Observe Vital Signs

Vital signs and BP are recorded frequently until stable. Heart rate and respirations are counted for 1 full minute, compared with 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 anesthetics, acid-base and electrolyte imbalance, hypoxia, surgical intervention, or trauma to conduction pathways (p. 896).

At least hourly, the lungs are auscultated for breath sounds. Diminished or absent sounds may indicate an area of atelectasis or a pleural effusion or pneumothorax, which necessitates further medical assessment. Temperature changes are typical during the early postoperative period. Hypothermia is expected immediately after surgery from 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 37.7° 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 commonly done after open-heart surgery. A catheter is passed into the radial artery or other 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.

Several IV lines are inserted preoperatively: a peripheral IV to give fluids and medications, and a CVP, which is usually inserted in a large vessel in the neck. Additionally, intracardiac monitoring lines are sometimes placed intraoperatively in the right atrium, left atrium, or pulmonary artery. Intracardiac lines allow assessment of pressures inside the cardiac chambers, providing vital information about volume status, cardiac output, and ventricular function. All lines must be cared for using strict aseptic technique, and patients must be carefully assessed for bleeding at the time of line removal.

Maintain Respiratory Status

Infants usually require mechanical ventilation in the immediate postoperative period. Early extubation in the operating room or early postoperative period is becoming more common. Children, especially those who did not require cardiopulmonary bypass, may be extubated in the operating room or in the first few postoperative hours. Suctioning is performed only as needed and performed carefully to avoid vagal stimulation (which can trigger cardiac dysrhythmias) and laryngospasm, especially in infants. Suctioning is intermittent and maintained for no more than 5 seconds at a time to avoid 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.

When weaning and extubation are completed, humidified oxygen is delivered by mask, hood, or nasal cannula to prevent drying of mucosa. The child is encouraged to turn and deep breathe at least hourly. Measures are employed to enhance ventilation and decrease pain, such as splinting of the operative site and use of analgesics. Chest tubes are inserted into the pleural or mediastinal space during surgery or in the immediate postoperative period to remove secretions and air to allow reexpansion of the lung. Drainage is checked hourly for color and quantity. Immediately after surgery 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 is greater in extensive heart surgery.

Chest tubes are usually removed on the first to third postoperative day. Removal of chest tubes is a painful, frightening experience. Analgesics such as morphine sulfate, often combined with midazolam (Versed), should be given before the procedure. Older children are forewarned 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 to prevent intake of air into the pleural cavity. 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 on all four sides to the skin so that an airtight seal is formed. It is left on for 1 or 2 days. Breath sounds are checked to assess for a pneumothorax.

Monitor Fluids

Intake and output of all fluids must be accurately calculated. Intake is primarily IV fluids; however, a record of fluid used to flush the arterial and CVP lines or to dilute medications is also kept. 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 assess the concentrating ability of the kidneys and to assess approximately the body’s degree of hydration. Renal failure is a potential risk from a transient period of low cardiac output.

Fluids are restricted during the immediate postoperative period to prevent hypervolemia, which places additional demands on the myocardium, predisposing the patient to cardiac failure. To monitor fluid retention, the child is weighed daily, and the same scale is used at approximately the same time each day to avoid errors in measurement. The child is usually given nothing by mouth for the first 24 hours. If an endotracheal (ET) tube is inserted, oral fluids are usually withheld until the child is extubated. Fluid restriction may be imposed even when oral fluids are given. The nurse calculates the distribution over a 24-hour period based on the child’s preoperative weight and drinking habits. The distribution should allow for most fluid to be given during the child’s most wakeful and active periods.

Provide Rest and Progressive Activity

After heart surgery, rest should be provided to decrease the workload of the heart and promote healing. 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, with periods of rest identified. The schedule should be shared with parents to allow them to visit at the most advantageous times, such as after a rest period when no special treatments are anticipated.

A progressive schedule of ambulation and activity is planned, based on the child’s preoperative activity patterns and postoperative cardiovascular and pulmonary function. Ambulation is initiated early, usually by the second postoperative day, when chest tubes, arterial lines, and assisted ventilatory equipment may be removed. Activity progresses from sitting on the edge of the bed and dangling the legs to standing up and sitting in a chair. Heart rate and respirations are carefully monitored 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.

Provide Comfort and Emotional Support

Heart surgery is both painful and frightening for children, and comfort is a primary nursing concern. Several incisions may be used for heart surgery. A median sternotomy is most common, following the sternum down the center of the chest. 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 from under the arm around the back to the scapula.

Most patients need IV analgesics for pain control during the immediate postoperative period. Patient-controlled analgesia may be used with children old enough to understand the concept. Nonsteroidal antiinflammatory drugs (NSAIDs) such as ketorolac (Toradol) may be used intravenously. Paralyzing agents may also be used with the analgesics for children who are agitated or hemodynamically unstable.

After extubation and removal of lines and tubes, pain can be satisfactorily controlled with oral medications such as ibuprofen, codeine with acetaminophen (Tylenol No. 3), or oxycodone and acetaminophen (Tylox). Acetaminophen alone provides adequate pain relief for most children at discharge. Sternotomy incisions are usually well tolerated, with some discomfort when walking and coughing. Thoracotomy incisions are usually more painful because the incision is through muscle; a more aggressive pain management plan with around-the-clock medications for several days is often necessary to allow for adequate rest, ambulation, and pulmonary hygiene.

