Figure 45

FIGURE 45-1 Congenital heart abnormalities
In most cases, the cause of CHD is not known. Some infants and children with CHD may appear
perfectly healthy, whereas others may be critically ill. Most infants and children with CHD can
be successfully managed with medications and surgeries.
ETIOLOGY AND INCIDENCE
 CHD affects 8 to 12 of every 1,000 neonates.

 Exact cause of CHD is unknown in 90% of cases.
 The heart begins as a single cell and develops into a four-chambered pumping system
during the third to eighth weeks of gestation.
 Associated factors for CHD include:
o Fetal or maternal infection during the first trimester (rubella).
o Chromosomal abnormalities (trisomy 21, 18, 13).
o Maternal insulin-dependent diabetes.
o Teratogenic effects of drugs and alcohol.
 Syndromes that include CHD:
o Marfan's syndrome: mitral valve prolapse (MVP), dilated aortic root.
o Turner's syndrome: aortic valve stenosis (AVS), coarctation of the aorta (CoA).
o Noonan's syndrome: dysplastic pulmonary valve.
o William's syndrome: supravalvular pulmonary stenosis (PS).
o DiGeorge syndrome: interrupted aortic arch (IAA), truncus arteriosus,
transposition of great arteries (TGA), tetralogy of Fallot (TOF).
o Down syndrome (trisomy 21): atrioventricular (AV) canal defect, ventricular
septal defect (VSD). About 50% of children with Down syndrome have a CHD.
COMMON CONGENITAL HEART MALFORMATIONS

Congenital heart defects can be classified into three categories: obstruction to blood flow,
increased pulmonary blood flow
(acyanotic lesions), and decreased pulmonary blood flow (cyanotic lesions).
 Obstructive lesions:
o ASâ€”valvular, subvalvular or supravalvular.
o CoA.
o PSâ€”valvular, subvalvular or supravalvular.
o IAA.
 Increased pulmonary blood flow (acyanotic):
o PDA.
o ASD.
o VSD.
o AV canal.
o PAPVR.
 Decreased pulmonary blood flow (cyanotic):
o TOF.
o TA.
o TAPVR.
o TGA.
o Truncus arteriosus.
o HLHS.
o DORV.
BOX 45-1 ABBREVIATIONS USED FOR CONGENITAL HEART DISEASE

AS Aortic stenosis
ASD Atrial septal defect
AV Atrioventricular
CoA Coarctation of the aorta
HLHS Hypoplastic left heart syndrome
IAA Interrupted aortic arch
IVC Inferior vena cava
LVOT
O Left ventricular outflow tract obstruction
PA Pulmonary artery
PAPVR Partial anomalous pulmonary venous return
PDA Patent ductus arteriosus
PFO Patent foramen ovale
PS Pulmonary stenosis
PVR Pulmonary vascular resistance
SVC Superior vena cava
TA Tricuspid atresia
TAPVRTotal anomalous pulmonary venous return
TGA Transposition of great arteries
TOF Tetralogy of Fallot
VSD Ventricular septal defect
AORTIC STENOSIS
Congenital AS may be caused by a bicuspid aortic valve with fused commissures that does not
open completely, by a hypoplastic aortic valve annulus or by stenosis above or below the aortic
valve (subvalvular or supravalvular stenosis). The result is turbulent blood flow across the aortic
valve and into the ascending aorta. Patients with AS must be evaluated for additional left heart
lesions that include CoA, mitral valve stenosis, hypertrophic cardiomyopathy, and hypoplastic
left heart. AS is the most common form of left ventricular outflow tract obstruction. It accounts
for 3% to 6% of congenital heart defects. AS may occur at any age, and it occurs more
commonly in boys than in girls. In most children, it is a progressive lesion that creates LVOTO.
Pathophysiology and Etiology
 Blood flows at an increased velocity across the obstructive valve or stenotic area and into
the aorta.
 During systole, left ventricular pressure rises dramatically to overcome the increased
resistance at the aortic valve.
 Myocardial ischemia may occur because of an imbalance between the increased oxygen
requirements related to the hypertrophied left ventricle (LV) and the amount of oxygen
that can be supplied.
 Left-sided heart failure results in an increased LV end diastolic pressure that is reflected
back to the left atrium and pulmonary veins.
Clinical Manifestations
Neonate
 Severe congestive heart failure (CHF).

