Nursing assessment

● SIgns and symptoms depend on the specific type of problem. Although they may present early in life, it is
also possible for them to go undetected until adulthood.
● Common signs:
○ Tachypnea
○ Tachycardia at rest
○ Cyanosis​: need to use proper exam light to check for cyanosis
■ If child is anemic, they may not have enough hemoglobin to appear cyanotic
■ Polycythemic children may appear cyanotic even if O​2​ saturation is normal
○ Fainting/syncope​: often a sign of an undiagnosed heart defect
○ Heart murmur​: necessitates an EKG
○ Activity intolerance
○ Respiratory infections​: due to pulmonary congestion
○ Poor feeding and growth (often the first sign), or weight gain caused by fluid retention and
hepatomegaly
○ Underdevelopment of limbs and muscles
○ Poor urine output due to decreased kidney perfusion
● Work-up for heart defects:
○ Careful history and physical
○ Chest X-ray​: provides information about heart size and pulmonary blood flow patterns
○ Echocardiogram​: high-frequency sound waves produce an image of cardiac structures; can be
used to make a prenatal diagnosis of congenital heart disease
○ EKG​: measure of electrical activity of the heart
○ Cardiac catheterization​: invasive and involves the use of contrast dye; can be both ​diagnostic​ and
interventional​ (e.g., balloon valvuloplasty, coil occlusion)

Cardiovascular disorders
● Congenital heart defects
○ Acyanotic heart defects​: ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus
arteriosus (PDA), coarctation of the aorta, aortic stenosis
■ Left to right shunts
■ Increased pulmonary blood flow (e.g., septal defect or patent ductus arteriosus)
■ Obstruction to blood flow from the ventricles (e.g., coarctation of the aorta or aortic
stenosis)
○ Cyanotic heart defects​: tetralogy of Fallot, truncus arteriosus, transposition of the great vessels
■ Right to left shunts
■ Decreased pulmonary blood flow (tetralogy of Fallot)
■ Mixing of deoxygenated and oxygenated blood within the heart chambers or great arteries
(transposition of the great vessels, truncus arteriosus)
■ Pulmonary stenosis may also be defined as cyanotic
○ Congestive heart failure
● Acquired heart defects
○ Rheumatic fever
○ Kawasaki disease
● Hemodynamic classification:
○ Increased pulmonary blood flow defects​ (VSD, ASD, PDA)
○ Obstructive defects ​(coarctation of aorta, aortic stenosis)
○ Decreased pulmonary blood flow defects​ (tetralogy of Fallot)
○ Mixed defects​ (transposition of the great vessels, truncus arteriosus)
Congenital heart defects​: heart anomalies that develop in utero and manifest at birth or shortly thereafter
● Incidence​: congenital heart defects occur in 4 to 10 children per 1,000 live births. It is the leading cause of
birth defect-related death, and causes twice as many deaths as pediatric cancers. The most common
anomaly is a VSD.
● Etiology​: the cause is usually unknown. Mothers with chronic health conditions such as diabetes or lupus
are more likely to have children with heart disease, and medications such as phenytoin are teratogenic and
may cause heart defects.
○ Multifactorial inheritance
■ Increased incidence of congenital cardiac defects if either parent or a sibling has a heart
defect
○ Environmental factors
■ Maternal exposure to certain agents​: alcohol, lithium, coumadin, retinoic acid
(chemotherapeutic/acne drug), sex hormones, and phenytoin
■ Maternal condition or infection​: viral infections such as rubella*; diabetes mellitus;
systemic lupus erythematosus (SLE); poorly controlled phenylketonuria (PKU)
● *Rubella vaccination can therefore prevent heart defects in a woman’s offspring
○ Genetic factors
■ Trisomy 21 ​(Down syndrome)
■ Turner syndrome ​(XO)
■ DiGeorge syndrome ​(small deletion of chromosome 22)
■ Heterotaxy syndrome​ (organs are located on reverse sides of body--e.g., liver is on the left
instead of the right)
■ VACTERL syndrome​ (associated with several birth defects)
■ Marfan syndrome associated with cardiomyopathy