In addition to pharmacologic pain control, every effort is made to minimize the discomfort of procedures, such as using a firm pillow or favorite stuffed animal placed against the chest incision during movement and performing treatments after pain medication is given, preferably at a time that coincides with the drug’s peak effect. Nonpharmacologic measures are used to lessen the perception of pain, and parents are encouraged to comfort their child as much as possible. (See also Pain Assessment; Pain Management, Chapter 7.)

Children may become depressed after surgery. This is thought to be caused by preoperative anxiety, postoperative psychologic and physiologic stress, and sensory overstimulation. Typically the child’s disposition improves on leaving the ICU.

Children may also be angry and uncooperative after surgery as a response to the physical pain and to the loss of control imposed by the surgery and treatments. They need an opportunity to express feelings, either verbally or through activity. Children often regress in their behavior during the stress of surgery and hospitalization. They also may express feelings of anger or rejection toward parents. The nurse can support the parents by being available for information and explaining all the procedures to them. The first few postoperative days are particularly difficult because parents see their child in pain and realize the potential risks from surgery. They often are overwhelmed by the physical environment of the ICU and feel useless because they can do so little for their child. The nurse can minimize such feelings by including parents in caregiving activities and comfort and play activities, by providing information about the child’s condition, and by being sensitive to their emotional and physical needs. The importance of their presence in making the child feel more secure is stressed, even if they do not provide physical care.

Pathophysiology

Organisms may enter the bloodstream from any site of localized infection. In the past, endocarditis was believed to be highly associated with invasive procedures; however, endocarditis is most likely to occur from routine exposure to bacteremia associated with usual daily activities, although it can also occur after procedures such as dental work (S. viridans); after invasive procedures involving the gastrointestinal-genitourinary tract; after cardiac surgery, especially if synthetic material is used (valves, patches, conduits); or from long-term indwelling catheters. The microorganisms grow on the endocardium, forming vegetations (verrucae), deposits of fibrin, and platelet thrombi. The lesion may invade adjacent tissues, such as aortic and mitral valves, and may break off and embolize elsewhere, especially in the spleen, kidney, and central nervous system.

Diagnostic Evaluation

The diagnosis of IE is suspected on the basis of clinical manifestations (Box 25-8). Several laboratory findings may suggest IE (e.g., ECG changes [prolonged PR interval], radiographic evidence of cardiomegaly, anemia, elevated erythrocyte sedimentation rate, leukocytosis, microscopic hematuria). Vegetations on the valve and abnormal valve function can often be visualized by echocardiography. Definitive diagnosis rests on growth and identification of the causative agent in the blood.

Therapeutic Management

Treatment should be instituted immediately and consists of administration of high doses of appropriate antibiotics intravenously for 2 to 8 weeks. Blood cultures are taken periodically to evaluate response to antibiotic therapy.

Prevention involves administration of prophylactic antibiotic therapy 1 hour before procedures known to increase the risk of entry of organisms in very high–risk patients. New guidelines only require prophylaxis in patients with the highest risk of poor outcome if they develop endocarditis (Box 25-9). Drugs of choice for prophylaxis include amoxicillin, ampicillin, clindamycin, cephalexin, cefadroxil, azithromycin, and clarithromycin.

Nursing Care Management

Ideally, the objective of nursing care is to counsel parents of high-risk children concerning the signs and symptoms of endocarditis and, in certain cases, the need for prophylactic antibiotic therapy before procedures such as dental work. The family’s regular dentist should be advised of the child’s cardiac diagnosis as an added precaution to ensure preventive treatment. SBE prophylaxis is now reserved for very high–risk patients. Many patients who met criteria established in the past may not require prophylaxis under the new guidelines (Wilson, Taubert, Gewitz, and others, 2007) (see Box 25-9). Parents should be counseled regarding the rationale for discontinuing prophylaxis and should be educated as to the fact that their child is still at risk. It is important that all children with congenital or acquired heart disease maintain the highest level of oral health to reduce the chance of bacteremia from oral infections.

Parents should also have a high index of suspicion regarding potential infections. Without unduly alarming them, the nurse stresses that any unexplained fever, weight loss, or change in behavior (lethargy, malaise, anorexia) must be brought to the practitioner’s attention. Such symptoms should not be self-diagnosed as a cold or flu. 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 drug therapy. In many cases, IV antibiotics may be administered at home with nursing supervision for part of the treatment course. Nursing goals during this period are (1) preparation of the child for IV infusion, usually with an intermittent-infusion device, and several venipunctures for blood cultures; (2) observation for side effects of antibiotics, especially inflammation along venipuncture sites; (3) observation for complications, including embolism and CHF; and (4) education regarding the importance of follow-up visits for cardiac evaluation, echocardiographic monitoring, and blood cultures.

