Observe Vital Signs and Arterial and Venous Pressures: Record vital signs frequently, including BP, until the child’s condition is stable. The heart rate and respirations are counted for 1 full minute, compared with the values on the ECG monitor, and recorded with activity. The heart rate is normally increased after surgery. The nurse observes cardiac rhythm and notifies the practitioner of any changes in regularity. Dysrhythmias may occur postoperatively secondary to administration of anesthetics, acid-base and electrolyte imbalance, hypoxia, surgical intervention, or trauma to conduction pathways.

At least hourly, auscultate the lungs for breath sounds. Diminished or absent breath sounds may indicate an area of atelectasis, pleural effusion, or pneumothorax. All such cases require further assessment. Auscultation guides the nurse’s selective use of postural drainage and percussion to those pulmonary lobes most in need. It also allows a more objective evaluation of effective ventilation.

Temperature changes are typical during the early postoperative period. Hypothermia is expected immediately after surgery due to hypothermia procedures, effects of anesthesia, and loss of body heat to the cool environment. During this period the child is kept warm to prevent additional heat loss. Infants may be placed under radiant heat warmers. During the next 24 to 48 hours the body temperature may rise to 37.8° C (100° F) or slightly higher as part of the inflammatory response to tissue trauma. After this period an elevated temperature is most likely a sign of infection and warrants immediate investigation for probable cause.

Intraarterial monitoring of BP is almost always done following open-heart surgery. Residual vasoconstriction after cardiopulmonary bypass makes indirect BP readings less reliable, and intraarterial monitoring permits continuous rather than intermittent observation. A catheter is passed into the radial artery or the dorsalis pedis or posterior tibial artery, and the other end is attached to an electronic monitoring system, which provides a continuous recording of the BP. The intraarterial line is maintained with a low-rate, constant infusion of heparinized saline to prevent clotting. Continuous BP readings are compared with those taken indirectly using a sphygmomanometer or oscillometric device (Dinamap). A discrepancy between the two may indicate a change in peripheral vascular resistance, a malfunction in the electronic device, or human error in using the wrong-size BP cuff. The nurse also observes for potential complications of intraarterial monitoring, such as arterial thrombosis, infection, air emboli, or blood loss through the catheter. Prevention of each of these hazards is similar to care for any other type of infusion line.

The intraarterial line is maintained with a low-rate, constant infusion of heparinized saline to prevent clotting. The amount of irrigant is recorded as intake fluid. The dressing at the site is changed daily.

Several IV lines are inserted preoperatively: a peripheral IV to give fluids and medications and a CVP line that is usually inserted in a large vessel in the neck. Intracardiac monitoring lines are placed intraoperatively in the RA, LA, or pulmonary artery. Intracardiac lines allow assessment of pressures inside the cardiac chambers, which give vital information on blood volume, cardiac output, ventricular function, pulmonary artery pressures, and responses to drug therapy in the immediate postoperative period. The RA and CVP lines may also be used to infuse fluids and medications. LA lines and pulmonary artery lines are used with more complex repairs. Intracardiac lines are used only in the ICU, although CVP lines may remain for use as a central IV line outside the ICU. All lines must be cared for using strict aseptic technique to prevent infection. Patients must be carefully assessed for bleeding at the time of line removal. See critical care texts for a more complete discussion of intracardiac lines.

Maintain Respiratory Status: Infants usually require mechanical ventilation in the immediate postoperative period. Children may be extubated in the operating room or in the first few postoperative hours, especially if cardiopulmonary bypass was not required. When weaning and extubation are completed, oxygen is delivered by mask, hood, or nasal cannula and is humidified to prevent drying of mucosa. Encourage the child to turn and deep breathe at least hourly. Every means is employed to enhance ventilation and decrease pain, such as splinting of the operative site and use of analgesics.

Suctioning is performed only as needed and is done carefully to avoid vagal stimulation (which can trigger cardiac dysrhythmias) and laryngospasm, especially in infants. Suctioning is intermittent and is maintained for no more than 5 seconds to prevent depleting the oxygen supply. Supplemental oxygen is administered with a manual resuscitation bag before and after the procedure to prevent hypoxia. The heart rate is monitored after suctioning to detect changes in rhythm or rate, especially bradycardia. The child should always be positioned facing the nurse to permit assessment of the child’s color and tolerance of the procedure.

Chest tubes may be inserted into the pleural or mediastinal space during surgery or in the immediate postoperative period to remove secretions and air and allow reexpansion of the lung. The chest tube is attached to a disposable water-seal drainage system. The underwater drainage prevents air from traveling up the tube into the pleural space and causing pneumothorax. Nursing considerations include (1) do not interrupt water-seal drainage unless the chest tube is clamped, (2) check for tube patency (fluctuation in the water-seal chamber), and (3) maintain sterility.

Check drainage hourly for color and quantity. Immediately postoperatively the drainage may be bright red, but afterward it should be serous. The largest volume of drainage occurs in the first 12 to 24 hours, and drainage is greater after extensive heart surgery.

Chest radiographs are taken when the tubes are inserted to check their location and after they are removed to evaluate the inflation of the lungs. Chest tubes are usually removed on the first to third postoperative day when drainage has diminished.

Removal of chest tubes can be an uncomfortable, frightening experience (see Atraumatic Care box). Warn children that they will feel a sharp, momentary pain. After the suture is cut, the tubes are quickly pulled out at the end of full inspiration in the extubated patient to prevent intake of air into the pleural cavity. (In the intubated patient, the tubes are pulled out on inspiration, since the lungs are stented open with the positive pressure ventilation.) A purse-string suture (placed when the tubes were inserted) is pulled tight to close the opening. A petrolatum-covered gauze dressing is immediately applied over the wound and securely taped to the skin on all four sides so that an airtight seal is formed. The dressing is checked for signs of drainage. It is removed the next day. Breath sounds are auscultated, since pneumothorax is a possible complication of chest tube removal. A chest x-ray film is usually obtained after removal to assess for pneumothorax or pleural effusion.

Provide Maximum Rest: After heart surgery maximum rest should be provided to decrease the workload of the heart and promote healing. Nursing care is planned according to the child’s usual activity and sleep patterns. The simplest way to ensure individualized, efficient, high-quality care is to plan at the beginning of the shift the nursing procedures to be done. Identify periods of rest. Share the schedule with parents to allow them to visit at the most advantageous times, such as after a rest period when no special treatments are anticipated.

Provide Comfort: Heart surgery is both painful and frightening for children, and providing comfort is a primary nursing concern. Several incisions are used for heart surgery. A median sternotomy following the sternum down the center of the chest is most common. A ministernotomy opens the lower sternum. A thoracotomy incision is most uncomfortable because it goes through muscle tissue. It allows access to the side of the chest through an incision that runs from under the arm around the back to the scapula.

Adequate pain control decreases postoperative complications such as atelectasis, pneumonia, and deep vein thrombosis by improving coughing and ambulation. Pain level is now considered the fifth vital sign. Many pain assessment tools are available for infants and children of different ages. (See Pain Assessment, Chapter 7.)

Continuous IV infusion of opioids, particularly morphine and fentanyl, is a safe and effective method of pain control. Patient-controlled analgesia may be used with children old enough to understand the concept (Macfadyen and Buckmaster, 1999). Epidural morphine is another option. Children receiving opioid infusions for a prolonged period are weaned slowly from the medication to prevent withdrawal symptoms. Nonsteroidal antiinflammatory drugs (NSAIDs) such as IV ketorolac (Toradol) or oral ibuprofen may be used to provide relief of moderate postoperative pain.

Most patients need IV analgesics for pain control during the 24- to 48-hour postoperative period. After lines and tubes have been removed and when patients are tolerating oral fluids, pain may be controlled with oral narcotics such as codeine or oxycodone, often combined with acetaminophen or an oral NSAID such as ibuprofen, or with acetaminophen alone. As noted earlier, thoracotomy incisions are usually more painful than sternotomies because the incision is through muscle. Higgins, Turley, Harr, and colleagues (1999) have found that round-the-clock use of acetaminophen or ibuprofen to augment narcotics is advantageous in providing pain relief after heart surgery. Acetaminophen or ibuprofen alone is usually adequate for pain control after discharge. (See Pain Management, Chapter 7.)

In addition to providing pharmacologic pain control, make every effort to minimize the discomfort of procedures by other means, such as by placing a firm pillow or favorite stuffed animal against the chest incision during coughing and performing treatments after pain medication is given, preferably at a time that coincides with the drug’s peak effect. Employ nonpharmacologic measures to lessen the perception of pain, and encourage parents to comfort their child as much as possible. (See Pain Management, Chapter 7.)

Monitor Fluids: Intake and output of all fluids must be accurately calculated. Intake is primarily IV fluids; however, the nurse also needs to keep a record of fluid used to flush the arterial and CVP lines or to dilute medications. Monitoring of output includes hourly recordings of urine (usually a Foley catheter is inserted and attached to a closed collecting device), drainage from chest and nasogastric tubes, and blood drawn for analysis. Urine is analyzed for specific gravity to evaluate the kidneys’ concentrating ability and to assess the body’s degree of hydration. Renal failure is a potential risk from a transient period of low cardiac output.