Ventricular septal defect (VSD)

● Most closely associated with​ Down syndrome ​(one of the reasons more children with Down syndrome
survive today is because we have the means to surgically correct large defects)
● Pathophysiology​: a hole between the ventricles allows oxygenated blood from the left ventricle to be
shunted to the right ventricle and recirculated to the lungs
● Signs​: poor feeding, failure to thrive, tachypnea, fatigue with feeding, heart murmur
○ Can result in​ pulmonary hypertension
○ May not be recognized until adulthood (e.g., when a woman becomes pregnant)
● Small defects may close spontaneously, but large defects can cause ​congestive heart failure ​and require
surgical closure
● Long-term prognosis is good

Atrial septal defect (ASD)

● Pathophysiology​: a hole between the atria shunts oxygenated blood from the left atria to the right ventricle
and recirculates the blood to the lungs. This creates an increased flow of oxygenated blood into the right
side of the heart.
● Signs​: SOB, fatigue (signs of CHF)
○ Usually no symptoms at birth--may not even begin to show symptoms until 30 years of age
● Most defects do not compromise children seriously, but​ surgical closure​ is recommended before the child
is school-age because the defect can lead to significant problems (congestive heart failure or dysrhythmias)
later in life if it goes uncorrected.

Ventricular/atrial septal defect treatment

● Occlusion device​: percutaneous wire “umbrella” is threaded into the heart through the femoral artery to close
the defect
 ● Pericardial patch​: causes blood clot and eventual scarring that closes the defect

Patent ductus arteriosus

● Pathophysiology​: an abnormal opening between the aorta and the pulmonary artery (normal ductus
arteriosus usually closes within 72 hours of birth). If the ductus arteriosus remains patent, oxygenated blood
from the higher-pressure aorta returns to the pulmonary artery and causes ​pulmonary hypertension​ due to
increased blood flow to the lungs.
● May require medical intervention with administration of ​indomethacin​/Indocin (a prostaglandin inhibitor), or
surgical closure​ if the indomethacin does not work. If asymptomatic, the condition may simply be
monitored on an outpatient basis.
○ Coil occlusion​: surgical procedure to close PDA, guided by fluoroscopy

Coarctation of the aorta

● Pathophysiology​: an obstructive narrowing of the aorta, most commonly at the aortic valve or the aorta
near the ductus arteriosus. This causes increased pressure proximal to the defect (blood going to the head
and upper extremities) and decreased pressure distal to the defect (blood going to the rest of the body and
lower extremities).
● Signs​: depend on exactly where in the aorta the obstruction is, but can include hypertension, pallor, leg
cramps, cold feet, and headache
○ A common finding is hypertension in the upper extremities and decreased or absent pulses in the
lower extremities
● May require ​surgical correction​. Mechanical ventilation and inotropic support are often necessary before
surgery. Patients are at risk for hypertension, ruptured aorta, aortic aneurysm, and stroke.

Aortic stenosis
● Pathophysiology​: an obstructive narrowing immediately before, at or after the aortic valve. This results in
diminished oxygenated blood flow from the left ventricle into the systemic circulation (decreased cardiac
output, left ventricular hypertrophy, and resultant pulmonary congestion). The obstruction tends to be
progressive, and sudden episodes of MI can occur with strenuous physical activity.
● Signs​: chest pain, fainting, fatigue, SOB, heart palpitations, murmur
○ Symptoms caused by low cardiac output
● Associated with​ rheumatic fever
● May require ​surgical correction
○ Transcatheter aortic valve replacement (TAVR)​: new valve (artificial or tissue graft) is threaded
through the femoral artery