Etiology

Strong evidence supports a relationship between upper respiratory tract infection with GABHS and subsequent development of RF (usually within 2 to 6 weeks). Acute RF is the result of an exaggerated immune response to bacteria in a susceptible host (Carapetis, McDonald, and Wilson, 2005). In almost all cases of RF a previous infection with GABHS can be documented by laboratory evidence of rising antibody titers. Prevention or treatment of GABHS infection prevents RF.

Diagnostic Evaluation

Diagnosis is based on a set of guidelines recommended by the American Heart Association (Newburger, Takahashi, Gerber, and others, 2004). These guidelines, known as modifications of the Jones criteria, suggest that the presence of two major manifestations or one major and two minor manifestations, such as fever and arthralgia, with supportive evidence of recent streptococcal infection, indicates a high probability of RF (see Nursing Care Guidelines box).

Children suspected of having RF are tested for streptococcal antibodies. The most reliable and best standardized test is an elevated or rising antistreptolysin O (ASO or ASLO) titer, which occurs in 80% of children with RF.

Therapeutic Management

The goals of medical management are (1) eradication of hemolytic streptococci, (2) prevention of permanent cardiac damage, (3) palliation of the other symptoms, and (4) prevention of recurrences of RF. Penicillin is the drug of choice, with erythromycin as a substitute in penicillin-sensitive children. Salicylates are used to control the inflammatory process, especially in the joints, and reduce the fever and discomfort. Bed rest is recommended during the acute febrile phase but need not be strict.

Prophylactic treatment against recurrence of RF is started after the acute therapy and involves monthly intramuscular injections of benzathine penicillin G (1.2 million units), two daily oral doses of penicillin (200,000 units), or one daily dose of sulfadiazine (1 g). The duration of long-term prophylaxis is uncertain, but 5 years since the last episode or age 18, longer with cardiac involvement, is suggested by the World Health Organization (Carapetis, McDonald, and Wilson, 2005).

Children who have had acute RF are susceptible to recurrent RF for the rest of their lives and should be followed medically for at least 5 years. Repeated infections are likely to result in rheumatic heart disease. Children and families must be aware of the need for continuing antibiotic prophylaxis for dental work, infection, and invasive procedures.

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 the disease. Because compliance is a major concern in long-term drug therapy, every effort is made to encourage adherence to the therapeutic plan (see Compliance, Chapter 22). 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, after 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 in 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; it sometimes even occurs 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 ability. Of utmost importance is stressing to parents and schoolteachers the involuntary, sudden nature of the movements; 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 caused by GABHS. This may involve actively participating in throat culture screening programs or in referring children with a possible streptococcal infection for testing.

Low-density lipoproteins (LDLs)—These contain low concentrations of triglycerides, high levels of cholesterol, and moderate levels of protein. LDL is the major carrier of cholesterol to the cells. Cells use cholesterol for synthesis of membranes and steroid production. Elevated circulating LDL is a strong risk factor in cardiovascular disease.

High-density lipoproteins (HDLs)—These contain very low concentrations of triglycerides, relatively little cholesterol, and high levels of protein. They transport free cholesterol to the liver for excretion in the bile. High levels of HDL are thought to protect against cardiovascular disease.

Diagnostic Evaluation

Hyperlipidemia is diagnosed on the basis of analysis of blood for a full lipid profile, drawn after a 12-hour fast. A screening thyroid-stimulating hormone (TSH) is useful to rule out hypothyroidism as a cause of secondary hypercholesterolemia. In overweight children, a fasting glucose may be obtained to assess for the potential of metabolic syndrome, which is a combination of multiple symptoms that are associated with increased cardiovascular risk in adults. Blood samples should be collected after having the child sit for 5 minutes, and the tourniquet should be applied immediately before the needle puncture, since posture and vascular stasis may affect results. Diagnostic values for acceptable, borderline, and high total cholesterol and LDL cholesterol levels are listed in Table 25-5.

Screening children for hypercholesterolemia is a controversial issue, with some authorities advocating universal screening and others proposing selective screening. Guidelines from the American Academy of Pediatrics’ Committee on Nutrition (American Academy of Pediatrics, 1998) recommend a strategy that combines two 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).

Therapeutic Management

The first step in the treatment of high cholesterol is oriented to lifestyle modification. The American Academy of Pediatrics (1998) guidelines advocate a heart-healthy diet for all children. Children with known elevated cholesterol should have individual nutritional counseling by a nutritionist with expertise in pediatric lipids.

Research continues to support the benefit of diets low in saturated fats (Van Horn, Obarzanek, Friedman, and others, 2005). Current thinking favors a “Mediterranean”-type diet. Whole grains, fruits, and vegetables form the foundation of this diet. 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. Daily aerobic exercise of at least 60 minutes a day is also recommended for children with high cholesterol. In addition, patients and parents should be counseled regarding the negative effects of smoking (both first hand and second hand).

For children with severe hypercholesterolemia who fail to respond to dietary modifications (after a 6- to 12-month trial), drug therapy may be necessary. Pharmacologic therapy is recommended for children with LDL cholesterol over 190 mg/dl without other risk factors or over 160 mg/dl in patients with two or more other risk factors. In most situations, medication is reserved for boys over 10 years old and for girls once they begin their menses.