 Metabolic acidosis.
 Tachypnea.
 Faint peripheral pulses, poor perfusion, poor capillary refill, cool skin.
 Poor feeding and feeding intolerance.
Child and Adolescent
 Chest pain on exertion, decreased exercise tolerance.

 Dyspnea, fatigue, shortness of breath.
 Syncope, light-headedness.
 Palpitations.
 Sudden death.
Diagnostic Evaluation
 Auscultation.
o Systolic ejection murmur heard best at right upper sternal border, radiates to neck.
o Ejection click.
o S2 splits normally or narrowly.
 Electrocardiogram (ECG): left ventricular hypertrophy (LVH) with a strain pattern may
be seen in severe cases.
 Chest X-ray: increased cardiac silhouette, increased pulmonary vascular markings. A
prominent aortic knob may be seen occasionally from poststenotic dilatation with
valvular AS.
 Echocardiogram: two-dimensional echocardiogram with Doppler study and color flow
mapping to visualize the anatomy and to estimate the gradient across the valve and
through the aorta.
Management
Neonate
 Stabilize with prostaglandin E1 (PGE1) infusion to maintain cardiac output through the
PDA.
 Inotropic support as needed.
 Intubation and ventilation as needed.
 Infective endocarditis prophylaxis (lifelong).
 Cardiac catheterization: aortic balloon valvuloplasty or aortic balloon angioplasty.
 Surgical valvotomy, commissurotomy, or myectomy/myotomy.
Child and Adolescent
 Medical management with close follow-up to monitor increasing gradient across the
aortic valve or through the aorta.
 Restrict strenuous exercise and anaerobic exercise (eg, weight lifting).
 Restrict participation in competitive sports.
 Aortic balloon valvuloplasty or aortic balloon angioplasty.
 Surgical intervention.
o Surgical valvotomy, commissurotomy, or myectomy/ myotomy.
o Aortic valve replacement.
 Mechanical prosthesis (St. Jude valve).
 Ross procedure (pulmonary autograft).
Complications
 CHF and pulmonary edema.

 Dizziness, light-headedness, and syncope.
 Palpitations, arrhythmias.
 Infective endocarditis.
 Sudden death.
COARCTATION OF THE AORTA

CoA is a discrete narrowing or a long segment hypoplasia of the aortic arch, usually in the
juxtaductal position. It accounts for 8% to 10% of congenital heart defects.
 The discrete narrowing or hypoplastic segment of the aorta increases the workload of the
left ventricle (increased LV systolic pressure).
 In a neonate with critical CoA, lower body blood flow occurs through the PDA (right-to-
left shunting).
 In the older child, collateral vessels grow and bypass the coarctation to perfuse the lower
body.
 The neonate with critical CoA (ductal dependent lesion):

o Asymptomatic until the PDA begins to close.
o After PDA closure: severe CHF, poor lower body perfusion, tachypnea, acidosis,
progressive circulatory shock, absent femoral and pedal pulses.
 The child or adolescent with CoA:
o Usually asymptomaticâ€”normal growth and development.
o Hypertension in the upper extremities, with absent or weak femoral pulses.
o Nosebleeds, headaches, leg cramps.
 Auscultationâ€”varies; nonspecific systolic ejection murmur.