Pulmonic stenosis
● Pathophysiology​: a heart valve disorder in which outflow of blood into the right ventricle is obstructed at the
pulmonic valve; symptoms are caused by reduction of blood flow to the lungs and ​right ventricular
hypertrophy
○ Relatively rare
● Signs​: heart murmur, chest pain, fainting, fatigue, failure to thrive, SOB
● May require ​balloon valvuloplasty​ or surgical correction

The three T’s of cyanotic heart disease:

● Tetralogy of Fallot​ is a combination of four defects:
○ 1)​ Ventricular septal defect
○ 2) ​Aorta placed over and above the VSD (overriding aorta)
○ 3)​ Pulmonary stenosis that obstructs right ventricular outflow
 ○ 4) ​Right ventricular hypertrophy (the severity of the pulmonary stenosis is related to the degree of
right ventricular hypertrophy and the extent of shunting)
● Truncus arteriosus​, in which one artery (truncus) rather than two arteries (aorta and pulmonary arteries)
arises from both ventricles
● Transposition of the great arteries​, in which the pulmonary artery leaves the left ventricle and the aorta exits
from the right ventricle

Tetralogy of Fallot
● Consists of 4 defects:​ pulmonary stenosis, VSD, overriding aorta, and right ventricular hypertrophy
○ Cyanosis due to unoxygenated blood pumped into the systemic circulation
○ Decreased pulmonary circulation due to the pulmonary stenosis
● Diagnosed with ​echocardiogram​ and corrected with ​staged surgery ​(3-4 different surgeries will be needed
to correct each defect)
● Signs​: cyanosis (especially of lower extremities), SOB, tachypnea, fainting, failure to thrive, murmur,
clubbing, fatigue
○ The child experiences​ “tet” spells​ (hypoxic episodes after crying, feeding, or when agitated) that
are relieved when the child squats or is placed in the knee-chest position. The ​knee-chest
position​ works to improve lung circulation because it increases vascular resistance and decreases
the right to left shunt of deoxygenated blood into the systemic circulation.

Truncus arteriosus
● Pathophysiology​: pulmonary artery and aorta do not separate as they should during gestation: one main
vessel receives blood from both the left ​and right ventricles. This causes blood to mix in the right and left
ventricles through a large VSD, and increased pulmonary resistance results in increased ​cyanosis​.
● If untreated, truncus arteriosus is ​fatal​; only the presence of the large VSD allows for survival at birth..
Surgery​ separates the aorta and pulmonary artery to correct the defect.

Transposition of great arteries

● Great vessels are reversed​: the pulmonary circulation arises from the left ventricle, and the systemic
circulation arises from the right ventricle
● Detected prenatally or within the first few hours after birth
● Incompatible with life (medical emergency) unless there is a coexisting VSD, ASD and/or PDA
● Prostaglandin E ​is given to keep the ductus arteriosus open until the defect can be repaired surgically
(opposite action to indomethacin)

Nursing assessment
● Murmur (present or absent), thrill or rub
● Cyanosis, clubbing of digits (usually after age 2)
● Poor feeding, poor weight gain, failure to thrive
● Frequent regurgitation
● Frequent respiratory infections
● Activity intolerance, fatigue
● Increased heart rate and rhythm; heart sounds
● Pulses (quality and symmetry)
● Blood pressure (upper and lower extremities, right and left)
● History of maternal infection during pregnancy (e.g., rubella)
● Polycythemia is common in children with cyanotic defects (compensatory mechanism)
● Older child/adult will have swollen ankles, but children who do not yet walk will have periorbital and genital
edema
Nursing plans and interventions
● Maintain nutritional status; feed small frequent feedings and provide high-calorie formula
● Maintain hydration (polycythemia increases risk for thrombus formation)
● Maintain neutral thermal environment (isolette may be used)
● Plan frequent rest periods
● Organize activities so as to disturb child only as indicated
● Administer digoxin and diuretics as prescribed
● Monitor for signs of deteriorating condition
● Teach family the need for prophylactic antibiotics prior to any invasive dental or other procedures due to the
risk for endocarditis
● Infants may require ​tube feeding ​to conserve energy
○ Infants being tube fed need to continue to satisfy sucking needs: offer nonnutritive sucking (e.g.,
pacifier)
● Nurse with specialized training should be present at the birth of any infant identified as having a heart defect
while in utero
● Assist with diagnostic tests, and support family during diagnosis
○ EKG
○ Echocardiography
○ Prepare family and child for cardiac catheterization