In the past, bile acid–binding resins were the only class of drugs recommended for treatment of children. This class of drug acts by binding bile acids in the intestinal lumen. Because they are not absorbed by the intestine, they do not produce systemic toxicity and are safe for children. Cholestyramine (Questran) and colestipol (Colestid) are both powders that are mixed with water or juice just before ingestion. Unfortunately, the vast majority of patients do not get adequate reduction in LDL-cholesterol from bile acid–binding resins. Many cannot tolerate the medication because of the taste; gritty texture; and side effects, the most significant being constipation, abdominal pain, gastrointestinal bloating, flatulence, and nausea. The most recent findings on lipid abnormalities in children recommends treatment with statins if pharmacologic therapy is indicated, using the previously outlined guidelines for treatment (McCrindle, Urbina, Dennison, and others, 2007). Statins are much more effective at lowering LDL cholesterol and triglycerides and raising HDL cholesterol. Statins work by inhibiting the enzyme necessary for cholesterol synthesis. Statins are most effective when taken in the evening and are started at the lowest possible dose in young people. Blood work should be followed closely and should include a fasting lipid profile, liver function tests, and creatinine kinase repeated at 4- and 8-week intervals initially and with dosage changes.

Patients beginning therapy with a statin should be counseled regarding rare but potentially serious side effects such as rhabdomyolysis, elevated transaminases, and elevated creatinine kinase. Patients should discontinue their medication and contact their practitioner if they develop dark urine or new muscle aches. Lastly, statin medications are not safe during pregnancy and therefore sexually active adolescents need to take adequate birth control measures. Very long–term studies are unlikely to be available over decades; however, in the shorter-term studies that have been completed, statins seem to have a similar safety profile for children as they do for adults (McCrindle, Urbina, Dennison, and others, 2007).

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 will be asked to keep a dietary history of the child before this visit. Sometimes they will need to complete a questionnaire regarding the child’s normal dietary habits during the preceding year. Families should be instructed to keep their child fasting for at least 12 hours before screening. It is important to schedule the blood test early in the morning and to arrange for nourishment immediately thereafter. At the visit, a full family history should be taken, including the health of both parents and all first-degree relatives. Specific questions should be asked regarding early heart disease, hypertension, strokes (CVAs), sudden death, hyperlipidemia, diabetes, and endocrine abnormalities.

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, behavioral risk factors for heart disease, such as smoking and exercise, should be reviewed. For management to be effective, parents need to understand the rationale for dietary or pharmacologic intervention. The key is prevention of future cardiovascular disease.

Stringent dietary guidelines may become an issue of control and a source of great stress for many families. Children should not be viewed as having a disease. Rather, the positive aspects of healthy eating, regularly exercising, and avoiding smoking should be emphasized. Basic dietary changes should be encouraged for the whole family so that the affected child is not singled out. Cultural differences must be considered and recommendations individualized. Substitution rather than elimination needs to be emphasized. 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 the lipid modifications. Parents are encouraged 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 also believe they have failed if the diet alone is not making a significant difference in their child’s lipid profile. They need to be reassured that a dietary approach alone is often not sufficient, especially for children with significantly elevated values.

Parents of children who require pharmacologic therapy need to 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. Follow-up phone calls by the nurse between visits allow parents to discuss their concerns and ask any questions that have arisen.

Diagnostic Evaluation

Nurses must be familiar with the standards of normal heart rate for the particular age-group (see inside back cover). An initial nursing responsibility is recognition of an abnormal heartbeat, either in rate or rhythm. When a dysrhythmia is suspected, the apical rate is counted for a 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. 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 done as soon as possible.

The basic diagnostic procedure is the ECG, including 24-hour Holter monitoring. Electrophysiologic cardiac catheterization allows for identification of the conduction disturbance and immediate investigation of drugs that may control 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.

Dysrhythmias can be classified according to various criteria, such as effect on heart rate and rhythm, as follows:

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 right ventricle from severe hypertrophy. Syncope reflects a limited cardiac output leading to decreased cerebral blood flow. Right-sided heart dysfunction is steadily progressive, and when symptoms of venous congestion and edema are present, 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, such as exercise and high altitudes, are avoided. Supplemental oxygen, especially at night while sleeping, is commonly used to relieve hypoxia. Patients are at risk for thromboembolic events leading to pulmonary emboli, so anticoagulation with warfarin (Coumadin) is often prescribed.

Vasodilator therapy (which relaxes vascular smooth muscle and reduces pulmonary artery pressure) can prolong survival of patients with PAH. Oral calcium channel blockers have been successful in some children. Continuous IV prostacyclin has been used with some success in children who did not respond to oral therapy. Both these therapies, although promising, have been used in only small numbers of patients and are expensive. Lung transplantation may be another treatment option.