During open-heart surgery, the cardiopulmonary pump is primed with a large volume of fluid (usually electrolyte solution), which may greatly dilute the patient’s blood. The large amount of fluid also diffuses into the interstitial spaces, causing total-body edema and pulmonary edema. Patients return from the operating room with fluid overload. Fluids are restricted to less than maintenance level during the first postoperative day, and drugs are used to promote diuresis. A return to maintenance fluid levels then occurs over the next few days as patients resume normal nutrition. Electrolyte levels are closely monitored because electrolyte imbalances, especially hypokalemia, are a common result of diuresis and fluid shifts, and electrolytes may need replacement.

Fluid requirements are based on the child’s weight and body surface area. The child is weighed daily, preferably in the morning, using the same scale and in similar clothing. The child is usually given nothing by mouth for the first 24 hours. Oral fluids are usually withheld until the child is extubated. Patients begin taking clear liquids when bowel sounds are heard and advance slowly to a regular diet. Nausea and vomiting are common in the first few days after surgery, likely a side effect of anesthesia and analgesics. Providing adequate nutrition, ideally by oral intake, becomes important by the fourth or fifth postoperative day. Consider nasogastric tube feedings or parenteral nutrition for patients who are unable to tolerate oral feedings.

Plan for Progressive Activity: Fatigue and weakness are common after heart surgery. However, moderate activity is essential to prevent pulmonary and vascular complications. Initially, turning, coughing, and deep breathing are sufficient to promote respiratory expansion. Passive range-of-motion exercises, especially to the lower extremities, are instituted to prevent venous stasis.

A progressive schedule of ambulation and activity is planned, based on the child’s preoperative activity patterns and postoperative cardiovascular and pulmonary function. Provide the child with toys to encourage movement. It is important to plan the activity at times when the child is well rested, is comfortable (usually has had analgesic medication), and is not scheduled for any strenuous procedure or treatment immediately afterward.

Ambulation is initiated early, usually by the second postoperative day, after extubation and when many lines and tubes have been removed. Patients progress from sitting on the edge of the bed and dangling the legs to standing up and to sitting in a chair while being assisted and assessed by the nursing staff. Carefully monitor the heart rate and respirations to assess the degree of cardiac demand imposed by each activity. Tachycardia, dyspnea, cyanosis, desaturation, progressive fatigue, or dysrhythmias indicate the need to limit further energy expenditure. After ambulation a rest period is scheduled.

Observe for Complications of Heart Surgery: Several complications can occur after heart surgery, most of which are related to open-heart surgery and the use of cardiopulmonary bypass. Many of the procedures discussed in the preceding paragraphs are aimed at preventing these problems. Only those that have not already been discussed are included here. A serious complication, infective endocarditis (bacterial), is discussed on p. 1382.

Pathophysiology

The microorganisms in IE usually grow on a section of the endocardium that has been subjected to abnormal blood streaming and turbulence, such as occurs when blood flow is restricted by an anatomic narrowing or forced through an abnormal opening. Growth may also begin where the abnormal jet of blood strikes the opposing endocardium, causing thickening of the lining or damage to the valvular endothelium. Changes in the endocardium predispose it to the deposition of platelets and fibrin and ultimately make the area susceptible to the growth of invading organisms.

Organisms may enter the bloodstream from any site of localized infection. Transient bacteremia also occurs with trauma to mucosal surfaces encountered in normal daily activities (brushing, flossing gums, chewing, etc.). The microorganisms grow on the endocardium, forming vegetations. The lesion may grow to invade adjacent tissues, such as aortic and mitral valves and myocardium, and may break off and embolize elsewhere, especially in the spleen, kidney, central nervous system, lung, skin, and mucous membranes.

Clinical Manifestations

The onset of symptoms is usually insidious, with unexplained low-grade, intermittent fever. Other common nonspecific symptoms are malaise, myalgias, arthralgias, headache, diaphoresis, and weight loss (Day, Gauvreau, Shulman, et al, 2009). Sometimes, children can manifest IE more acutely with high fevers and rapidly declining health, requiring immediate hospitalization and treatment.

A new murmur or a change in a previously existing one is frequently found as a result of damage to valves or perforation of the myocardium. Another finding, especially in those with prolonged illness, is splenomegaly. Other signs that result from embolus formation elsewhere in the body include splinter hemorrhages (thin black lines) under the nails, Osler nodes (red, painful intradermal nodes with white centers found on the pads of the phalanges), Janeway spots (painless hemorrhagic areas on the palms and soles), and petechiae on the oral mucous membranes, although these signs are less common in children than in adults. Neonates may have feeding difficulties, respiratory distress, tachycardia, HF, or symptoms of septicemia (Ferrieri, Gewitz, Gerber, et al, 2002).

Diagnostic Evaluation

A combination of laboratory and other findings may support the diagnosis of IE, such as ECG changes (AV block), anemia, an elevated erythrocyte sedimentation rate, leukocytosis, microscopic hematuria, and radiographic evidence of cardiomegaly. Definitive diagnosis can be made after growth of the organism and identification of the causative agent in the blood. Several blood specimens (three are recommended) are drawn for culturing to rule out contamination during venipuncture and dilution. Strict sterile technique is practiced in obtaining cultures to avoid contamination. As soon as an organism is isolated, sensitivity studies are done to determine appropriate antibiotic therapy. Vegetation formation and myocardial abscess may be visualized on 2-D echocardiography. Transesophageal echocardiography is used in patients with unsatisfactory transthoracic echo windows. Echocardiographic findings include new or increasing valvular insufficiency, vegetations, and abscesses. A diagnosis of culture-negative endocarditis is made when the patient has echocardiographic or clinical evidence of IE but no organism can be cultured (Ferrieri, 2002).

The Duke criteria are guidelines for the diagnosis of IE in adults and are useful in the diagnosis of childhood endocarditis as well. These criteria divide signs and symptoms into major and minor criteria. The two major criteria are positive blood culture results and echocardiographic evidence of endocardial involvement (vegetations or new valvular regurgitation); minor criteria include fever, predisposing risk factors, and vascular and immunologic signs (Durack, Lukes, and Bright, 1994).

Therapeutic Management

Treatment for IE includes the administration of high-dose antibiotics given intravenously for 2 to 8 weeks to completely eradicate the infecting microorganism (Taubert and Gewitz, 2008). Infectious disease specialists should be consulted to assist in determining the appropriate regimen for each patient. Blood cultures are performed periodically to evaluate the response to antibiotic therapy. In cases in which antibiotic therapy is unsuccessful, HF develops, valvular obstruction is present, or recurrent systemic emboli occur, surgical intervention is warranted.

Early medical treatment for IE is successful in many patients. However, cases diagnosed late; those caused by antibiotic-resistant organisms or fungi; or those that involve HF, embolic events, or significant valvular dysfunction carry a higher mortality rate and may necessitate surgical intervention. Death is most often caused by HF, myocardial infarction from coronary emboli, or cardiac perforation. Nonfatal complications result from embolism to other structures, especially to the central nervous system (causing hemiplegia, aphasia, meningitis, convulsions), kidney (resulting in hematuria, proteinuria), spleen, and bowel.

Prevention of Endocarditis

The American Heart Association has established new guidelines for the prevention of IE (Wilson, Taubert, Gewitz, et al, 2007). The new guidelines no longer recommend antibiotic prophylaxis for all patients with CHD. The data reviewed in the recent AHA statement conclude that antibiotic prophylaxis prevents only a small percentage of the cases of IE and that the risk of widespread use of antibiotics exceeds the potential benefit. The majority of cases of IE occur randomly rather than in association with a particular procedure. Therefore the new guidelines now recommend antibiotic prophylaxis only in those patients with the highest risk of adverse outcomes if they get IE (Wilson, Taubert, Gewitz, et al, 2007). High-risk patients are defined as those patients with cardiac conditions listed in Box 34-8.

Prevention in these high-risk patients involves administration of prophylactic antibiotic therapy 1 hour before dental procedures (Table 34-5). If a patient is taking an antibiotic for another reason, it is recommended that they wait 10 days after antibiotics are completed to have a dental procedure so that normal flora can regenerate. If a patient requires chronic antibiotic therapy, a different antibiotic should be used for prophylaxis. Additional procedures that require prophylaxis (only in this high-risk group) include invasive procedures of the respiratory tract and procedures on infected skin or musculoskeletal tissue. Antibiotic prophylaxis is no longer recommended for gastrointestinal-genitourinary procedures. Maintenance of excellent oral hygiene is still of utmost importance, especially in children with CHD.

Nursing Care Management

The objective of nursing care is to counsel parents of high-risk children concerning the need for prophylactic antibiotic therapy before procedures such as dental work. The family’s regular dentist should be advised of existing cardiac problems in the child as an added precaution to ensure preventive treatment in appropriate patients. These children should also maintain the highest level of oral health to reduce the chance of bacteremia from oral infections. (See also discussion on dental care in Chapter 14.)

