General Appearance and Nutritional State

The physician should note whether the child is in distress, well nourished or undernourished, and happy or cranky. Obesity should also be noted; besides being associated with other cardiovascular risk factors such as dyslipidemia, hypertension, and hyperinsulinemia, obesity is also an independent risk factor for coronary artery disease.

Chromosomal Syndromes

Obvious chromosomal abnormalities known to be associated with certain congenital heart defects should be noted by the physician. For example, about 40% to 50% of children with Down syndrome have a congenital heart defect; the two most common defects are endocardial cushion defect (ECD) and ventricular septal defect (VSD). A newborn with trisomy 18 syndrome usually has a congenital heart defect. Table 2-1 shows cardiac defects associated with selected chromosomal abnormalities along with other hereditary and nonhereditary syndromes.

Hereditary and Nonhereditary Syndromes and Other Systems Malformations

Congenital cardiovascular anomalies are associated with a number of hereditary or nonhereditary syndromes and malformations of other systems. For example, a child with a missing thumb or deformities of a forearm may have an atrial septal defect (ASD) or VSD (e.g., Holt-Oram syndrome [cardio-limb syndrome]). Newborns with CHARGE ( c oloboma, h eart defects, choanal a tresia, growth or mental r etardation, g enitourinary anomalies, e ar anomalies) association show a high prevalence of conotruncal abnormalities (e.g., tetralogy of Fallot [TOF], double-outlet right ventricle (RV), persistent truncus arteriosus). A list of cardiac anomalies in selected hereditary and nonhereditary syndromes is given in Table 2-1 . Certain congenital malformations of other organ systems are associated with an increased prevalence of congenital heart defects ( Table 2-2 ).

Color

The physician should note whether the child is cyanotic, pale, or jaundiced. In cases of cyanosis, the degree and distribution should be noted (e.g., throughout the body, only on the lower or upper half of the body). Mild cyanosis is difficult to detect. The arterial saturation is usually 85% or lower before cyanosis is detectable in patients with normal hemoglobin levels (see Chapter 11 ). Cyanosis is more noticeable in natural light than in artificial light. Cyanosis of the lips may be misleading, particularly in children who have deep pigmentation. The physician should also check the tongue, nail beds, and conjunctiva. When in doubt, the use of pulse oximetry is confirmatory. Children with cyanosis do not always have cyanotic congenital heart defects. Cyanosis may result from respiratory diseases or central nervous system disorders. Cyanosis that is associated with arterial desaturation is called central cyanosis. Cyanosis associated with normal arterial saturation is called peripheral cyanosis. Even mild cyanosis in a newborn requires thorough investigation (see Chapter 14 ).

Peripheral cyanosis may be noticeable in newborns who are exposed to cold and those with congestive heart failure (CHF) because, in both conditions, peripheral blood flow is sluggish, losing more oxygen to peripheral tissues. Cyanosis is also seen in polycythemic patients with normal O ₂ saturation (see Chapter 11 for the relationship between cyanosis and hemoglobin levels). Circumoral cyanosis, cyanosis around the mouth, is found in normal children with fair skin. Isolated circumoral cyanosis is not significant. Acrocyanosis is a bluish or red discoloration of the fingers and toes of normal newborns in the presence of normal arterial oxygen saturation.

Pallor may be seen in infants with vasoconstriction from CHF or circulatory shock or in severely anemic infants. Newborns with severe CHF and those with congenital hypothyroidism may have prolonged physiologic jaundice. Patent ductus arteriosus (PDA) and pulmonary stenosis (PS) are common in newborns with congenital hypothyroidism. Hepatic disease with jaundice may cause arterial desaturation because of the development of pulmonary arteriovenous fistula (e.g., arteriohepatic dysplasia).

Clubbing

Long-standing arterial desaturation (usually longer than 6 months’ duration), even if too mild to be detected by an inexperienced person, results in clubbing of the fingernails and toenails. When fully developed, clubbing is characterized by a widening and thickening of the ends of the fingers and toes, as well as by convex fingernails and loss of the angle between the nail and nail bed ( Fig. 2-1 ). Reddening and shininess of the terminal phalanges are seen in the early stages of clubbing. Clubbing appears earliest and most noticeably in the thumb. Clubbing may also be associated with lung disease (e.g., abscess), cirrhosis of the liver, and subacute bacterial endocarditis. Occasionally, clubbing occurs in normal people, such as in familial clubbing.