 Chest X-rayâ€”cardiomegaly and pulmonary edema or pulmonary venous congestion.
 ECGâ€”varies; normal or right ventricular hypertrophy (RVH) in infants and LVH in
older children.
 Two-dimensional echocardiogram with Doppler study and color flow mapping identifies
area of aortic arch narrowing and associated lesions (bicuspid aortic valve, VSD, PDA).
 Invasive studies (cardiac catheterization) usually not needed to make the initial diagnosis;
may need aortic angiography to identify collateral vessels before surgery.
 Cardiac magnetic resonance imaging may be done to noninvasively assess the location
and degree of narrowing and identify collateral vessels.
Management
Critical Coarctation in the Neonate
 Medical management:
o Resuscitation and stabilization with PGE1 infusion: monitor for complications
related to PGE1 therapy (fever, apnea).
o Intubation and ventilation as needed.
o Infective endocarditis prophylaxis (lifelong).
o Anticongestive therapy (digoxin and Lasix) and inotropic support as needed.
o Assess renal, hepatic, and neurologic function.
 Balloon angioplasty may be indicated for infants who are a high surgical risk.
 Surgical intervention: usually performed as soon as the diagnosis is made.
o Subclavian flap repair (Waldhausen procedure).
o End-to-end anastomosis.
o Dacron patch repair.
Coarctation in the Child or Adolescent
 Surgical intervention.
o End-to-end anastomosis.
o Dacron patch.
 Medical management for hypertension (beta-adrenergic blockers).
Recurrent Coarctation in the Neonate or Child
 Balloon angioplasty in the cardiac catheterization laboratory.

 Redo surgical intervention.
Complications
 Systemic hypertension.
 CHF.
 Cerebral hemorrhage.
 Left ventricular failure.
 Aortic aneurysm.
PULMONARY STENOSIS
The pulmonary valve opens during systole to let blood flow from the right ventricle into the main
PA. Obstruction to flow can occur at three levels: subvalvular, valvular, or supravalvular level.
The most common cause of RV outflow tract obstruction is pulmonary valve stenosis. PS
accounts for 8% to 12% of CHDs.
 Critical PS in the neonate: blood flows into the right atrium, across a patent foramen
ovale (PFO) into the left heart; pulmonary blood flow comes from a left-to-right shunt
through a PDA.
 Right ventricular pressure increases to pump blood across the obstructive pulmonary
valve.
 RVH develops in response to the increased pressure gradient across the pulmonary valve.
 Signs of right-sided heart failure include hepatic congestion, neck vein distention,
elevated central venous pressure (CVP).
Critical PS in the Neonate
 Hypoxia.
 Tachypnea.
 RV failure.
Mild to Moderate PS in the Child and Adolescent

 Asymptomatic.
 Decreased exercise tolerance, fatigue, dyspnea.
 Chest pain.
 Auscultation: systolic ejection murmur heard best at the left upper sternal border; ejection
click.
 ECG: varies; normal in mild cases and RVH in moderate to severe cases.
 Chest X-ray: varies; may show right ventricular enlargement; poststenotic dilatation of
PA.
 Two-dimensional echocardiography with Doppler study and color flow mapping to
visualize the sites of obstruction, observe the degree of RVH, and estimate the pressure
gradient across the valve.
 Cardiac catheterization is usually not needed for the initial diagnosis.
Management
Neonate with Critical PS
o Stabilize and improve oxygen saturations with PGE1 infusion.
o Intubation and ventilation as needed.
o Inotropic support as needed.
 Balloon pulmonary valvuloplasty.
 Blalock-Taussig shunt (Gore-Tex graft between the subclavian artery and the PA to
supply pulmonary blood flow).
Child and Adolescent with PS
o Close follow-up to monitor and record RV to PA gradient and assess RV function.
o Restrict strenuous exercise and participation in competitive sports.
o Refer for intervention when RV pressure is greater than two-thirds of the systemic
pressures.
 Balloon pulmonary valvuloplasty in the cardiac catheterization laboratory.
 Surgical intervention:
o Valvotomy or valvectomy for dysplastic pulmonary valve.
o Patch repair of the right ventricular outflow tract.
o Placement of an RV-to-PA conduit.
Complications
 Cyanosis (critical PS in the neonate).