Parents should also have a high index of suspicion regarding potential infections. Without unduly alarming them, the nurse should educate patients and families to bring unexplained fever, weight loss, or change in behavior (lethargy, malaise, anorexia) to the practitioner’s attention. Such symptoms should not be self-diagnosed as a cold or flu, and children at risk (e.g., those with CHD) should have blood drawn for culture if they have a fever without an obvious source. Early diagnosis and treatment are important in preventing further cardiac damage, embolic complications, and growth of resistant organisms.

Treatment of endocarditis requires long-term parenteral antibiotics. In some cases IV antibiotics may be administered at home via a peripherally inserted central catheter, with nursing supervision for part of the treatment course. Nursing goals during this period include (1) preparation of the child for IV infusion, usually with an intermittent-infusion device, and performance of several venipunctures to draw blood for culture and laboratory values; (2) observation for side effects of antibiotics, especially inflammation along venipuncture sites along with assessments of renal function; (3) observation for complications, including embolism and HF; and (4) education regarding the importance of follow-up visits for cardiac evaluation, echocardiographic monitoring, and blood culturing.

Etiology

Strong evidence supports a relationship between upper respiratory tract infection with GABHS and subsequent development of RF (usually within 2 to 6 weeks). In almost all cases of RF a previous infection with GABHS can be documented by laboratory evidence of rising antibody titers. Diagnosis and treatment of GABHS infection prevents RF.

Pathophysiology and Clinical Manifestations

The principal manifestations of RF are observed in the heart, joints, skin, and central nervous system. Inflammatory hemorrhagic bullous lesions, called Aschoff bodies, are formed, which cause swelling, fragmentation, and alterations in the connective tissue. Aschoff bodies are found in virtually all patients with clinical rheumatic activity. These lesions occur in the heart, blood vessels, brain, and serous surfaces of the joints and pleura.

The major cardiac manifestation of RF is carditis involving the endocardium, pericardium, and myocardium. In the acute illness, clinical signs and symptoms reflect valvulitis, myocarditis, and pericarditis. Clinically, rheumatic carditis is most commonly associated with the mitral valve. The presence of an apical systolic murmur reflecting mitral regurgitation is a common clinical finding in acute rheumatic carditis. This murmur is a long, high-pitched, blowing murmur that begins with the first heart sound (S₁) and continues throughout systole. Other murmurs in the acute phase may reflect aortic regurgitation. In addition, myocarditis produces tachycardia that is out of proportion to the degree of fever, especially during rest or sleep. Signs and symptoms of HF may result, and chest radiographic examination may demonstrate cardiomegaly. Signs and symptoms of pericarditis include muffled heart sounds because of pericardial effusion. In addition, the patient may demonstrate a pericardial friction rub and complain of chest pain. Pericardial effusions can be documented by echocardiography. Patients with mitral or aortic valve involvement may experience progressive valvular damage as time passes. Carditis is the only manifestation that can lead to permanent damage (Narula, Chandrasekhar, Rahimtoola, et al, 1999).

The second major manifestation is polyarthritis caused by edema, inflammation, and effusions in joint tissue. The arthritis is reversible and migratory, favoring large joints such as the knees, elbows, hips, shoulders, and wrists. The affected joint is swollen, hot, red, and exquisitely painful for 1 or 2 days, after which a different joint is affected. Joint manifestations usually accompany the acute febrile period, most often in the first 1 to 2 weeks; however, they can persist for 4 weeks in untreated patients.

The third major manifestation is erythema marginatum. This is a distinct erythematous macule with a clear center and wavy, well-demarcated border. This transitory, nonpruritic rash is most often found on the trunk and proximal portion of the extremities.

The fourth major manifestation is the development of subcutaneous nodules, which are small (0.5- to 1-cm), nontender swellings that persist indefinitely after the onset of the disease and gradually resolve with no resulting damage. They are rare but may be found in crops over bony prominences such as the feet, hands, elbows, scalp, scapulae, and vertebrae.

The last major manifestation, which reflects central nervous system involvement, is chorea, referred to as St. Vitus dance or Sydenham chorea. Chorea is characterized by sudden, aimless, irregular movements of the extremities, involuntary facial grimaces, speech disturbances, emotional lability, and muscle weakness that can be profound. It is usually exaggerated by anxiety and attempts at deliberate fine motor activity and is relieved by rest, especially sleep. Chorea is seen almost exclusively in children, with a higher incidence in females (World Health Organization, 2004). The time course of symptoms of chorea are variable, but 75% of patients recover in 6 months.

In addition to these major manifestations, minor manifestations that may support the diagnosis include arthralgia and fever, which may be low grade and which often spikes in the late afternoon. Laboratory findings reflect an inflammatory process. Other vague signs and symptoms include unexplained epistaxis, abdominal pain that may be severe enough to simulate appendicitis, weakness, fatigue, pallor, anorexia, and weight loss.

Diagnostic Evaluation

No single symptom or laboratory test can provide a definitive diagnosis of RF. Rather, the diagnosis is based on a set of guidelines by the American Heart Association (Dajani, Ayoub, Bierman, et al, 1993). These guidelines are designed to aid in the diagnosis only of the initial episode of RF (Ferrieri, 2002) (Box 34-9). Clinical and laboratory findings are divided into major and minor manifestations; evidence of recent streptococcal infection is present in the majority of instances.

Although the majority of patients with RF meet these criteria, in three circumstances exceptions are allowed. In patients who have chorea as the only symptom and in patients who are seen late with continued carditis, the late diagnosis may preclude the presence of supporting manifestations and laboratory findings. Finally, a recurrence of RF in a patient with a previous history of the disorder may not fulfill the standard Jones criteria. However, if a single major or several minor manifestations are seen in a patient who has a history of prior disease along with evidence of recent GABHS infection, the diagnosis may be made without strict adherence to the criteria.

Streptolysin O (O because it is oxygen labile) is a streptococcal extracellular product that produces lysis of the red blood cell. Antistreptolysin O (ASLO) titers measure the concentration of antibodies formed in the blood against this product. Normally the titers begin to rise about 7 days after onset of the infection and reach maximum levels in 4 to 6 weeks. Therefore a rising titer demonstrated by at least two ASLO tests is the most reliable evidence of recent streptococcal infection. Normal values are between 0 and 120 Todd units. Elevations over 333 Todd units indicate recent streptococcal infection in children.

Therapeutic Management

The goals of management include (1) eradication of GABHS (primary prevention), (2) prevention of permanent cardiac damage, (3) palliation of the other symptoms, and (4) prevention of recurrences (secondary prevention) (Gerber, Baltimore, Eaton, et al, 2009).

Penicillin (oral or intramuscular injections) remains the drug of choice, with macrolides or cephalosporins as a substitute in penicillin-sensitive children. Initial therapy includes a full 10-day course of penicillin or an alternative antibiotic.

Children who have had acute RF are susceptible to recurrent RF for the rest of their lives, and therefore prophylactic treatment against RF recurrence is started immediately after the initial course of antibiotics is complete. Secondary prevention involves monthly intramuscular injections of benzathine penicillin G, two daily oral doses of penicillin V, or one daily dose of sulfadiazine-sulfisoxazole or erythromycin. The duration of long-term prophylaxis varies and depends on whether the child has had cardiac involvement (Table 34-6).

The use of SBE prophylaxis should follow the recent American Heart Association guidelines that recommend prophylaxis only for very-high-risk patients. Therefore SBE prophylaxis is no longer recommended for patients with RF unless they have had a valve replacement. (Wilson, Taubert, Gewitz, et al, 2007).

Salicylates are used to reduce fever and discomfort and to control the inflammatory process, especially in the joints. Patients with arthritis from RF are generally responsive to salicylate therapy; however, salicylates should not be administered before diagnosis, since their use may mask the polyarthritis. Administration of prednisone may be indicated in some patients with heart failure. Neither salicylates nor prednisone has been shown to affect cardiac sequelae. Traditionally, bed rest or at least limited activity has been recommended during the acute illness.

Patients who have symptoms of heart failure due to significant valvular heart disease require medical therapy for their HF. They are also at risk for atrial fibrillation and embolic complications. Surgery may be indicated in this group of patients and may include valve repair or valve replacement.

Nursing Care Management

The objectives of nursing care for the child with RF are to (1) encourage compliance with drug regimens, (2) facilitate recovery from the illness, (3) provide emotional support, and (4) prevent recurrence of the disease. Because compliance is a major concern in long-term drug therapy, make every effort to encourage adherence to the therapeutic plan. (See Compliance, Chapter 27.) When compliance is poor, monthly injections may be substituted for daily oral administration of antibiotics, and children need preparation for this often dreaded procedure.

Interventions during home care are primarily concerned with providing rest and adequate nutrition. Usually, once the febrile stage is over, children can resume moderate activity and their appetite improves. If carditis is present, the family must be aware of any activity restrictions and may need help choosing less strenuous activities for the child.

One of the most disturbing and frustrating manifestations of the disease is chorea. The onset is gradual and may occur weeks to months after the illness, sometimes even in children who have not been diagnosed with RF. It may be mistaken for nervousness, clumsiness, behavioral changes, inattentiveness, and learning disability. It is usually a source of great frustration to the child because the movements, incoordination, and weakness severely limit physical skill. The child needs an opportunity to verbalize feelings. Of utmost importance is stressing to parents and schoolteachers that the movements are involuntary and sudden, that the chorea is transitory, and that all manifestations eventually disappear.