Diagram of normal and clubbed fingers.

Respiratory Rate, Dyspnea, and Retraction

The physician should note the respiratory rate of every infant and child. If the infant breathes irregularly, the physician should count for a whole minute. The respiratory rate is faster in children who are crying, upset, eating, or feverish. The most reliable respiratory rate is that taken during sleep. After finishing a bottle of formula, an infant may breathe faster than normal for 5 to 10 minutes. A resting respiratory rate more than 40 breaths/min is unusual, and that more than 60 breaths/min is abnormal at any age. Tachypnea, along with tachycardia, is the earliest sign of left-sided heart failure. If the child has dyspnea or retraction, this may be a sign of a more severe degree of left-sided heart failure or a significant lung pathology.

Sweat on the Forehead

Infants with CHF often have a cold sweat on their foreheads. This is an expression of heightened sympathetic activity as a compensatory mechanism for decreased cardiac output.

Acanthosis Nigricans

Acanthosis nigricans is a dark pigmentation of skin creases most commonly seen on the neck in the majority of obese children and those with type 2 diabetes. It is also found in the axillae, groins, and inner thighs and on the belt line of the abdomen. Rarely, acanthosis occurs in patients with Addison disease, Cushing syndrome, polycystic ovary syndrome (Stein-Leventhal syndrome), hypothyroidism, and hyperthyroidism. This condition is associated with insulin resistance and a higher risk of developing type 2 diabetes.

Inspection of the Chest

Precordial bulge, with or without actively visible cardiac activity, suggests chronic cardiac enlargement. Acute dilatation of the heart does not cause precordial bulge. Pigeon chest (pectus carinatum), in which the sternum protrudes on the midline, is usually not a result of cardiomegaly.

Pectus excavatum (undue depression of the sternum) rarely, if ever, causes significant cardiac embarrassment. Rather, it may be a cause of a pulmonary systolic murmur or a large cardiac silhouette on a posteroanterior view of a chest roentgenogram, which compensates for the diminished anteroposterior diameter of the chest. As a group, children with a significant pectus excavatum have a shorter endurance time than normal children.

Harrison’s groove, a line of depression in the bottom of the rib cage along the attachment of the diaphragm, indicates poor lung compliance of long duration, such as that seen in large left-to-right shunt lesions.

Peripheral Pulses

• 1.
  

  The physician should count the pulse rate and note any irregularities in the rate and volume. The normal pulse rate varies with the patient’s age and status. The younger the patient, the faster the pulse rate. Increased pulse rate may indicate excitement, fever, CHF, or arrhythmia. Bradycardia may mean heart block, effects of drugs, and so on. Irregularity of the pulse suggests arrhythmias, but sinus arrhythmia (an acceleration with inspiration) is normal.

  
  
• 2.
  

  The right and left arm and an arm and a leg should be compared for the volume of the pulse. Every patient should have palpable pedal pulses, of the dorsalis pedis, tibialis posterior, or both. It is often easier to feel pedal pulses than femoral pulses. Attempts at palpating a femoral pulse often wake up a sleeping infant or upset a toddler. If a good pedal pulse is felt, coarctation of the aorta (COA) is effectively ruled out, especially if the blood pressure (BP) in the arm is normal.

  
  
• 
  

  Weak leg pulses and strong arm pulses suggest COA. If the right brachial pulse is stronger than the left brachial pulse, the cause may be COA occurring near the origin of the left subclavian artery or supravalvular aortic stenosis (AS). A weaker right brachial pulse than the left suggests aberrant right subclavian artery arising distal to the coarctation.

  
  
• 3.
  

  Bounding pulses are found in aortic run-off lesions such as PDA, aortic regurgitation (AR), large systemic arteriovenous fistula, or persistent truncus arteriosus (rarely). Pulses are bounding in premature infants because of the lack of subcutaneous tissue and because many have PDA.

  
  
• 4.
  

  Weak, thready pulses are found in cardiac failure or circulatory shock or in the leg of a patient with COA. A systemic-to-pulmonary artery (PA) shunt (either classic Blalock-Taussig shunt or modified Gore-Tex shunt) or subclavian flap angioplasty for repair of COA may result in an absent or weak pulse in the arm affected by surgery. Arterial injuries resulting from previous cardiac catheterization may cause a weak pulse in the affected limb.