Cardiac catheterization
● Risks of catheterization are similar to those for a child undergoing cardiac surgery: arrhythmias, bleeding,
perforation, phlebitis, and arterial obstruction at the entry site
● Pre-catheterization assessment:
○ Vital signs, pulse oximetry
○ Accurate height (necessary for correct catheter size) and weight
○ History of allergic reactions (iodine)
○ Symptoms of infection​: may be a reason to cancel the procedure (e.g., diaper rash if femoral
access is required)
○ Mark pedal pulses
○ NPO for at least 4-6 hours before procedure
○ Polycythemic children may need IV fluids to prevent dehydration and hypoglycemia
○ Laboratory analysis​: coagulopathies, CBC, electrolytes
● Post-catheterization problems
○ Cardiopulmonary instability
○ Thrombosis
○ Vessel dissection
○ Bleeding at site
● Post-catheterization nursing care
○ Try to keep legs still for 4 to 8 hours: wrap legs in sheet or have parent hold child
○ Monitor vital signs, observe insertion site; check distal pulses q 15 min for 1 hour, and then q 30
min (this is why you marked the distal pulses before surgery)
○ Observe for bleeding at site, pallor, loss of pulses, coolness of extremity
○ Push fluids to flush dye out of body due to risk for nephrotoxicity; advance diet from clear fluidsd as
tolerated
○ Observe for reactions to dye (vomiting, rash, increased creatinine, decreased urinary output)
○ Pressure on incision site if bleeding occurs (hold pressure 2.5 cm above the percutaneous skin site
to localize pressure over the vessel puncture)
○ Instruct parents to avoid tub baths for several days (may shower)
Nursing plans and interventions for surgery
● Activity after surgery​: ambulation, play
○ “Cardiac cripples:” parents are overprotective and don’t allow child to play; this results in further
deconditioning of child
● Sternal precautions​ for 6 weeks:
○ Do not lift child under arms
○ Do not lift more than 10 lbs
○ Do not pull on arms or reach backwards
● Prepare child as appropriate for age:
○ Show ICU
○ Explain chest tubes, IV lines, monitors, dressings, and ventilator
○ Show family and child waiting area for families
○ Use a doll or drawing for explanations
○ Provide emotional support