Nurses also have a role in prevention, primarily in screening school-age children for sore throats that may be caused by GABHS. This may involve actively participating in throat culture screening programs or referring children with a possible streptococcal infection for testing.

Pathophysiology

KD involves widespread inflammation of the small and medium-sized blood vessels (Connor and McCance, 2000), with the coronary arteries being the most susceptible to damage. During the acute stage of the illness there is progressive inflammation of the small vessels (capillaries, venules, arterioles) along with pancarditis. This inflammation is reflected in the clinical signs and symptoms and in laboratory test results. Inflammatory markers (C-reactive protein level and erythrocyte sedimentation rate) are elevated in the acute illness. The vasculitis progresses to the medium-sized muscular arteries, potentially damaging the walls of the vessels and leading to the formation of coronary artery aneurysms in some children. Initial evidence of enlargement of the coronary arteries by echocardiogram can be detected as early as day 7 of illness. Affected vessels continue to enlarge for several weeks and generally reach their largest diameter approximately 4 to 6 weeks from the onset of fever. Longer duration of fever (most likely reflecting the severity of inflammation) is strongly associated with the development of aneurysms. Aneurysms of the peripheral vessels (axillary, brachial, iliac, cervical, and renal arteries) can occur, although this is rare and usually is seen only in children who also have giant coronary aneurysms (>8 mm). In the acute phase, myocarditis (inflammation of the myocardium) is common. Decreased LV function may be evident on echocardiogram; however, the majority of children do not have clinical signs of heart failure. Ventricular function usually improves after the administration of IV immune globulin (IVIG). Occasionally, however, a child will be seen with severe ventricular dysfunction and/or cardiogenic shock. The systemic inflammation gradually subsides and eventually ceases with normalization of inflammatory markers 6 to 8 weeks from the onset of fever.

Over time, aneurysmal vessels try to heal through multiplication of cells in the vessels in an attempt to restore a “normal” lumen diameter. This process is called myointimal proliferation. The smaller the dilation, the more likely that the vessel will regress to a normal size. However, even if the lumen size is restored, the affected vessel may not be completely normal. The walls of these vessels are thicker and may be subject to scarring and calcification, especially at the distal ends of the aneurysm in patients with large aneurysms.

Almost all of the morbidity and morality resulting from KD are due to cardiac complications and mainly occur in patients who have giant aneurysms (≥8 mm). Coronary thrombosis may result from sluggish blood flow in a dilated or aneurysmal vessel. Over the years, stenosis and scarring may also lead to impeded blood flow, which can result in myocardial ischemia or infarction.

Clinical Manifestations

The course of KD can be divided into three phases: acute, subacute, and convalescent. The acute phase begins with an abrupt onset of high fever that is unresponsive to antibiotics and antipyretics. Over the next week or so, the diagnostic symptoms become evident. The bulbar conjunctivae of the eyes become reddened, with clearing around the iris (limbal sparing). The eyes are generally dry, without significant drainage. Inflammation of the pharynx and the oral mucosa develops, with red, cracked lips and the characteristic “strawberry tongue” (the normal coating of the tongue sloughs off, leaving the large papillae exposed, so that the tongue resembles a strawberry). The rash of KD differs from child to child but is never vesicular and is most often accentuated in the perineum. Often the area affected by the rash may desquamate. In addition, the child’s hands and feet become edematous, and the palms and soles become erythematous. The child may have cervical lymphadenopathy (a single node ≥1.5 cm). The node is not usually very tender or red. To meet “classic” criteria, children have prolonged fever (≥4 days) along with four out of five of the diagnostic criteria. During the acute stage, the child is typically very irritable and inconsolable. This behavior may continue for several weeks. Approximately one third of patients develop a temporary arthritis beginning in the small joints. Cardiac manifestations during this period include myocarditis with resultant potential ECG changes, decreased LV function, pericardial effusion, and mitral regurgitation. Generally, these findings are subclinical, but occasionally children with poor function are seen with symptoms of cardiogenic shock. On physical examination, the child may be tachycardic with a gallop rhythm. The coronary arteries may begin to show enlargement during this phase.

The subacute phase begins with resolution of the fever and lasts until all outward clinical signs of KD have disappeared. If changes in the coronary arteries occur, some enlargement or dilation is generally evident by echocardiography during the second week of illness. Damaged vessels can continue to enlarge and reach their maximum diameter approximately 4 to 6 weeks from the onset of illness. Thrombocytosis and hypercoagulability in a child with expanding aneurysms and disrupted blood flow place him or her at risk for coronary thrombosis. During the subacute period, the child may develop periungual desquamation (peeling that begins under the fingertips and toes) of the hands and feet. Arthritis may be evident during this phase and can affect the larger weight-bearing joints. Irritability persists during this period.

In the convalescent phase the clinical signs of KD have mostly resolved, but the laboratory values are still abnormal. The erythrocyte sedimentation rate and C-reactive protein level may remain elevated, which reflects lingering inflammation. Thrombocytosis may still be present. Arthritis may continue into this stage, and coronary complications may remain a concern as coronary dimensions peak 4 to 6 weeks from the onset of illness. This phase is complete when all blood values return to normal (6 to 8 weeks after onset). At the end of this stage, parents report that the child appears to have returned to normal in terms of temperament, energy, and appetite.

Diagnostic Evaluation

Currently no specific diagnostic test exists for KD. Therefore the diagnosis is established on the basis of clinical findings and associated laboratory results. The criteria in Box 34-10 should be used as guidelines. Many children with KD do not meet standard diagnostic criteria, and infants often have an incomplete presentation. It is therefore important to consider KD as a possible diagnosis in any infant or child with prolonged elevated temperature that is unresponsive to antibiotics and is not attributable to another cause. The American Heart Association recently published guidelines that include a diagnostic algorithm to guide the evaluation and treatment of patients with incomplete symptoms who have prolonged fever or other features of KD (Fig. 34-16).

Associated laboratory findings, when combined with clinical data, can be helpful in making the diagnosis. The typical child with KD is anemic and has a leukocytosis with a “shift to the left” (increased immature white blood cells) during the acute phase. Thrombocytosis with hypercoagulability becomes evident in the subacute phase and peaks approximately 3 weeks after the onset of fever. An elevated erythrocyte sedimentation rate and C-reactive protein level reflect ongoing inflammation and generally persist for 6 to 8 weeks. The erythrocyte sedimentation rate can be further elevated by the administration of IVIG; therefore it is better to measure C-reactive protein level as an indicator of inflammation. Microscopic urinalysis reveals a sterile pyuria with mononuclear cells that will not be evident with a regular dipstick test, since the white blood cells are not polymorphonuclear neutrophils. Transient elevation of liver enzymes may occur during the acute phase, reflecting inflammation of the liver. Examination of cerebrospinal fluid may show aseptic meningitis (presence of inflammatory cells). Albumin levels may be lower than normal.

Echocardiograms are used to monitor myocardial and coronary artery status. Obtain a baseline echocardiogram at the time of diagnosis for comparison with future studies and to evaluate ventricular and valvular function. Common findings on echocardiogram during the acute phase include pericardial effusions, mitral regurgitation, and decreased ventricular function. Follow-up echocardiograms should be performed at approximately 2 weeks after onset and again at 6 to 8 weeks from the onset of fever to determine the diameter of the coronary arteries and to assess LV contractility and valvular function. More frequent studies to assess coronary dimensions may be indicated in patients who have continued fever, those who require retreatment with IVIG, and those with coronary dilation on baseline or other studies.

Therapeutic Management

The current treatment of KD includes high-dose IVIG along with salicylate therapy. High-dose IVIG has been shown to reduce the duration of fever and the incidence of coronary artery abnormalities when given within the first 10 days of illness and optimally within the first 7 days. A single large infusion of 2 g/kg over 8 to 12 hours is recommended (Newburger, Takahashi, Gerber, et al, 2004).

Aspirin is given initially in an antiinflammatory dosage (80 to 100 mg/kg/day in divided doses every 6 hours) to control fever and symptoms of inflammation. The duration of therapy varies among institutions. Once fever has subsided and the child has been afebrile for 48 to 72 hours, the aspirin dosage is generally decreased to an antiplatelet dosage (3 to 5 mg/kg/day). Low-dose aspirin therapy is continued in patients without echocardiographic evidence of coronary abnormalities until the platelet count has returned to normal (6 to 8 weeks). If the child develops coronary abnormalities, low-dose (antiplatelet) salicylate therapy is continued indefinitely. Additional anticoagulation therapy, such as clopidogrel or warfarin administration, may be indicated in children with coronary enlargement. Children with giant aneurysms (>8 mm), who are at the greatest risk for morbidity and mortality, are usually maintained on warfarin and aspirin (Newburger, Takahashi, Gerber, et al, 2004). International normalized ratio (INR) levels are maintained at 2.0 to 3.0 in these patients.