  
  
• 5.
  

  Pulsus paradoxus (paradoxical pulse) is suspected when there is marked variation in the volume of arterial pulses with the respiratory cycle. The term pulsus paradoxus does not indicate a phase reversal; rather, it is an exaggeration of normal reduction of systolic pressure during inspiration. When arterial BP is being monitored through an indwelling arterial catheter, the presence of pulsus paradoxus is easily detected by a wide swing (>10 mm Hg) in arterial pressure. In a child without arterial pressure monitoring, accurate evaluation requires sphygmomanometry ( Fig. 2-2 ). Pulsus paradoxus may be associated with cardiac tamponade secondary to pericardial effusion or constrictive pericarditis or to severe respiratory difficulties seen with asthma or pneumonia. It is also seen in patients who are on ventilators with high pressure settings, but in these cases, the BP increases with inflation.

  
  

  
  

  
  

  

  
  

  
  

  Diagram of pulsus paradoxus. Note the reduction in systolic pressure of more than 10 mm Hg during inspiration. EXP, expiration; INSP, inspiration.

  
  

The presence of pulsus paradoxus is confirmed by the use of a sphygmomanometer as described below.

• a.
  

  The cuff pressure is raised about 20 mm Hg above the systolic pressure.

  
  
• b.
  

  The pressure is lowered slowly until Korotkoff sound 1 is heard for some but not all cardiac cycles, and the reading is noted (line A on Fig. 2-2 ).

  
  
• c.
  

  The pressure is lowered further until systolic sounds are heard for all cardiac cycles, and the reading is noted (line B on Fig. 2-2 ).

  
  
• d.
  

  If the difference between readings A and B is greater than 10 mm Hg, pulsus paradoxus is present.

Chest

One should palpate the following on the chest: apical impulse, point of maximal impulse (PMI), hyperactivity of the precordium, and palpable thrill.

1

What is the currently recommended BP measuring method?

In the recent past, two Task Forces of the NIH, 1977 and 1987, recommended BP cuff selection based on the length of the arm, initially recommending the cuff width to be two thirds of the arm length and later changing it to three fourths of the length of the arm. These Task Forces have provided normal BP standards based on these unscientific methods. The BP cuff selection based on the length of the arm is scientifically unsound and violates the physical principles underlying indirect BP measurement, which have been established a century ago. For adults, the Special Task Force of the American Heart Association (AHA) has recommended the correct cuff selection method based on the thickness (or circumference) of the arm, and it has been in use since 1950. The correct width of the BP cuff is 40% to 50% of the circumference of the limb on which the BP is being measured ( Fig. 2-3 ). In 1988, the AHA’s Special Task Force extended the same cuff selection method to children as well, but the 1987 NIH Task Force did not. In 2004, the NHBPEP has accepted the correct BP cuff selection method of the AHA.

Diagram showing a method of selecting an appropriate-sized blood pressure cuff. The selection is based on the thickness rather than the length of the arm. The end of the cuff is at the top, and the cuff width is compared with the diameter or circumference of the arm. The width of the inflatable part of the cuff (bladder, cross-hatched areas) should be 125% to 155% of the diameter or 40% to 50% of the circumference of the arm.

The following summarizes current views on BP measurement techniques recommended by the AHA as well as the NHBPEP:

• a.
  

  The BP cuff width should be 40% to 50% of the circumference (equivalent to 125% to 155% of the diameter) of the extremity with the cuff long enough to completely or nearly completely encircle the extremities (endorsed by both groups).

  
  
• b.
  

  The NHBPEP recommends Korotkoff phase 5 (K5) as the diastolic pressure, but this recommendation is debatable based on a number of earlier reports. Earlier studies indicate that K4 agrees better with direct intraarterial diastolic pressure for children 12 years of age and younger (endorsed by the AHA, NIH Task Forces, and the Bogalusa Heart Study [ Hammond et al, 1995 ]).

  
  
• c.
  

  Both groups recommend averaging two or more readings (because the averaged values are closer to the basal BP level and are more reproducible).

  
  
• d.
  

  Both the AHA and the NHBPEP recommend the sitting position with the arm at the heart level.