Therapeutic Management

Treatment is directed toward correcting the underlying cause whenever feasible. However, in most affected children this is not possible, and treatment is aimed at managing CHF (p. 906) and dysrhythmias. Digoxin, diuretics, and aggressive use of afterload reduction agents have been found to be helpful in managing symptoms in those with dilated cardiomyopathy. Practice guidelines for the management of heart failure in children have recently been outlined and provide an in-depth review of available therapies (Rosenthal, Chrisant, Edens, and others, 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 left ventricular 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 (AICD) should be considered for patients at high risk of sudden death due to ventricular dysrhythmias. Anticoagulants may be given to reduce the risk of thromboemboli, a complication of the sluggish circulation through the heart. For worsening heart failure and signs of poor perfusion, IV inotropic or vasodilating drugs may be needed. Severely ill children may require mechanical ventilation, oxygen administration, and IV medications. Heart transplantation may be a treatment option for patients who have worsening symptoms despite maximum medical therapy.

Nursing Care Management

Because of the poor prognosis in many children with cardiomyopathy, nursing care is consistent with that for any child with a life-threatening disorder (see Chapter 18). One of the most difficult adjustments for the child (especially the normally active youngster with hypertrophic cardiomyopathy) may be the realization of failing health and the need for restricted activity. The child should be included in decisions regarding activity and allowed to discuss feelings, particularly if the disease follows a progressively fatal course. After symptoms of CHF or dysrhythmias develop, the same nursing interventions are implemented as discussed on pp. 877–879. If heart transplantation is considered, the needs of the child and family are great 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 22).

Nursing Care Management

Successfully caring for a child after a heart transplant requires the expertise and dedication of many members of the health care team. Nurses play vital roles in assessment, coordination of care, psychosocial support, and patient and family education. The heart transplant recipient must be carefully monitored for signs of rejection, infection, and the side effects of the immunosuppressant medications. The patient’s and family’s psychosocial well-being also needs to be assessed to identify issues such as increased family stress, depression, substance abuse, and school problems. Noncompliance with an intense medication regimen, especially during adolescence, can lead to serious medical problems and can be fatal. Immunosuppressants and nursing implications are discussed in Chapter 27 in relation to renal transplantation. Care of the immunosuppressed child is reviewed in Chapter 26. Psychosocial concerns and appropriate interventions for the child with a life-threatening disorder are presented in Chapter 18.

The first 6 months to 1 year after the transplant are most intense, since the risk of complications is greatest and the patient and family are adjusting to a new lifestyle. Patients are monitored closely by the health care team, with frequent visits and laboratory tests. Care is usually shared between local health care providers and the transplant center. Many patients are able to return to school and other age-appropriate activities within 2 to 3 months after the transplant.

 Stage 1 hypertension includes patients who have BP readings between the 95th and 99th percentiles.

 Stage 2 hypertension describes patients with BP readings over the 99th percentile plus 5 mm Hg.

Etiology

Most instances of hypertension observed in young children occur secondary to a structural abnormality or an underlying pathologic process, although this is being challenged by screening programs of relatively healthy children. The most common cause of secondary hypertension 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 causes of essential hypertension are undetermined, but evidence indicates that both genetic and environmental factors play a role. The incidence of hypertension has been shown to be higher in children whose parents are hypertensive. African-Americans have a higher incidence of hypertension than Caucasians, and in these persons it develops earlier, is frequently more severe, and results in death at an earlier age. Environmental factors that contribute to the risk of developing hypertension include obesity, salt ingestion, smoking, and stress.

Diagnostic Evaluation

From the increasing numbers of hypertensive or potentially hypertensive children and adolescents being identified, a BP determination should be a routine part of annual assessment in healthy children over 3 years old. BP readings should be done in children less than 3 years old who have high-risk family histories or those with individual risk factors, including CHD, kidney disease, malignancy, transplant, certain neurologic problems, or systemic illnesses known to cause hypertension. Although clinical manifestations associated with hypertension depend largely on the underlying cause, some observations can provide clues to the examiner that an elevated BP may be a factor (Box 25-10). In infants and very young children who cannot communicate symptoms, observation of behavior provides clues, although gross behavioral changes may not be apparent until complications are present.

No definitive cutoff values are used in the diagnosis of hypertension in the pediatric patient. The Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents (National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents, 2004) provides normative data for children (see inside cover). BP tables now include the 50th, 90th, 95th, and 99th percentiles for BP readings based on age, gender, and height percentiles. These guidelines are based on auscultatory readings, and therefore this is currently the preferred method of assessment. These charts take into account differences in body height. It is therefore important to note that a child who is large for his or her age may normally have a higher BP than a child of average size. Before a diagnosis is made, BP should be measured on at least three separate occasions.

A careful medical history and family history should be obtained to screen for other relatives with hypertension or other cardiovascular risk factors. In children with suspected hypertension, initial laboratory data include a urinalysis, renal function studies such as creatinine and blood urea nitrogen, a lipid profile, complete blood count, and electrolytes. Depending on the severity of hypertension, additional testing may be indicated. Testing may include a renal ultrasound to measure kidney size and Doppler flow to detect the possibility of a renal cause, a cardiac echocardiogram to evaluate the presence of end-organ involvement such as left ventricular hypertrophy, and a retinal examination.

Therapeutic Management

Therapy for secondary hypertension involves diagnosis and treatment of the underlying cause. In 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 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. Nonpharmacologic measures, such as weight control in overweight patients, increased exercise, limited salt intake, and avoidance of stress and smoking, carry no risk and should be instituted first, except in severe cases. 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.