Nursing Care Management

The nursing care of children with KD is challenging. Inpatient care focuses on symptomatic relief, emotional support, diagnostic assistance, medication administration, and education of the child and family.

In the initial phase of illness, the nurse must monitor the child’s cardiac status carefully. Intake and output and daily weight measurements are recorded. Although the child may be reluctant to eat and therefore may be partially dehydrated, fluids need to be administered with care because of the usual finding of myocarditis. Assess the child frequently for signs of HF, including decreased urinary output, gallop rhythm, tachycardia, and respiratory distress. Cardiac monitoring is suggested in the following cases: before the initial ECG and echocardiogram are recorded and shown to be normal, during the infusion of IVIG (because of the large fluid load), in children younger than 1 year of age, and in any child with cardiac symptoms. Sedation is generally required before echocardiography in children younger than to 3 years of age, since the child must remain still for up to 1 hour to obtain adequate visualization of the coronary arteries and cardiac structures and function.

Nursing care focuses primarily on symptomatic relief. To minimize skin discomfort, application of cool cloths and unscented lotions and use of soft, loose clothing are helpful. During the acute phase, mouth care, including application of lubricating ointment to the lips, is important for the mucosal inflammation. Offer clear liquids and soft foods and monitor temperature carefully. It is important to document temperature just before aspirin administration, since fever reflects ongoing inflammation and may indicate the need for further treatment. If the temperature is very high, acetaminophen may be given in addition to high-dose aspirin. (See Controlling Elevated Temperatures, Chapter 27.) If arthritis develops, passive range-of-motion exercise may be indicated and can be done most easily during the child’s bath.

The administration of IVIG should follow the same guidelines as for administration of any blood product, with frequent monitoring of vital signs. Patients must be watched for allergic reactions. (See Table 35-2.) The nurse must monitor cardiac status because of the large fluid volume being administered to patients who may have subclinical myocarditis or diminished LV function. Check patency of the IV line because extravasation can result in tissue damage. Hypercoagulability and venous fragility often make it difficult to maintain IV access in children with KD (Connor and McCance, 2000).

Patient irritability is perhaps the most challenging problem. These children need to be placed in a quiet environment that promotes adequate rest. Their parents need to be supported in their efforts to comfort an often inconsolable child. They may need time away from their child, and nurses can often provide respite care for the family. Parents need to understand that irritability is a hallmark of KD and that they need not feel guilty or embarrassed about their child’s behavior.

Etiology

Hypertension in young children, compared to older adolescents and adults, more commonly is secondary to a structural abnormality or an underlying pathologic process, although the results of screening programs of relatively healthy children have challenged this view. The most common cause of secondary hypertension in young children is renal disease, followed by cardiovascular, endocrine, and some neurologic disorders. As a rule, the younger the child and the more severe the hypertension, the more likely it is to be secondary. The conditions associated with secondary hypertension in children and adolescents are listed in Box 34-11.

The causes of primary hypertension are undetermined. There is evidence that both genetic and environmental factors play a role. The incidence of hypertension is greater in children with a family history of hypertension. African-Americans have a higher incidence of hypertension than Caucasians. In the African-American population hypertension develops earlier and is frequently more severe. Environmental factors that contribute to the risk of developing hypertension include obesity; salt ingestion; smoking; lead exposure; medications, including certain stimulant drugs; and stress.

Clinical Manifestations

Although the clinical manifestations associated with hypertension depend largely on the underlying cause, some observations can provide clues to the practitioner that an elevated BP may be a factor. Adolescents and older children with hypertension may complain of frequent headaches, dizziness, or changes in vision. In infants or young children who cannot communicate symptoms, observation of behavior provides clues, although gross behavioral changes may not be apparent until complications are present. Parents of infants and small children who have been treated for hypertension report that their child had previously been irritable and often indulged in an abnormal degree of head banging or rubbing.

Diagnostic Evaluation

It is clear from the increasing numbers of cases of hypertension and prehypertension being identified in children and adolescents that a BP determination should be a routine part of annual assessment in all children older than 3 years of age. Measure BP in children of any age if they are diagnosed as having or are suspected of having coarctation of the aorta, unexplained heart failure, unexplained heart murmurs, prematurity, unexplained seizures or other neurologic signs, an abdominal mass or masses, edema, ascites, evidence of renal failure, hypernatremia, failure to thrive, possible obstructive sleep apnea, respiratory distress, hyperlipidemia, or unexplained headaches. Because hypertension is strongly associated with obesity, a BMI should be calculated for each child at the routine physical examination.

Before a diagnosis of hypertension is made, measure BP in the sitting position on at least three separate occasions. To obtain an accurate reading, take care to quiet the child or relax the adolescent while the measurement is recorded to avoid false readings caused by excitement. The chief cause of falsely elevated BP readings is the use of improperly fitting, narrow cuffs. Therefore attention to correct measurement technique is essential. (See Blood Pressure, Chapter 6.) Note that a child who is large for his or her age may normally have a higher BP than a child who is of average size. Document upper and lower extremity BP when hypertension is suspected, along with the presence of femoral pulses. Twenty-four-hour BP (ambulatory BP [AMBP]) monitoring devices detect changes in pressure throughout the day and night, and thus may give a more realistic picture. These devices are especially helpful in diagnosing white-coat hypertension and are most easily used with older children or adolescents, who are able to tolerate being attached to an ambulatory monitor. The child or parent should keep a log during AMBP monitoring to document level of activity or, at a minimum, sleep and waking times. AMBP monitors should document readings at least hourly and ideally more often. Evaluation of the child with high BP is aimed at assessment of lifestyle and additional risk factors, detection of potential secondary causes, and documentation of the presence or absence of end-organ effects. Table 34-7 outlines the recommended workup for children with BP over the 90th percentile (National High Blood Pressure Education Program Working Group, 2004).

Take a careful medical history of the child or adolescent, including additional medical problems and current medications, drug use, or smoking. In addition, obtain a thorough family history to screen for other relatives with hypertension or other cardiovascular risk factors. In children with documented hypertension or prehypertension, initial laboratory data are also obtained.

The extent of additional testing depends on the degree of BP elevation. Diagnostic testing may include urinalysis, urine culture, renal function studies such as a creatinine and blood urea nitrogen levels, a lipid profile, fasting glucose level, complete blood count, and electrolyte levels. Additional laboratory data may include urine and blood catecholamine, renin, and aldosterone levels. In children who have significant hypertension, secondary causes should be investigated thoroughly. A renal ultrasonographic scan provides a first-line screen for renovascular hypertension or other renal disease (e.g., polycystic kidneys). Additional renovascular imaging may include a 99mTc dimercaptosuccinic acid (DMSA) scan (to rule out scarring) and/or a renal CT angiogram. Echocardiography is indicated in patients with BPs over the 90th percentile to assess LV hypertrophy. A finding that the child’s LV mass is higher than normal would support the presence of chronically elevated BP, since heart muscle thickens in response to chronic hypertension. A retinal examination may provide additional information about end-organ effects.

Therapeutic Management

Therapy for secondary hypertension involves diagnosis and treatment of the underlying cause. In those cases amenable to surgical repair, the nature of the condition, the type of surgery, and the child’s age are all important considerations. Children or adolescents with consistently elevated BP readings from no known cause (primary hypertension) or those with secondary hypertension not amenable to surgical correction may be treated with a combination of nonpharmacologic and pharmacologic interventions.

Dietary practices and lifestyle changes are important in the control of hypertension both for children and for adults and should be instituted first, except in severe cases.

Because obesity and hypertension are closely related, a weight reduction program is recommended for overweight youngsters. In salt-sensitive children, high salt intake increases the risk of hypertension for those genetically predisposed and aggravates existing hypertension unless salt intake is limited. Regular aerobic exercise augments weight reduction and alone has been shown to normalize BP. The exercise regimen is individualized to the child’s interest. It is helpful to quantify how much time is spent doing sedentary activities (e.g., watching television, using the computer, playing video games) compared with how much time daily is spent in aerobic activities. Stress reduction strategies may be beneficial and include biofeedback and relaxation. Smoking should be avoided.

Drug therapy is initiated with caution in children. Because the long-term effects of antihypertensive agents on children are not known, drug treatment of asymptomatic children with mild or borderline hypertension is not recommended. Antihypertensive drug therapy is indicated for treating those patients who have significant elevations of BP despite nonpharmacologic intervention. This includes children and adolescents with symptomatic hypertension; those with secondary hypertension; those with end-organ evidence of hypertension (e.g., increased LV mass); and those who have significant additional risk factors, such as diabetes (National High Blood Pressure Education Program Working Group, 2004).

The National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) recommends beginning pharmacologic therapy with one drug and adding other agents only if control is not obtained. The goal of therapy is to reduce BP levels below the 95th percentile. If additional risk factors (e.g., diabetes or renal disease) are present, however, BP values should ideally be reduced to less than the 90th percentile.

The oral antihypertensive drugs used most often in children are the ACE inhibitors (lisinopril, captopril, and enalapril), beta blockers (propranolol, atenolol [Tenormin]), calcium channel blockers (amlodipine [Norvasc]), angiotensin receptor blockers (losartan [Cozaar]), and diuretics (hydrochlorothiazide [HydroDIURIL]), to mention a few.