 Arrhythmia; sudden death.
 Right ventricular failure.
PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is a normal fetal connection between the left PA and the descending aorta.
During fetal life, blood flow is shunted away from the lungs through the ductus arteriosus and
directly into the systemic circulation. PDAs are common in premature neonates who weigh less
than 1,500 g. They account for 5% to 10% of CHDs, excluding premature neonates.
 During fetal life, the ductus arteriosus allows blood to bypass the pulmonary circulation
(fetus receives oxygen from the placenta) and flow directly into the systemic circulation.
 After birth, the ductus arteriosus is no longer needed. Functional closure usually occurs
within 48 hours after birth. Anatomic closure is completed by age 2 to 3 weeks.
 When the ductus arteriosus fails to close, blood from the aorta (high pressure) flows into
the low-pressure PA, resulting in pulmonary overcirculation.
 Increased pulmonary blood flow leads to a volume-loaded LV.
Clinical Presentation
Small to Moderate-Sized PDA
Usually asymptomatic.
Large PDA
 CHF, tachypnea, frequent respiratory tract infections.

 Poor weight gain, failure to thrive.
 Feeding difficulties.
 Decreased exercise tolerance.
 Auscultation: continuous murmur heard best at left upper sternal border. Hyperactive
precordium with large PDAs.
 Wide pulse pressure; bounding pulses.
 Chest X-ray: varies; normal or cardiomegaly with increased pulmonary vascular
markings.
 ECG: varies; normal or LVH.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to
visualize the PDA with left-to-right blood flow.
 Cardiac catheterization is not needed for the initial diagnosis.
Management
 In the symptomatic premature neonate: indomethacin given I.V.

o Monitor growth and development.
o Reassess for spontaneous PDA closure.
o Increase caloric intake as needed for normal weight gain.
o Diuretics: furosemide (Lasix), spironolactone (Aldactone).
o Infective endocarditis prophylaxis for 6 months after surgery or coil occlusion.
 Cardiac catheterization:
o For small PDAs coil occlusion.
o For larger PDAs a closure device may be used.
 Surgical management through PDA ligation.
Complications
 CHF, pulmonary edema.

 Pulmonary hypertension/pulmonary vascular occlusive disease.
 Recurrent pneumonia.
ATRIAL SEPTAL DEFECT

ASD is an abnormal communication between the left and right atrias. ASDs account for 9% of
CHDs. There are three types:
 Ostium secundum ASD: the most common type of ASD; abnormal opening in the middle
of the atrial septum.
 Ostium primum ASD: abnormal opening at the bottom of the atrial septum; increased
association with cleft mitral valve and atrioventricular defects.
 Sinus venosus ASD: abnormal opening at the top of the atrial septum; increased
association with partial anomalous pulmonary venous return.
 Blood flows from the higher-pressure left atrium across the ASD into the lower-pressure
right atrium (left-to-right shunt).
 Increased blood return to the right heart leads to right ventricular volume overload and
right ventricular dilation.
 Increased pulmonary blood flow leads to elevated pulmonary artery pressures.
 Usually asymptomatic.
 Clinical symptoms vary depending on type of associated defects:
o CHF (usually not until the third or fourth decade of life).
o Frequent upper respiratory infections (URIs).
o Poor weight gain.
o Decreased exercise tolerance.
 Auscultation: soft systolic ejection murmur heard best at the left upper sternal border;
widely split, fixed second heart sound.
 Chest X-ray: varies; normal to right atrial and ventricular dilation, increased pulmonary
markings.
 ECG: varies; right axis deviation and mild RVH or right bundle-branch block.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to identify
the site of the ASD and associated lesions and document left-to-right flow across the
atrial septum.
 Cardiac catheterization usually not needed for initial diagnosis; performed if defect can
be closed using an atrial occlusion device (device can be used only in ostium secundum
defects).
Management
o Monitor and reassess (spontaneous closure rate is small but may occur up to age
2).
o Treatment with anticongestive therapy (digoxin and Lasix) may be necessary if
signs of CHF are present (usually not until third to fourth decade of life if ASD
unrepaired).
o Infective endocarditis prophylaxis for 6 months after surgery or atrial occlusion
devise is used.
 Cardiac catheterization for placement of an atrial occlusion device for ostium secundam
defects.
 Surgical intervention:
o Primary repair: suture closure of the ASD.
o Patch repair of the ASD.
Complications
 CHF (rare).
 Embolic stroke.
 Pulmonary hypertension.
 Atrial arrhythmias.
VENTRICULAR SEPTAL DEFECT