Drug therapy is instituted with caution in children with significant elevations of BP resistant to nonpharmacologic intervention. The treatment should begin with one drug and should add other drugs only if control is not obtained. The oral antihypertensive drugs used in children include the beta blockers, ACE inhibitors, calcium channel blockers, angiotensin-receptor blockers, and diuretics. The goal is to achieve a normotensive state throughout the day without accompanying drug side effects.

Nursing Care Management

BP measurement should always be a part of the routine assessment of children over 3 years old and patients under 3 years old who are considered to be at high risk for hypertension. To obtain an accurate reading, care is taken 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).

Nursing counseling and guidance of affected children are challenges. Education aimed at understanding hypertension and its implication over the life span is essential in promoting patient and family compliance with both nonpharmacologic and pharmacologic therapies (see Compliance, Chapter 22).

Home BP measurements can facilitate surveillance in youngsters with chronic hypertension and can document effectiveness of therapy. A family member can be instructed in how to take and record accurate BP measurements, thus decreasing the number of trips to a health care facility. This individual needs to understand when to contact the practitioner regarding elevated values. 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 nutritionist 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 be encouraged to participate in any of the management strategies, the child’s compliance is likely to be greater.

Young hypertensive women should avoid oral contraceptives because of their pressor effects. Other options need to be presented before this form of birth control is discontinued (see Contraception, Chapter 17).

If drug therapy is prescribed, the nurse needs to provide information to the family regarding the reasons for it, how the drug works, and possible side effects. General instructions for antihypertensive drugs include:

The need for follow-up is stressed, especially because antihypertensive therapy can sometimes be safely discontinued if BP remains under control over time.

Pathophysiology

The principal area of involvement is the cardiovascular system. During the initial stage of the illness, extensive inflammation of the arterioles, venules, and capillaries occurs. In addition, segmental damage to the medium-sized muscular arteries, mainly the coronary arteries, can occur, causing the formation of coronary artery aneurysms in some children. When death occurs (in< 0.05% of cases), it is usually the result of myocardial ischemia from coronary thrombosis or, over time, severe scar formation and stenosis in coronary aneurysms (Wilder, Palinkas, Kao, and others, 2007).

Clinical Manifestations

Because no specific diagnostic test exists for KD, the diagnosis is established on the basis of clinical findings and associated laboratory results (Box 25-11). These criteria should be used as guidelines. It is important to note that many children with KD do not fulfill 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.

KD manifests in three phases: acute, subacute, and convalescent. The acute phase begins with the abrupt onset of high fever that is unresponsive to antibiotics and antipyretics. The child then develops the remaining diagnostic symptoms. During this stage the child is typically very irritable. The subacute phase begins with resolution of the fever and lasts until all clinical signs of KD have disappeared. During this phase the child is at greatest risk for the development of coronary artery aneurysms. Echocardiograms are used to monitor myocardial and coronary artery status. A baseline echocardiogram should be obtained at the time of diagnosis for comparison with future studies. Irritability persists during this phase. In the convalescent phase, all the clinical signs of KD have resolved, but the laboratory values have not returned to normal. This phase is complete when all blood values are normal (6 to 8 weeks after onset). At the end of this stage the child has regained his or her usual temperament, energy, and appetite.

Therapeutic Management

The current treatment of KD includes high-dose IV gamma globulin along with salicylate therapy. Gamma globulin has been demonstrated to be effective at reducing the incidence of coronary artery abnormalities when given within the first 10 days of the illness. A single, large infusion of 2 g/kg over 10 to 12 hours is recommended. Retreatment with intravenous gamma globulin is indicated in patients who continue with fever after treatment.

Aspirin is given initially in an antiinflammatory dose (80 to 100 mg/kg/day in divided doses every 6 hours) to control fever and symptoms of inflammation. After fever has subsided, aspirin is continued at an antiplatelet dose (3 to 5 mg/kg/day). Low-dose aspirin 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, salicylate therapy is continued indefinitely. Additional anticoagulation (e.g., clopidogrel [Plavix], enoxaparin [Lovenox], or warfarin) may be indicated in children who have medium-sized or giant coronary artery aneurysms.

Nursing Care Management

In the initial phase 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. The child should be assessed frequently for signs of CHF, including decreased urinary output, gallop rhythm (an additional heart sound), tachycardia, and respiratory distress.

Administration of gamma globulin should follow the same guidelines as for any blood product, with frequent monitoring of vital signs. Patients must be watched for allergic reactions. Cardiac status must be monitored because of the large volume being administered to patients with myocarditis and diminished left ventricular function.

Most nursing care focuses on symptomatic relief. To minimize skin discomfort, cool cloths; unscented lotions; and soft, loose clothing are helpful. During the acute phase, mouth care, including lubricating ointment to the lips, is important for mucosal inflammation. Clear liquids and soft foods can be offered.

Patient irritability is perhaps the most challenging problem. These children need 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.