Pharmacologic intervention is tailored to meet the needs of the individual child and is determined by the hypotensive effect produced and the appearance of any side effects. For example, ACE inhibitors and angiotensin receptor blockers have been effective in children with diabetes or certain renal diagnoses, whereas beta blockers and calcium channel blockers are often used by children with a history of migraine headaches (National High Blood Pressure Education Program Working Group, 2004). The goal is to achieve a normotensive state throughout the day without accompanying side effects. For many antihypertensive drugs, minimal data are available regarding side effects in children. Therefore consider any behavioral or physical changes that occur after institution of therapy a possible effect, and revise therapy as needed.

Nursing Care Management

The nurse is a valuable link in the delivery of health care for hypertension in the pediatric age-group. Active in detection, diagnosis, and therapy in any setting—hospital, school, clinic, private office, public health service, and private practice—nurses are frequently the primary contact in well-child care and follow-up clinics. They are often the liaison between the family and the health care services.

A BP measurement should be part of the routine assessment of children over the age of 3 years and in younger children who have risk factors for hypertension. In carrying out the procedure, it is important for the nurse to use the correct cuff size. Any questionable reading is repeated. Ideally, BP should be assessed with the child in the sitting position with both feet on the floor. The right arm should be used for consistency in measurements. In addition, initial comparisons should be made between the upper and lower extremities.

Nursing counseling and guidance of affected children is a challenge. Education aimed at understanding hypertension and its implications over the life span is essential in promoting patient and family compliance with both nonpharmacologic and pharmacologic therapies. (See Compliance, Chapter 27.)

Home BP measurement can facilitate surveillance in youngsters with chronic hypertension and can document the effectiveness of therapy. A family member can be instructed in how to take and record accurate BP measurements, which can decrease the number of trips to a health care facility. This individual needs to understand when to contact the practitioner regarding elevated values. When this option is not feasible, the school nurse can often be a valuable resource in monitoring BP.

The nurse plays an important role in assessing individual families and providing targeted information regarding nonpharmacologic modes of intervention, such as diet, weight loss, smoking cessation, and exercise programs. If extensive dietary counseling is required, the child should be referred to a registered dietitian with expertise in working with children and adolescents. Exercise regimens should be individualized. Schoolchildren and young adolescents generally prefer team sports rather than individual training, which they may view as a burden rather than an enjoyable activity. If peers and family members can participate in any of the management strategies, the child is more likely to comply with the plan.

If drug therapy is prescribed, the nurse needs to provide information to the family regarding the reasons for drug therapy, how the drug works, frequency of BP monitoring, and possible side effects of the medication (Box 34-12). Explain that the drug needs to be taken consistently to achieve any prolonged control of BP. Stress the need for follow-up, especially because antihypertensive therapy can sometimes be safely discontinued if BP remains under control over time.

Learning needs vary greatly depending on developmental levels and individual differences. Some children and families require a great deal of support, education, and guidance, whereas others need only education and periodic follow-up. A positive approach is essential; negative feedback will only alienate the family. Exploring the reasons for difficulty in compliance can often provide realistic alternatives. Continued education, support, and reinforcement for positive behavior are major nursing responsibilities.

• Positive family history of elevated cholesterol or early heart disease

• Cigarette smoking

• Diabetes (type 1 or 2)

• Obesity

• Hypertension

• Chronic inflammatory diseases

• Cancer

• Transplantation

• CHD

• A history of KD

Diagnostic Evaluation and Screening

Diagnosis of hyperlipidemia is based on analysis of blood. A blood specimen for determination of a full lipid profile should be drawn after a 12-hour fast. Total cholesterol and HDL cholesterol values obtained at any time in the nonfasting state are also accurate. It is important to note that lipid values can be affected by febrile illnesses and therefore lipids should not be drawn within 3 weeks of a febrile illness. Average lipid values vary by age and gender, with HDL values decreasing in boys as they go through puberty. Children are considered to have elevated total cholesterol if their total cholesterol value is more than 200 mg/dl and/or their LDL cholesterol value is more than 130 mg/dl. Some providers recommend using age- and gender-specific cutpoints.

Screening of children for hypercholesterolemia remains controversial. Consistent with previous recommendations, current guidelines continue to recommend a two-pronged strategy, providing complementary approaches: (1) a population approach that aims to lower the average levels of blood cholesterol among all American children through population-wide changes in nutrient intake and eating patterns, and (2) an individualized approach based on selective screening (see Evidence-Based Practice box). First presented by the National Cholesterol Education Panel in 1992, selective screening of individuals thought to be at high risk was recommended by the American Academy of Pediatrics in their 2008 statement (Daniels, Greer, and the Committee on Nutrition, 2008). Children more than 2 years old should be screened if they have a first- or second-degree relative with lipid abnormalities or with early cardiovascular disease (<55 years in a man or <65 years in a woman). In addition, perform screening in children with individual risk factors (see Box 34-2). The major change in the recent guidelines is that a full fasting profile is now recommended as the initial screen in children with familial or individual risk factors, including obesity. In addition, the American Academy of Pediatrics endorses screening for children with an unknown family history and those who have disease-state risk factors, such as diabetes (Daniels, Greer, and the Committee on Nutrition, 2008). Individualized treatment for these patients includes lifestyle modification and possibly lipid-lowering medication in the most severely affected.

Therapeutic Management

Treatment of high cholesterol levels in children begins with lifestyle modification. The American Heart Association and the American Academy of Pediatrics recommend a heart-healthy diet for all American children, with dietary counseling recommended for those children with known elevated cholesterol values (Daniels, Greer, and the Committee on Nutrition, 2008). A heart-healthy diet focuses on a balanced intake for children over 2 years old, favoring low-fat dairy products, avoiding trans fats, and reducing sweetened beverages. The recommended diet is rich in fruits and vegetables and whole grains. In addition, children with abnormal LDL cholesterol levels should reduce saturated fat intake to 7% of total calories and dietary cholesterol intake to less than 200 mg/day.

Research supports the benefit of diets low in saturated fats and trans fats and higher in monounsaturated fats (such as those found in olive and canola oil). It is important to provide accurate dietary information to families. Food labeling is confusing. Without counseling, patients tend to replace foods high in fat with foods low in fat but high in simple sugars; this can raise triglyceride values, decreasing the effectiveness of nutritional interventions and providing wasted calories.

Current thinking favors a Mediterranean-type diet, based on whole grains, fruits, and vegetables. In addition, this diet allows the use of monounsaturated fats, such as olive oil and canola oil, which have beneficial effects on HDL cholesterol values. The use of these fats also makes the diet more realistic and helps keep children satiated. Dietary recommendations can be confusing, and individualized guidelines should be provided by a certified nutritionist or dietitian with expertise in lipid management.

If the child’s BMI is elevated, weight management should be addressed. In addition to food choice, portion size may also be an issue for these children. Increased physical exercise should be encouraged (ideally 45 minutes to 1 hour, 5 days a week) and is critical to the success of a weight-loss program. Population recommendations meant to decrease cardiovascular risk have been in place for almost two decades, yet unfortunately the incidence of childhood obesity in this country has tripled during the last 25 years (de Ferranti and Ludwig, 2008). Education and programs aimed at decreasing this trend and increasing heart-healthy living (e.g., diet, exercise, avoidance of smoking) are extremely important to decrease cardiovascular risk for the next generation. Assessment of BMI, education, referral to weight-loss programs if indicated, and follow-up should be an integral part of every well-child visit.

Dietary changes alone can decrease LDL values 10% to 15%; however, it is likely that children with severe genetic hypercholesterolemia will require lipid-lowering medication in addition to lifestyle modification. Lipid-lowering drugs used in children and teenagers include bile acid–resin binders such as cholestyramine (Questran) and colestipol (Colestid), hydroxymethylglutaryl–coenzyme A (HMG-CoA) reductase inhibitors (statins such as atorvastatin, simvastatin, pravastatin). Nicotinic acid can help raise HDL cholesterol but is rarely used in pediatrics because of symptoms of flushing and documentation of elevated liver transaminases.

The American Academy of Pediatrics guidelines (Daniels, Greer, and the Committee on Nutrition, 2008; McCrindle, Urbina, Dennison, et al, 2007) lowered the recommended age for initiation of lipid-lowering medication, including statins, to children as young as 8 years old in patients with:

Bile acid–resin binders act by binding bile acids in the intestinal lumen. Because they are not absorbed by the intestine, they are not thought to produce systemic toxicity and are generally safe for children. Cholestyramine and colestipol are both powders that are mixed with water or juice just before ingestion. Many patients cannot tolerate resin binders because they have a gritty texture and do not dissolve completely in water. Side effects may include constipation, abdominal pain, gastrointestinal bloating, flatulence, and nausea. The average dosage for a child is 4 g three times daily or 6 g twice daily. Colesevelam (Welchol) comes in a pill form (625 mg/tablets); the effective dosage is two or three tablets taken twice a day.

