period after the loss of consciousness occurs. Neurocardiogenic syncope is more common in girls, and often one of the parents has a history of a tendency to faint. Incontinence during an event is more common with primary seizures than with syncope. The frequency of episodes may be informative as well. Recurrent fainting should always be investigated. It is important to know whether the fainting episode happened with position change or prolonged standing (both of which are common precipitants for neurocardiogenic syncope) or while supine (a more worrisome situation). One should explore any triggers for the fainting, such as being startled or exercise, both of which are ominous symptoms and strongly suggestive of ventricular arrhythmias. Fainting sometimes follows respiratory symptoms. When these symptoms have a sudden onset, they should be considered to be at least potentially related to an arrhythmia and investigated fully. Nonrotatory dizziness also may be cardiac in origin and should be explored in a manner similar to complaints of syncope.

Family history is critical in evaluating both fainting and palpitations. A family history of sudden or unexpected death in a relative younger than 35 years should be a red flag in a person presenting with syncope or palpitations. This topic needs to be explored creatively by asking not only about sudden death but also about unexplained drownings or motor vehicle collisions. Known familial arrhythmia syndromes, such as long QT syndrome, arrhythmogenic right ventricular cardiomyopathy, or catecholaminergic polymorphic ventricular tachycardia are worrisome and should raise concern if they occur in close family members. (Even if you can’t say the names of these syndromes, most of the family members know them and can tell you if you ask!) Similarly, asking about younger persons in the family (i.e., those younger than 35 years) with automatic implantable cardio defibrillators and why they were placed also will help identify children at risk of familial rhythm disorders.

Chest pain

Chest pain tends to occur more often in older than in younger children, although some young children also may report having chest pain. The vast majority of episodes of chest pain in children are functional or benign. True cardiac chest pain is distinctly unusual. In exploring the history of pain for an individual patient, you may wish to use the CLORIDE mnemonic (see Table 10–4) or a mnemonic of your own. The CLORIDE mnemonic can be described as follows:

• Exacerbating and Alleviating Factors: Elucidation of the factors that make the pain better or worse is potentially very useful. Pain accompanying pericarditis or pleuritis often is better in the sitting position and worse when lying flat. Cardiac chest pain resulting from coronary insufficiency, from whatever cause, often comes on only with exercise and is relieved with rest. Chest pain resulting from gastroesophageal reflux often is worse after eating spicy foods. Asking whether patients have noticed anything specific that makes the pain worse or better may be the key to the diagnosis.

FIGURE 10–17 Eliciting a carotid bruit. In patients with this benign murmur, there is no thrill in the suprasternal notch. The murmur of aortic stenosis is also well heard over the right carotid artery, but a thrill is usually poresent in the suprasternal notch.

Chest pain may be associated with other symptoms, such as palpitations or shortness of breath. Either of these features, particularly if the chest pain is achy, dull, or heavy, should prompt one to consider a potential cardiac etiology. This situation is also true if the chest pain is associated with fainting or occurs in a person predisposed to organic chest pain, such as patients with Marfan syndrome or homozygous familial hypercholesterolemia.

Social history also is important in the evaluation of chest pain. Not uncommonly, children and adolescents present with chest pain relatively early after someone in the family has died of a cardiac condition. Often, this reported pain reflects increased anxiety on the part of both the child and family. Carefully obtaining a history and performing a physical to exclude evidence of cardiac disease, along with definitive reassurance that the child does not have the same condition as the grandfather or aunt or whoever had the disease, often can be extremely helpful.