Pathophysiology

A healthy child’s circulatory system is able to transport oxygen and metabolic substrates to body tissues, which require a constant source for these essential needs. The cardiac output and distribution to the various body tissues can change rapidly in response to intrinsic (myocardial and intravascular) or extrinsic (neuronal) control mechanisms. In shock states these mechanisms are altered or challenged.

Reduced blood flow, as in hypovolemic shock, causes diminished venous return to the heart, low CVP, low cardiac output, and hypotension. Vasomotor centers in the medulla are signaled, causing a compensatory increase in the force and rate of cardiac contraction and constriction of arterioles and veins, thereby increasing peripheral vascular resistance. Simultaneously the lowered blood volume leads to the release of large amounts of catecholamines, antidiuretic hormone, adrenocorticosteroids, and aldosterone in an effort to conserve body fluids. This causes reduced blood flow to the skin, kidneys, muscles, and viscera to shunt the available blood to the brain and heart. Consequently, the skin feels cold and clammy, there is poor capillary filling, and glomerular filtration rate and urinary output are significantly reduced.

As a result of impaired perfusion, oxygen is depleted in the tissue cells, causing them to revert to anaerobic metabolism, producing lactic acidosis. The acidosis places an extra burden on the lungs as they attempt to compensate for the metabolic acidosis by increasing respiratory rate to remove excess carbon dioxide. Prolonged vasoconstriction results in fatigue and atony of the peripheral arterioles, which leads to vessel dilation. Venules, less sensitive to vasodilator substances, remain constricted for a time, causing massive pooling in the capillary and venular beds, which further depletes blood volume.

Complications of shock create further hazards. Central nervous system hypoperfusion may eventually lead to cerebral edema, cortical infarction, or intraventricular hemorrhage. Renal hypoperfusion causes renal ischemia with possible tubular or glomerular necrosis and renal vein thrombosis. Reduced blood flow to the lungs can interfere with surfactant secretion and result in acute respiratory distress syndrome (ARDS), characterized by sudden pulmonary congestion and atelectasis with formation of a hyaline membrane. Gastrointestinal tract bleeding and perforation are always a possibility after splanchnic ischemia and necrosis of intestinal mucosa. Metabolic complications of shock may include hypoglycemia, hypocalcemia, and other electrolyte disturbances.

Diagnostic Evaluation

The etiology of shock can be discerned from the history and the physical examination. The severity of the shock is determined by measurements of vital signs, including CVP and capillary filling (Box 25-13). Shock can be regarded as a form of compensation for circulatory failure. Because of the progressive nature of shock, it can be divided into the following three stages or phases:

At all stages the principal differentiating signs are observed in the (1) degree of tachycardia and perfusion to extremities, (2) level of consciousness, and (3) BP. Additional signs or modifications of these more universal signs may be present depending on the type and cause of the shock. Initially the child’s ability to compensate is effective; therefore early signs are subtle. As the shock state advances, signs are more obvious and indicate early decompensation.

Additional signs may be present, depending on the type and cause of the shock. In early septic shock there are chills, fever, and vasodilation, with increased cardiac output that results in warm, flushed skin (hyperdynamic, or ‘hot,’ shock). A later and ominous development is disseminated intravascular coagulation (see Chapter 26), the major hematologic complication of septic shock. Anaphylactic shock is frequently accompanied by urticaria and angioneurotic edema, which is life threatening when it involves the respiratory passages (see Anaphylaxis, below).

Laboratory tests that assist in assessment are blood gas measurements, pH, and sometimes liver function tests. Coagulation tests are evaluated when there is evidence of bleeding, such as oozing from a venipuncture site, bleeding from any orifice, or petechiae. Cultures of blood and other sites are indicated when there is a high suspicion of sepsis. Renal function tests are performed when impaired renal function is evident.

Therapeutic Management

Treatment of shock consists of three major interventions: (1) ventilation, (2) fluid administration, and (3) improvement of the pumping action of the heart (vasopressor support). The first priority is to establish an airway and administer oxygen. After the airway is ensured, circulatory stabilization is the major concern. Establishment of adequate IV access, ideally with multilumen central lines, is essential to deliver fluids and medications.

Nursing Care Management

The child who is in shock requires intensive observation and care. The initial action is to ensure adequate tissue oxygenation. The nurse should be prepared to administer oxygen by the appropriate route and to assist with any intubation and ventilatory procedures indicated. Other procedures and activities that require immediate attention are establishing an IV line, weighing the child, obtaining baseline vital signs, placing an indwelling catheter, obtaining blood gases and other measurements, and administering medications as indicated. The child is best positioned flat with the legs elevated.

The nurse’s responsibilities are to monitor the IV infusion, intake and output, vital signs (including CVP), and general systems assessments on a routine basis. IV medications are titrated according to patient responses, and vital signs are taken every 15 minutes during the critical periods and thereafter as needed. Urinary output is measured hourly; blood gases, hematocrit, pH, and electrolytes are monitored frequently to assess the child’s status and the efficacy of therapy. An apnea and cardiac monitor is attached and monitored continuously. In the initial stages of acute shock, more than one nurse is often needed to manage all the necessary activities that must be carried out simultaneously (see Emergency Treatment box).