Congestive heart failure

● Condition in which the heart is unable to pump effectively the volume of blood that is presented to it
● CHF is a common complication of congenital heart disease. It reflects the increased workload of the heart
caused by shunts or obstructions. Two objectives in treating CHF are to reduce the workload of the heart
and increase cardiac output.
● Clinical manifestations:
○ Low cardiac output; tachycardia at rest; decreased peripheral perfusion (resulting in cold hands and
feet); cardiomegaly due to increased cardiac workload
○ Oliguria
■ Infants: < 1 mL/kg/hr
■ Children and adults: < 0.5 mL/kg/hr
○ Left-sided failure​: symptoms are due to ​pulmonary congestion
■ Tachypnea, retractions, nasal flaring, cough
○ Right-sided failure​: symptoms are due to ​systemic venous congestion
■ Hepatomegaly, edema, pleural/pericardial effusions, jugular venous distention
○ Other signs and symptoms​: diaphoresis, poor feeding, irritability, and failure to thrive
● Nursing assessment: ​tachypnea, shortness of breath, tachycardia, difficulty feeding, cyanosis, grunting,
wheezing, pulmonary congestion, edema (face, eyes of infants), weight gain, diaphoresis (especially head),
hepatomegaly
● Management​:
○ 1) Promote myocardial efficiency​: ​digoxin​, afterload reducers
○ 2) Minimize volume overload​: ​diuretics​, may require fluid/sodium restriction (usually not)
○ 3) Decrease cardiac workload​: minimize energy expenditures by maintaining temperature, limiting
physical activity, treating infections, reducing the work of breathing through positioning, and
keeping child calm
○ 4) Provide adequate nutrition
■ Small, frequent feedings
■ Position during feeding
■ Breast vs. bottle​: supplement breastfeeding with high-calorie feedings, and use a soft
nipple on a bottle to ease the work of suchking. Give the infant 30 minutes to feed and if
the infant is not able to get enough in this time, finish the feeding by gavage. High-calorie
formulas may be from 24 to 30 kcal/oz (whereas regular is generally 20 kcal/oz).
● Gavage​ and ​gastrostomy​ feedings
■ Remember that feeding for a child is like exercise for an adult--it’s exhausting, but without
it the child cannot get stronger
 ■ Children with CHF have an increased metabolic rate due to poor cardiac function
(increased heart rate and respiratory rate)
■ Feedings often hampered by fatigue
● Nursing interventions and plans:
○ Report any unusual weight gain
○ Maintain strict I&O
○ Provide low-sodium diet or formula
○ Gavage feed infants if unable to get adequate nutrition by mouth
○ Continue to care for infant or child with a congenital defect as indicated
○ Monitor vital signs frequently and report signs of increasing distress
○ Assess respiratory functioning frequently; elevate head of bed or use infant seat to prevent
aspiration and easy respiratory effort
○ Administer oxygen therapy as prescribed
○ Administer digoxin and diuretics
■ Managing ​digoxin​: nurse must take child’s apical pulse for 1 minute to assess
bradycardia. Digoxin should be held if the pulse is low for the child’s age (less than 100
bpm for infants, 80 bpm for children, and 60 bpm for adults).
● Therapeutic blood levels are ​0.8 to 2 ng/mL​: blood will need to be drawn
frequently to check levels
● Give digoxin ​before or ​after meals, ​not​ with (do not mix with formula or food)
● Teach parents to administer on a regular basis (do not skip or “make up for”
missed doses); keep in a safe place; and to take the child’s pulse prior to
administration and know when to call the caregiver.
● Signs of digoxin toxicity​: vomiting is a common sign that is often overlooked
because it is mistaken for “spitting up.” Other GI symptoms include anorexia,
diarrhea and abdominal pain; neurological signs include fatigue, muscle
weakness, and drowsiness.
○ Considerations​: digoxin toxicity is often caused by administration of
digoxin and diuretics together (​hypokalemia​); growth spurts make it
difficult to adjust dosage as child ages.
○ Weig frequently (may be every shift for infants); when frequent weighings are required, weigh
patient on the same scale at the same time of day so that accurate comparisons can be made
○ Parent education
■ Managing high stress levels
■ Feeding
■ Medication administration​:​ ​digoxin​, ​furosemide​/Lasix, ​captopril​/Capoten
■ When to call provider​: increased sweating, decreased urine output, poor feeding,
unexplained change in weight, edema, dyspnea at rest, extreme fatigue, coughing at
night, restlessness/confusion, dizziness/fainting, chest pain, and tachycardia.