Patients who take resin binders should take one multivitamin supplement daily, since bile acid–binding agents may interfere with the absorption of fat-soluble vitamins. Since they can interfere with absorption of other medications, any other medications should be given at least 1 hour before or 6 hours after the bile acid–binding agent is ingested. The results of a complete blood count; chloride and folate levels; and serum concentrations of vitamins A, D, and E should be evaluated yearly.

HMG-CoA reductase inhibitors (statins) continue to be the most effective medication for lipid lowering and are the first-line treatment for adults. Statins are being used more commonly in children and adolescents, especially those with severe familial dyslipidemia. Statin medications are most effective if taken in the evening. Because statins are metabolized by the liver, baseline liver function tests (alanine aminotransferase, aspartate aminotransferase) results, as well as creatine kinase levels, are obtained before the initiation of therapy. These tests, along with a fasting lipid profile, are repeated at approximately 4 weeks and 8 weeks after the initiation of therapy and with any dosage changes. Once the patient is taking a stable dosage, laboratory tests are repeated at 6-month intervals. In addition to elevation of liver transaminase levels, other potentially serious side effects include rhabdomyolysis, which can cause renal failure. Rhabdomyolysis has been reported rarely in adult patients. Instruct patients to discontinue the medication and contact their clinician if they experience the new onset of muscle aches or dark brown urine. In addition, female patients need to be educated that statins are considered teratogenic and cannot be taken during pregnancy. Oral contraceptives may be given in conjunction with lipid-lowering medication; however, they may increase lipid values, potentially necessitating modification of the statin dosage.

A relatively new drug, ezetimibe, works by inhibiting cholesterol absorption. It lowers LDL levels by preventing intestinal uptake of dietary and biliary cholesterol. Recommended use is in combination therapy with a statin, further lowering LDL values. This medication is currently approved for children older than 10 years of age with extremely severe hyperlipidemia. However, clinical trials have shown inconclusive evidence regarding this drug’s clinical benefit. Several large clinical trials are in process, and more information should be available in the next few years.

Nursing Care Management

Nurses play an important role in the screening, education, and support of children with hyperlipidemia and their families. When a child is referred to a lipid clinic, it is essential that the family be adequately prepared for the first visit. Generally, the parents are asked to keep a dietary history for the child before this visit. Sometimes they need to complete a questionnaire regarding the child’s normal dietary habits over the preceding year. Families are instructed to keep their child fasting for at least 12 hours before screening. Therefore it is important to schedule the blood test early in the morning and to arrange for nourishment immediately thereafter. At the visit, a complete individual and family health history is taken. The family history should include both biologic parents and all first-degree relatives. Questions are asked about early heart disease, hypertension, CVAs, sudden death, hyperlipidemia, diabetes, metabolic syndrome, and endocrine abnormalities. Nurses may also uncover risk factors when obtaining a health history for other purposes. It is therefore important that nurses be familiar with current screening practices and with available resources for children with positive family histories.

Parents and extended families should be informed about cholesterol and hyperlipidemia. This education should include a brief introduction to the different lipoprotein categories, including cholesterol, HDL, LDL, and triglycerides. Also, review behavioral risk factors for heart disease, such as smoking and lack of exercise. For management to be effective, parents and older children need to understand the rationale for dietary and pharmacologic intervention in the prevention of future cardiovascular disease.

Nutritional education is part of the treatment of any child or teenager with high cholesterol and ideally should be provided by a nutritionist with expertise in lipid disorders. Dietary compliance may become an issue of control and a source of great stress for many families, particularly for teenagers. Children with high cholesterol levels should not be viewed as having a disease. Instead, emphasize the positive aspects of healthy eating, regular exercise, and avoidance of smoking. Encourage basic dietary changes for the whole family, so that the affected child is not singled out. The focus is positive, with emphasis on making healthy dietary choices, such as substituting chicken and fish for hot dogs and hamburgers and substituting frozen yogurt for ice cream (Box 34-14). Cultural differences must be considered and recommendations individualized. For example, it is more realistic to suggest frying food in a monounsaturated oil such as canola oil than to forbid frying food altogether in families in which this is common practice. Emphasize substitution rather than elimination. Visual aids (e.g., test tubes depicting the amount of fat in a hot dog) are often helpful, especially for children. Diets should be flexible and individually tailored by a nutritionist experienced in combining recommendations that meet both the nutritional demands of the growing child and lipid modifications. Encourage parents to participate in dietary and educational sessions, ask questions, and share ideas and experiences.

Parents often feel guilty about the hereditary component of hyperlipidemia. Many of these same parents believe they have failed if diet alone is not making a significant difference in their child’s lipid profile. They are reassured that a dietary approach alone is often not sufficient, especially for children with values higher than the 95th percentile.

Parents of children who require pharmacologic therapy must understand the purpose, dosage, and possible side effects of the various drugs. Medication schedules should remain flexible and should not interfere with the child’s daily activities. As an example, children of elementary school age may have better compliance if they take a resin-binding agent (e.g., cholestyramine, colestipol) twice a day (i.e., before school and at night) rather than the standard three times a day. Follow-up phone calls by the nurse between visits allow parents to discuss their concerns and ask any questions that have arisen.

Diagnostic Evaluation

Before diagnosing an infant or child with an abnormal heart rate, nurses must be familiar with the standards for normal heart rate in the particular age-group. (See inside back cover.) Heart rate patterns considered normal for a particular child can vary tremendously. An initial nursing responsibility is recognition of an abnormal heartbeat, either in rate or in rhythm. When a dysrhythmia is suspected, the apical rate is counted for 1 full minute and compared with the radial rate, which may be lower because not all of the apical beats are felt. Consistently high or low heart rates should be regarded as suspicious. Accurate nursing assessment is essential. The patient should be placed on a cardiac monitor with recording capabilities. A 12-lead ECG yields more information than the monitor recording and should be taken as soon as possible. Recent advances in bedside telemetry allow storage of ECG tracings for later analysis.

Several advances in the diagnosis of cardiac dysrhythmias have greatly improved the understanding and treatment of these conditions in children. The basic diagnostic procedure is the ECG, including 24-hour Holter monitoring. However, more definitive procedures include both noninvasive and invasive techniques.

Electrophysiologic cardiac catheterization allows identification of conduction disturbances and immediate investigation of drugs that may control the dysrhythmia. Electrode catheters are introduced intravenously and directed toward the right side of the heart. The heart is then selectively stimulated to induce dysrhythmias. Once a dysrhythmia occurs, different antidysrhythmic drugs are administered intravenously to monitor which pharmacologic agent is most successful in terminating the dysrhythmia.

Another procedure that may be employed is transesophageal recording. An electrode catheter is passed to the lower esophagus and, when in position at a point proximal to the heart, is used to stimulate and record dysrhythmias.

The onset and diagnosis of a cardiac dysrhythmia are frightening experiences for parents and older children. Sometimes the dysrhythmia rapidly leads to heart failure and a medical crisis. In this situation parents need much support to express their feelings and to understand the diagnosis and its treatment. Often parents and children have an unspoken fear of potential death even if the dysrhythmia is benign, and repeated explanations are needed to relieve anxiety.

Clinical Manifestations

The clinical manifestations include dyspnea with exercise, chest pain, and syncope. Dyspnea is the most common symptom and is caused by impaired oxygen delivery. Chest pain is the result of coronary ischemia in the RV from severe hypertrophy. Syncope reflects a limited cardiac output leading to decreased cerebral blood flow. Right-sided heart dysfunction is steadily progressive as the pulmonary vessels become obstructed and the pulmonary artery pressure increases. The RV hypertrophies to attempt to maintain a normal cardiac output. With time and continued increases in pulmonary vascular resistance, the cardiac output decreases. When signs of right-sided heart failure with systemic venous congestion and edema are evident, the prognosis is poor.

Therapeutic Management

Although no cure is known, several therapies have shown promise in slowing the progression of the disease and improving quality of life. In general, situations that may exacerbate the disease and cause hypoxia are avoided. Exercise prescriptions are specific to each patient. Patients should avoid high altitudes because of the relative hypoxia, and some patients have moved to sea level to slow the progress of the disease. Supplemental oxygen is commonly used to relieve hypoxia, especially at night while sleeping. Patients with PAH are at risk for thromboembolic events. Anticoagulation therapy has been shown to increase survival in adults. Many patients are treated with warfarin to prevent pulmonary embolism, which can be fatal. Digoxin and diuretics are often used to treat right-sided heart failure.

A number of new drug treatments have been used in this patient population in the past decade and have shown promise in improving both quality of life and survival. Medications are often used in combination, and different drugs are used at different stages of illness. Current research efforts are expanding the understanding of the disease and offering new treatments. Several medications are in clinical trials. Evidence-based guidelines for medical therapy and reviews of therapies in clinical trials have been outlined (Badesch, Abman, Ahearn, et al, 2004; Galie, Seeger, Naeije, et al, 2004).

For patients who respond to vasodilator drug testing during cardiac catheterization, oral calcium channel blockers have been successful and are the treatment of choice. Some patients eventually become nonresponders and then need another therapy. For patients who are nonresponders in vasodilator testing, bosentan, an endothelin-receptor antagonist, is available; it reduces pulmonary artery pressure and resistance and is safe and well tolerated in children (Barst, Ivy, Dingemanse, et al, 2003). It has been used in combination with IV prostacyclin.