2

How good are BP standards recommended by the NHBPEP?

Normative BP standards recommended by the NHBPEP are not as good as it was made to believe for several reasons. Readers should be aware of a few major flaws in the NHBPEP’s normative values.

• a.
  

  BP data presented in the NHBPEP standards are not obtained by using the same methodology as the program recommends, nor are they from a nationally representative population. They are obtained through an arm’s-length–based cuff selection method, which is currently abandoned because of its unscientific nature. These values are also from single measurement rather than the averages of multiple readings, as currently recommended. In other words, the original source of the elaborate BP standards of the NHBEP is one that has been abandoned by the program itself, yet the program’s recommended BP standards are from these abandoned studies.

  
  
• b.
  

  Expressing children’s BP levels by age and height percentiles is statistically unsound and unjustified on highly variable office BP readings. Height has no statistically important role in children’s BP levels. Partial correlation analysis in the San Antonio Children’s Blood Pressure Study (SACBPS) shows that when auscultatory BP levels were adjusted for age and weight, the correlation coefficient of systolic BP with height was very small ( r = 0.068 for boys; r = 0.072 for girls), whereas when adjusted for age and height, the correlation of systolic pressure with weight remained high ( r = 0.343 for boys; r = 0.294 for girls). These findings indicate that the contribution of height to BP levels is negligible. The apparent correlation of height to BP levels may all be secondary to a close correlation that exists between height and weight ( r = 0.86). A similar conclusion was reached with oscillometric BP levels in the same study. Although weight is very important contributor to BP, weight cannot be used as a second variable because this would interfere with the detection of high BP in obese children. Thus, we found no rationale to use anything other than age and gender to express children’s normative BP standards.

  
  
• c.
  

  Recommending additional computations requiring the use of scientifically unsound complex BP tables on such highly variable office BP readings is unreasonable and unproductive relative to what is to be gained by such efforts. Analyzing unscientifically obtained data by additional computations does not improve their value.

  
  
• d.
  

  The NHBPEP does not point out that the auscultatory and oscillometric BP readings are not interchangeable. SACBPS, in which both auscultatory and oscillometric methods were used, found that oscillometric systolic pressures are significantly higher than auscultatory BP readings (see later discussion for further details). This finding is important in view of the popular use of oscillometric devices in BP measurements in pediatric practice.

  
  
• e.
  

  As a national guideline, the NHBPEP does not emphasize the important contribution of the “white-coat phenomenon” in office BP readings. The white-coat phenomenon refers to the finding that BP readings obtained in a health care facility are often significantly higher than those obtained outside the facility. The white-coat phenomenon may be the most common cause of high BP readings, not true hypertension, in pediatric practice.

3

Which normal BP standards should be used and why?

• a.
  

  Because the NIH Task Forces’ BP standards (of 1987) were obtained by using the unscientific, arm’s-length–based, cuff selection method, these BP standards are no longer acceptable.

  
  
• b.
  

  The BP standards of the NHBPEP (2004) are riddled with major flaws as discussed earlier; they are not the best standards. Health care providers who choose to use these standards should be aware of the flaws. Although not acceptable as reliable pediatric BP standards, the NHBPEP’s normative values are presented in Appendix B for the sake of completeness ( Tables B-1 , Tables B-2 ).

  
  
• c.
  

  Normative BP percentile values from SACBPS are recommended as a better alternative to BP standards than the NHBPEP’s standards until nationwide data using the currently recommended methods become available. These are the only available BP standards that have been obtained according to the currently recommended method. In the SACBPS, BP levels were obtained in more than 7000 schoolchildren of three ethnic groups (African American, Mexican American, and non-Hispanic white) enrolled in kindergarten through the 12th grade in the San Antonio, Texas, area. Both the auscultatory and oscillometric (model Dinamap 8100) methods were used in the study, and the data were the averages of three readings. No consistent ethnic difference was found among the three ethnic groups, but there were important gender differences. Auscultatory BP data were expressed according to age and gender ( Park, 2001 ). These BP standards are normally distributed from the mean value, and thus the effect of obesity is not a problem in using the standards ( Figs. 2-4 and 2-5 ). Percentile BP values for these figures are presented in Appendix B ( Table B-3 , Table B-3 ).

  
  

  
  

  
  

  

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