A VSD is an abnormal communication between the right and left ventricles. It is the most
common type of congenital heart defect, accounting for approximately 25% of all CHDs. VSDs
vary in the size (small and restrictive to large and nonrestrictive defect), number (single versus
multiple), and type (perimembranous or muscular).
 Blood flows from the high-pressure left ventricle across the VSD into the low-pressure
right ventricle and into the PA, resulting in pulmonary overcirculation.
 A left-to-right shunt because of a VSD results in increased right ventricular pressure and
increased PA pressure.
 The increased pulmonary venous return to the left side of the heart results in left atrial
dilation.
 Long-standing pulmonary overcirculation causes a change in the pulmonary arterial bed,
leading to increased pulmonary vascular resistance. High pulmonary vascular resistance
(PVR) can reverse the blood flow pattern that leads to a right-to-left shunt across the
VSD (Eisenmenger's syndrome), resulting in cyanosis. Once this develops, the child is no
longer a candidate for surgical repair.
 Small VSDsâ€”usually asymptomatic; high spontaneous closure rate during the first year
of life.
 Large VSDs.
o CHF: tachypnea, tachycardia, excessive sweating associated with feeding,
hepatomegaly.
o Frequent URIs.
o Poor weight gain, failure to thrive.
o Feeding difficulties.
o Decreased exercise tolerance.
 Auscultation: harsh systolic regurgitant murmur heard best at the lower left sternal border
(LLSB); systolic thrill felt at LLSB, narrowly split S2.
 Chest X-ray: varies; normal or cardiomegaly and increased pulmonary vascular
markings. Pulmonary vascular markings are directly proportionate to the amount of left-
to-right shunting.
 ECG: varies; normal to biventricular hypertrophy.
the size, number, and sites of the defects, estimate pulmonary artery pressure, and
identify associated lesions.
 Cardiac catheterization usually not needed for initial diagnosis; may be needed to
calculate the size of the shunt or to assess PVR. May be performed if defect can be closed
using a ventricular occlusion device (device can be used only in muscular defects).
Management
Small VSD
o Usually no anticongestive therapy is needed.
o Infective endocarditis prophylaxis for 6 months after surgical implantation of a
ventricular occlusion device.
 Cardiac catheterization for placement of a ventricular occlusion device for muscular
defects (for Qp:Qs > 2:1).
 Surgical intervention is usually not necessary.
Moderate to Large VSD
 Medical Management:
o CHF management: digoxin and diuretics (furosemide, spironolactone) and
afterload reduction.
o Avoid oxygen; oxygen is a potent pulmonary vasodilator and will increase blood
flow into the PA.
o Increase caloric intake: fortify formula or breast milk to make 24 to 30 cal/oz
formula; supplemental nasogastric feeds as needed.
o Infective endocarditis prophylaxis for 6 months after surgery/ventricular device
occluder.
 Cardiac catheterization for placement of a ventricular occlusion device for muscular
defects (for Qp:Qs > 2:1).
 Refer for surgical intervention.
o Usually repaired before age 1.
o One-stage approach: preferred surgical plan; patch closure of VSD.
o Two-stage approach: first surgery is to band the PA to restrict pulmonary blood
flow; second surgery is to patch close the VSD and remove the PA band.
Long-Term Follow-Up
 Monitor ventricular function.