Continuous IV prostacyclin has been used with success in children who did not respond to a trial of vasodilation during catheterization. The drug imitates a natural prostacyclin that dilates smooth muscle. It also prevents thrombus formation. It is given by a continuous IV infusion through an indwelling catheter with a portable battery-operated pump. It has been shown to improve exercise capacity and survival.

Nitric oxide is an endothelium-derived relaxing factor. When inhaled, it can relax pulmonary vascular smooth muscle. It is short acting and is inactivated by contact with hemoglobin in the capillary bed. It is a selective pulmonary vasodilator with minimal hemodynamic side effects. Nitric oxide has been used most often in the ICU to manage acute pulmonary hypertensive crisis after congenital cardiac surgery or for diagnostic purposes in the cardiac catheterization laboratory.

Lung transplantation may be another treatment option for children, primarily those with severe disease. Patients with PAH and Eisenmenger syndrome have had a higher early mortality after lung transplantation than other lung transplant patients. Bilateral lung transplantation is the procedure of choice (Doyle, McCrory, Channick, et al, 2004).

As new information is learned and new drugs and new combination therapies are tested and evaluated, the management of patients with PAH will continue to evolve.

Nursing Care Management

The diagnosis of PAH is devastating for the child and family. There is no known cure, and the treatments require significant lifestyle changes and commitment on the part of patient and family to make them successful. Anxiety, depression, and fear of the future are common. Patients and families require extensive education about the disease and its management. They need emotional support to cope with a poor prognosis and make decisions about treatment options.

The medical treatment is complex and involves different medications and therapies. Families are often referred to a specialized center that has experience in the management of PAH. This may involve travel far from home with associated emotional and financial hardships. The patient and family must cope with the symptoms of the disease and the side effects of the treatment. Dealing with a continuous IV infusion or continuous oxygen administration requires a major adjustment in lifestyle to accommodate the therapy. The prostacyclin infusion cannot be interrupted at any time, since symptoms can worsen and cause acute pulmonary hypertensive crisis, which can be fatal. Backup systems must be in place at all times. The patient and family must make a commitment to adhere to a complex regimen of preparing the infusion, maintaining the equipment, and maintaining sterility of the central line. Treatments are expensive, so insurance coverage and financial issues are critical. Nurses have an important role in preparing families to perform these complex therapies. Discharge planning involves many team members and outside agencies. The nurse has a pivotal role in coordinating the child’s care in the hospital and the transition to home.

Therapeutic Management

Treatment is directed at correcting the underlying cause whenever feasible. In most affected children, however, this is not possible, and treatment is aimed at managing HF (see p. 1354) and dysrhythmias. Administration of digoxin and diuretics and aggressive use of afterload-reduction agents have been found to be helpful in managing symptoms in those with dilated cardiomyopathy. The use of beta blockers, specifically carvedilol, is limited in pediatric patients, but one study reported improvement in symptoms in some patients (Bruns, Chrisant, Lamour, et al, 2001). Practice guidelines for the management of heart failure in children have been outlined and provide an in-depth review of available therapies (Rosenthal, Chrisant, Edens, et al, 2004). Digoxin and inotropic agents are usually not helpful in the other forms of cardiomyopathy because increasing the force of contraction may exacerbate the muscular obstruction and actually impair ventricular ejection. Beta blockers such as propranolol or calcium channel blockers such as verapamil (Calan) have been used to reduce LV outflow obstruction and improve diastolic filling in those with hypertrophic cardiomyopathy.

Careful monitoring and treatment of dysrhythmias are essential. The placement of an implantable defibrillator should be considered for patients at high risk of sudden death due to ventricular arrhythmias. Anticoagulants may be given to reduce the risk of thromboembolism, a complication of the sluggish circulation through the heart. For worsening heart failure and signs of poor perfusion, severely ill children may benefit from mechanical ventilation, oxygen administration, IV inotropic support, and IV administration of afterload-reduction agents such as milrinone. Mechanical support devices such as extracorporeal membrane oxygenation or LV assist devices may be used in patients with progressive decline in cardiac status. Extracorporeal membrane oxygenation is employed primarily for infants and younger patients. Its use is limited to several weeks or less because of complications such as bleeding and infection. Ventricular assist devices, currently available for older children and adolescents, can be used for longer periods. Risks include infection and embolic complications. Both devices can be used as a bridge to heart transplantation to allow more time to wait for a donor organ. Heart transplantation may be a treatment option for patients who have worsening symptoms despite maximum medical therapy (see below).

Nursing Care Management

Because of the poor prognosis for many children with cardiomyopathy, nursing care is consistent with that for any child with a life-threatening disorder. (See Chapter 23.) One of the most difficult adjustments for the child may be the realization of failing health and the need for restricted activity, especially if the child is a normally active youngster. Include the child in decisions regarding activity and allow him or her to discuss feelings, particularly if the disease follows a progressive and fatal course. Once symptoms of HF or dysrhythmias develop, implement the same nursing care as discussed on pp. 1354-1360. If cardiac transplantation is being considered, the child and family have great needs in terms of psychologic preparation and postoperative care. The nurse plays an important role in assessing the family’s understanding of the procedure and long-term consequences. Children of school age and older should be fully informed to give their assent to the procedure. (See Informed Consent, Chapter 27.)

• CHD is the most common form of cardiac disease in children and the most common congenital anomaly.

• The most common tests used in assessing cardiac function are radiography, ECG, echocardiography, and cardiac catheterization.

• Cardiac catheterization procedures can be divided into three groups: (1) diagnostic procedures, including angiography, that measure pressures and saturations to establish a cardiac diagnosis; (2) interventional procedures, in which catheters or balloon devices are used to correct cardiac defects; and (3) electrophysiologic procedures for diagnosis and treatment of dysrhythmias.

• Cardiac catheterization provides important information about oxygen saturation of blood within the chambers and great vessels, pressure changes, changes in cardiac output or stroke volume, and anatomic abnormalities.

• Several prenatal factors may increase the child’s risk for CHD: maternal rubella during pregnancy, maternal alcoholism, and maternal type 1 diabetes.

• Congenital heart defects can be divided into four main groups, as determined by hemodynamic patterns: (1) defects that result in increased pulmonary blood flow, (2) obstructive defects, (3) defects that result in decreased pulmonary blood flow, and (4) mixed defects.

• Cardiac output is determined by the interaction of several factors: preload, afterload, contractility, and heart rate.

• Clinical consequences of congenital heart defects include HF and hypoxemia. A child can have both hypoxemia and HF, although usually they occur independently.

• Clinical manifestations of HF are impaired myocardial function (tachycardia, cardiomegaly), pulmonary congestion (dyspnea, tachypnea, orthopnea, cyanosis), and systemic congestion (hepatosplenomegaly, edema, distended veins).

• Nursing measures in the care of a child with HF are to assist in improving cardiac function, decrease cardiac demands, reduce respiratory distress, maintain nutritional status, promote fluid loss, and provide family support.

• Clinical manifestations of hypoxemia are cyanosis, polycythemia, clubbing, and delayed growth and development. The child is at increased risk for hypercyanotic spells, CVAs, and BE.

• Caring for the child with CHD and the family requires helping them adjust to the disorder and cope with the effects of the defect and fostering growth-promoting family relationships.

• Preoperative care of the child with a congenital defect involves introducing the child and family to the hospital and preparing them for preoperative and postoperative procedures.

• Provision of postoperative care includes observing vital signs and arterial and venous pressures, maintaining respiratory status, allowing maximum rest, providing comfort, monitoring fluids, planning for progressive activities, giving emotional support, observing for complications of surgery, and planning for discharge and home care.

• Acquired cardiovascular disorders include IE, RF, KD, systemic hypertension, hyperlipidemia, cardiomyopathy, and cardiac dysrhythmias.

• Prevention of BE in certain children with CHD involves administration of prophylactic antibiotics when specific procedures are performed.

• Acute RF is a systemic inflammatory disease that can damage the cardiac valves and is associated with previous GABHS infection. Its incidence has increased in some areas of the United States.

• KD is an extensive inflammation of small vessels and capillaries that may progress to involve the coronary arteries, causing aneurysm formation. The administration of IVIG is an important aspect of treatment.

• Education of children with hypertension and their families focuses on drug therapy, diet control, and appropriate exercise.

• Cholesterol screening in children is controversial; currently children with known risk factors for hyperlipidemia are screened and treated as needed. The influence of childhood cholesterol levels on later development of CAD is under investigation.

• Cardiomyopathy, or abnormality of the myocardium, is a serious and often fatal disorder. Heart transplantation may offer more favorable options for some children than drug therapy or other treatment regimens.

• Common dysrhythmias in children include slow rhythms (bradycardias, heart block) and fast rhythms (sinus tachycardia, SVT).

• Heart transplantation may benefit infants and children with cardiomyopathy and complex congenital heart defects resulting in severe ventricular dysfunction.

Hypercyanotic Spell

The Infant with a Tachydysrhythmia