Rheumatic fever
● An acquired inflammatory disease that can involve the heart, joints, skin, serous surfaces, and brain
● Associated with an antecedent ​beta-hemolytic streptococcal infection​; thought to be caused by antibody
cross-reactivity with the organism
● Most common in the developing world: 325,000 children each year are affected
● Permanent damage to the heart valves (usually the aortic and mitral valves) can occur
● Management: ​goals are to 1) eradicate the hemolytic streptococci, 2) prevent permanent cardiac damage,
3) provide palliation of other symptoms, and 4) prevent recurrences of rheumatic fever.
● Nursing assessment:
○ Occurs 2 to 4 weeks after throat infection
 ○ Fever
○ Painful joints
○ Chest pain, SOB
○ Tachycardia, even during sleep
○ Migratory large joint pain
○ Chorea (irregular involuntary movements)
○ Rash (​erythema​ ​marginatum​)
○ Lab findings​: elevated ​ESR​, elevated ​ASO​ (antistreptolysin O) ​titer​ indicating strep infection
● Nursing plans and interventions:
○ Monitor vital signs
○ Assess for increasing signs of cardiac distress
○ Encourage bed rest (does not need to be strict)
○ Assist with ambulation
○ Reassure child and family that ​chorea​ is temporary
○ Administer medications to eradicate acute infection and to prevent recurrence:
■ Penicillin​ or ​erythromycin ​(an alternative for children who are penicillin-sensitive)
■ Aspirin​ for antiinflammatory and anticoagulant actions (rheumatic fever and Kawasaki
disease are the exceptions to the rule of not giving aspirin to children)
○ Teach home care program: long-term compliance with medication is a major concern
○ Explain the necessity for prophylactics:
■ Antibiotics taken PO or IM
■ IM penicillin G each month
■ Inform dentist and other health care providers of diagnosis so they can evaluate the
necessity of prophylactic antibiotics
○ Penicillin​ ​G​ ​IM​:
■ Prophylaxis for recurrence of rheumatic fever
■ Allergic reactions range from rashes to anaphylactic shock and death
■ Penicillin G is released very slowly over several weeks, giving sustained levels of
concentration
■ Have emergency equipment available whenever medication is administered
■ Always determine existence of allergies to penicillin and cephalosporins; check
chart/record and inquire of client and family

Kawasaki disease (mucocutaneous lymph node syndrome)

● Acute systemic vasculitis affecting skin, mucous membranes, lymph nodes, and coronary arteries; can
damage blood vessels and cause permanent damage to the main arteries of the heart, resulting in ​coronary
artery aneurysm
● Cause of the disease is unknown, but could be an infection that triggers an autoimmune response
● Usually seen in Asian children (common in Japan) younger than 5 years of age: a leading cause of acquired
heart disease in children in the US and Japan
● Early treatment is necessary to decrease the chances of permanent heart damage
● Nursing assessment​:
○ Acute phase
■ High fever for more than 5 days, not responsive to antipyretics or antibiotics
■ Conjunctival redness, “strawberry tongue”
■ Red, swollen hands and feet
■ Peeling of the hands and feet (usually at the end of/after the acute phase)
○ Convalescent phase
■ Starts when all signs of the illness are gone and ends when lab values have returned to
normal (6-8 weeks)
 ○ Child has extreme irritability during the disease process
● Nursing plans and interventions:
○ Administer ​intravenous immunoglobulin​ (​IVIG​): titrate upward slowly and watch closely for a
systemic reaction
■ Effective at reducing the incidence of coronary artery abnormalities when given within the
first 10 days of the illness
■ Single, large infusion of 2 g/kg over 10 to 12 hours recommended
■ Live immunizations will need to be deferred for 11 months after administration of IVIG
because the body might not produce enough antibodies during this period of time
○ Administer high-dose ​aspirin
○ Monitor cardiac status: document child’s I&O and daily weight
○ Minimize skin discomfort with lotions and cool compresses
○ Initiate meticulous mouth care
○ Monitor intake of clear liquids and soft foods
○ Support family as they comfort child during periods of irritability
○ Provide discharge teaching and home referral
○ Most children have a complete recovery. If an aneurysm of the coronary arteries is detected,
echocardiograms will be repeated periodically for several years.
■ Without treatment, coronary artery problems occur in 25% of cases, and 1% of these
children will die