 Monitor for subaortic membrane and double-chamber RV.
Complications
 CHF.
 Frequent URIs.
 Failure to thrive; poor weight gain.
 Eisenmenger's syndrome.
 Pulmonary hypertension.
 Aortic insufficiency.
TETRALOGY OF FALLOT
TOF is the most common complex congenital heart defect; it accounts for 6% to 10% of all
CHDs. The four abnormalities of TOF include the following:
 A large, nonrestrictive VSD.
 Aortic override.
 Pulmonary stenosis (right ventricular outflow tract obstruction).
 Right ventricular hypertrophy.
 Degree of cyanosis depends on the size of the VSD and the degree of right ventricular
outflow tract obstruction (RVOTO).
 Obstruction of blood flow from the right ventricle to the PA results in deoxygenated
blood being shunted across the VSD and into the aorta (right-to-left shunt causes
cyanosis).
 RVOTO can occur at any or all of the following three levels: pulmonary valve stenosis,
infundibular stenosis, or supravalvular stenosis.
 The right ventricle becomes hypertrophied as a result of the increased gradient across the
RVOT.
 Minimal RVOTO results in a pink TOF variant, with the physiology behaving more like
a large, nonrestrictive VSD.
 Clinical manifestations are variable and depend on the size of the VSD and the degree of
RVOTO.
 Cyanosis.
o Neonate may have normal oxygen saturations; as the infant grows, the RVOTO
increases and the oxygen saturation falls.
o Neonate with unacceptably low oxygen saturation needs PGE1 infusion to
maintain ductal patency and adequate oxygen saturation.
o Cyanosis may initially be observed only with crying and with exertion.
 Polycythemia.
 Decreased exercise tolerance.
 A common clinical manifestation years ago was squatting, a posture characteristically
assumed by older children to increase systemic vascular resistance and to encourage
increased pulmonary blood flow. Squatting is rarely seen currently because TOF is now
surgically repaired during the first year of life.
 Hypercyanotic spells (formerly known as Tet spells): a life-threatening hypoxic event
with a dramatic decrease in oxygen saturations; mechanism is usually infundibular
spasm, which further obstructs pulmonary blood flow and increases right-to-left flow
across the VSD.
o Typical hypoxic spells occur in the morning soon after awakening; during or after
a crying episode; during or after a feeding; during painful procedures such as
blood draws.
o Typical scenario includes tachypnea, irritability, and increasing cyanosis,
followed by flaccidity and loss of consciousness.
o Home treatment for the caregiver: soothe the infant and place him or her in a
knee-chest position; notify the health care provider immediately.
o Hospital treatment includes knee-chest position, sedation (morphine), oxygen,
beta-adrenergic blockers (propranolol, esmolol) to relax the infundibulum, and
administration of medications to increase systemic vascular resistance
(phenylephrine).
o Hypercyanotic spells usually prompt the cardiologist to refer for surgical
intervention.
 Auscultation: harsh systolic ejection murmur heard best at the upper left sternal border
(RVOT murmur); single second heart sound; during a hypercyanotic spell the murmur
disappears.
 Chest X-ray: varies; normal or decreased pulmonary vascular markings. The heart may
appear â€œboot shapedâ€ because of a concave main PA with an upturned apex
resulting from RVH.
 ECG: varies; normal or RVH.
the structural abnormalities, estimate the degree of RVOTO and assess the coronary
artery pattern.
 Cardiac catheterization is usually not needed for the initial diagnosis. May be performed
before surgical intervention to identify the location and number of VSDs, the PVR, the
degree of RVOTO, and the presence of any coronary abnormalities.
Management
 Medical Management:
o Monitor oxygen saturation level.
o Monitor growth and development.
o Monitor for hypercyanotic spells (many spells go unnoticed by parents).
o Restrict strenuous activity and participation in competitive sports.
 Balloon pulmonary angioplasty (rarely).
 Surgical intervention: Palliative versus definitive repair.
o Many centers prefer definitive, one-stage repair.
o Potential obstacles for one-stage repair: abnormal coronary artery distribution
(left anterior descending arises from right coronary artery and crosses RVOT);
multiple VSDs; hypoplastic branch pulmonary arteries; small infant weighing less
than 5.5 lb (2.5 kg).
o Palliative surgery: modified Blalock-Taussig shunt (BT shunt); tube Gore-Tex
graft between the left subclavian artery and the PA: increased pulmonary blood
flow results in higher oxygen saturations.
o Definitive surgery: patch closure of VSD, relief of right ventricular outflow tract
obstruction; with or without transannular patch across the pulmonary valve.
Long-Term Follow-Up
 Assess RV outflow tract, monitor degree of pulmonary insufficiency.