Throughout the intense activity, support for the family must not be overlooked. Someone should contact family members at frequent intervals to inform them about what is being done and whether there is any progress. Ideally, someone should remain with the parents to serve as liaison between them and the intensive care team. However, this is not always feasible in such a critical situation. As soon as possible, the family should be allowed to see the child. A member of the clergy or a social worker may be called to help provide comfort and support.

Clinical Manifestations

The onset of clinical symptoms usually occurs within seconds or minutes of exposure to the antigen, and the rapidity of the reaction is directly related to its intensity: the sooner the onset, the more severe the reaction. The reaction may be preceded by symptoms of uneasiness, restlessness, irritability, severe anxiety, headache, dizziness, paresthesia, and disorientation. The patient may lose consciousness. Cutaneous signs of flushing and urticaria are common early signs, followed by angioedema, most notable in the eyelids, lips, tongue, hands, feet, and genitalia.

Bronchiolar constriction may follow, causing narrowing of the airway; pulmonary edema and hemorrhage also may occur. Laryngeal edema with severe acute upper airway obstruction may be life threatening and requires rapid intervention. Shock occurs as a result of mediator-induced vasodilation, which causes capillary permeability and loss of intravascular fluid into the interstitial space. Sudden hypotension and impaired cardiac output with poor perfusion are seen.

Therapeutic Management

Successful outcome of anaphylactic reactions depends on rapid recognition and institution of treatment. The goals of treatment are to provide ventilation, restore adequate circulation, and prevent further exposure by identifying and removing the cause when possible.

A mild reaction with no evidence of respiratory distress or cardiovascular compromise can be managed with subcutaneous administration of antihistamines, such as diphenhydramine (Benadryl) and epinephrine.

Moderate or severe distress presents a potentially life-threatening emergency. Establishing an airway is the first concern, as with all shock states. Epinephrine is given subcutaneously or intravenously as an antihistamine and to support the cardiovascular system and increase BP. Other routes for giving epinephrine are intramuscular and via the airway, either nebulized or injected through an ET tube. In severe anaphylaxis, epinephrine by any route is better than none. Fluids are given to restore blood volume. Additional vasopressors may be given to improve cardiac output.

Prevention of a reaction is preferable. Preventing exposure is more easily accomplished in children known to be at risk, including those with (1) a history of previous allergic reaction to a specific antigen; (2) a history of atopy; (3) a history of severe reactions in immediate family members; and (4) a reaction to a skin test, although skin tests are not available for all allergens. Desensitization may be recommended in certain cases.

Nursing Care Management

When an anaphylactic reaction is suspected, both immediate intervention and preparation for medical therapy are nursing responsibilities. Ventilation is ensured by placing the child in a head-elevated position, unless contraindicated by hypotension, to facilitate breathing and administer oxygen. If the child is not breathing, CPR is initiated and emergency medical services are summoned.

If the cause can be determined, measures are implemented to slow the spread of the offending substance. An IV infusion is established immediately. Emergency medications are given intravenously whenever possible; however, epinephrine may be given subcutaneously (see Emergency Treatment box, above left). Vital signs and urinary output are monitored frequently. Medications are administered as prescribed, with regular assessment to monitor effectiveness and to detect signs of side effects of medication and fluid overload.

To prevent an anaphylactic reaction, parents are always asked about possible allergic responses to foods, latex, medications, and environmental conditions. These are displayed prominently on the patient’s chart. The specific allergen is noted, as is the type and severity of the reaction. Parents are excellent historians, especially when the child has displayed a pronounced reaction to a substance. Drugs, including related drugs (e.g., penicillin, nafcillin), and other items, such as latex, that have produced a reaction previously are never used. If the child is allergic to insect venom, the family is instructed to purchase an emergency kit to be kept with the child at all times. Both the family and the child, if the child is old enough, are taught how to use the equipment. The patient should carry medical identification at all times.

Diagnostic Evaluation

Diagnosis is established on the basis of the criteria established by the Centers for Disease Control and Prevention’s toxic case definition (Box 25-15). A history of tampon use contributes to the diagnosis. Additional laboratory tests include cultures from blood, vagina, cervix, and any discharge. Other laboratory tests are those that facilitate the management of shock.

Therapeutic Management

The management of TSS is the same as management of shock of any cause and may range from supportive care in mild cases to hospitalization and intensive care in severe cases. Appropriate parenteral antibiotics are usually administered after cultures are obtained.

Nursing Care Management

Because the disease is relatively rare, the major efforts of nursing are directed toward prevention. The association between the disease and the use of tampons provides some direction for education. Avoiding the use of tampons offers the most certain preventive measure, although this approach is probably unacceptable to most adolescent girls, who prefer the freedom, comfort, and inconspicuousness that tampons afford.

Adolescent girls who use tampons can be taught general hygiene measures, such as good hand washing and careful insertion to avoid vaginal abrasion. It is wise to modify their use, alternating with sanitary napkins—perhaps using the napkins during the night, when at home during the day, and when flow is slight. Young girls are advised not to use superabsorbent tampons and not to leave any tampon in the body for more than 4 to 6 hours.