 Monitor RV function and exercise tolerance.
 Monitor for arrhythmias.
Complications
 Hypoxia.
 Hypercyanotic spells.
 Polycythemia.
 CHF: rare; associated with pink TOF.
 Right ventricular dysfunction.
 Ventricular arrhythmias.
TRANSPOSITION OF THE GREAT ARTERIES

Transposition of the great arteries (TGA) occurs when the PA arises off the left ventricle and the
aorta arises off the right ventricle. It accounts for 5% to 10% of CHDs. Associated lesions
include VSD, ASD, PDA, PS, and CoA.
 This defect results in two parallel circulations:

o The right atrium receives deoxygenated blood from the inferior vena cava (IVC)
and superior vena cava (SVC); blood flow continues through the tricuspid valve
into the right ventricle and is pumped back to the aorta.
o The left atrium receives richly oxygenated blood from the pulmonary veins; blood
flow continues through the mitral valve into the left ventricle and is pumped back
into the PA.
 To sustain life, there must be an accompanying defect that allows mixing of
deoxygenated blood and oxygenated blood between the two circuits, such as PDA, ASD,
PFO, or VSD.
 Neonates born with TGA with an intact ventricular system are usually more cyanotic and
sicker than neonates born with TGA with a VSD.
Symptoms evident soon after birth; clinical scenario is influenced by the extent of
intercirculatory mixing.
 Cyanosis.
 Tachypnea.
 CHF.
 Auscultation: varies; no murmur, or a murmur related to an associated defect, single S2.

 Chest X-ray: varies; neonate chest X-ray usually normal; cardiomegaly with a narrow
mediastinum (egg-shaped cardiac silhouette) and increased pulmonary markings; or
decreased pulmonary markings with pulmonary stenosis.
 ECG: RVH or biventricular hypertrophy.
the structural abnormalities: transposed vessels, coronary artery pattern, and degree of
mixing across the atrial septum plus associated lesions.
Management
Medical Management
 Stabilize with PGE1 infusion.

 Treat pulmonary overcirculation with digoxin and diuretics as needed.
 Intubate and ventilate as needed.
Cardiac catheterization
 Balloon atrial septostomy (Rashkind) is indicated for severe hypoxia to create or improve
atrial level mixing.
Surgical Management
 Arterial switch operation (Jatene)â€”procedure of choice:

o Ideally performed during the first week of life.
o The aorta and PA are switched back to their anatomically correct ventricle above
the level of the valve.
o Coronary arteries are transferred to the new aorta.
o Associated lesions are also repaired at this time.
 Rastelli operationâ€”performed for TGA, VSD, and PS.
o Repaired during the first year of life.
o VSD patch repaired to include LV to aortic outflow continuity with pulmonary
blood flow provided via an RV to PA homograft.
 Atrial switch operation: Mustard or Senning procedure:
o Rerouting of atrial blood flow: RA â†’ mitral valve â†’ LV â†’ PA and LA â†’
tricuspid valve â†’ RV â†’ Ao
o Restores oxygenated blood into the systemic system and deoxygenated blood
guided to the pulmonary system.
o Disadvantages:
 RV is left as the systemic ventricleâ€”will develop RV dysfunction.
 Increased incidence of atrial dysrhythmias and baffle obstruction.
Complications
 Severe hypoxia.
 Multiorgan ischemia.
 Arrhythmias.
 RV dysfunction.
 Coronary artery obstruction leading to myocardial ischemia or death.
TRICUSPID ATRESIA
Tricuspid atresia involves the absence of the tricuspid valve and hypoplasia of the right ventricle.
Associated defects such as an ASD, VSD, or PDA are necessary for survival. Tricuspid atresia
accounts for 1% to 3% of CHDs.
 With TA, systemic venous return enters the right atrium and cannot continue into the RV;
blood flows across an atrial septal opening into the left atrium.
 Pulmonary blood flow occurs through a PDA or VSD.
 Cyanosis.
 Tachypnea.
 Auscultation: murmurs vary depending on the associated lesions; single second heart
sound.
 Chest X-ray: pulmonary vascular markings related to the amount of pulmonary blood
flow (usually decreased); normal to slightly increased cardiac silhouette.
 ECG: superior axis; right and left atrial hypertrophy; LVH.
 Two-dimensional echocardiogram identifies the atretic tricuspid valve and hypoplastic
RV; Doppler study and color flow mapping documents the right-to-left atrial shunt and
the size of the PDA or VSD.
 Cardiac catheterization may be necessary to delineate anatomy.
Management
Medical Management
 Stabilize with PGE1 infusion.

Surgical Management
 First surgeryâ€”neonate:
o BT shuntâ€”indicated if pulmonary blood flow is insufficient.
o Pulmonary artery bandâ€”indicated if pulmonary blood flow is excessive.
o No treatment is required if pulmonary blood flow is balanced.
 Second surgery: ages 6 to 9 months:
o Bidirectional Glenn shunt: end-to-side anastomosis of the SVC to the right PA.
 Third surgery: ages 18 months to 3 years:
o Fontan completion: IVC to PA connection (extracardiac conduit or intracardiac
baffle).
Complications
 CHF.
 Persistent pleural effusion (especially after Stage II and Stage III repairs).
 Thrombus formation in the systemic venous system.
 Rarely, heart block.
HYPOPLASTIC LEFT HEART SYNDROME

HLHS is a constellation of left heart abnormalities that include the following:
 Critical mitral stenosis or atresia.

 Hypoplastic LV.
 Critical aortic stenosis or atresia.
 Hypoplastic ascending aorta with severe coarctation of the aorta.
Hypoplastic left heart syndrome accounts for 1% of all CHDs. It is the most common
cause of death from cardiac defects in the first month of life.
 The left side of the heart is underdeveloped and essentially nonfunctional.

 The right ventricle supports the pulmonary and systemic circulations.
 An atrial septal defect allows blood to flow from the left atrium into the right heart.
 Blood flows right to left across the patent ductus arteriosus and into the descending aorta
to deliver oxygen and nutrients to the body.
 Neonate may appear completely well initially, but becomes critically ill when the PDA
closes.
 Once the PDA begins to close:
o Tachypnea due to CHF.
o Decreased urine output.
o Poor feeding and feeding intolerance.
o Lethargic; change in level of alertness.
o Pallor; gray.
o Weak peripheral pulses.
o Cyanosis.
 Auscultation: single S2; usually no heart murmur is present, but occasionally a soft
systolic ejection murmur may be heard.
 Chest X-ray: cardiac silhouette varies (normal to increased size); increased pulmonary
markings and pulmonary edema.
 ECG: RV hypertrophy; decrease electrical forces in V5 and V6.
the structural abnormalities and the altered blood flow patterns.
 A cardiac catheterization is usually not needed for initial diagnosis. It may be performed
if a balloon atrial septostomy is needed to improve oxygenation.
Management
Medical Management
 Resuscitation and stabilization with PGE1 infusion.

 Inotropic support as needed (dopamine, dobutamine).
 Assess hepatic, renal, and neurologic function.
 Refer for surgical intervention.
Cardiac catheterization
 May need balloon atrial septostomy to allow unrestrictive LA to RA blood flow.
Surgical Management
 Palliative, staged repair:

o Stage I Norwood (neonate): reconstruction of the hypoplastic aorta using the PA
and an aortic or pulmonary allograft, an atrial septectomy, repair of the
coarctation and placement of a BT shunt.
o Stage II: bidirectional Glenn shunt (ages 6 to 9 months): transect the SVC off the
right atrium and directly suture end to side to right PA; ligate BT shunt.
o Stage III: Fontan (ages 18 months to 3 years): IVC to PA connection (extracardiac
conduit or intracardiac baffle).
 Cardiac transplantation.
Complications
 Cyanosis.
 Persistent pleural effusion (especially after Stage II and Stage III repairs).
 Thrombus formation in the systemic venous system.
 Cardiovascular collapse.
 Multisystem failure.
 Death.
NURSING CARE OF THE CHILD WITH CONGENITAL HEART DISEASE

Nursing Assessment
 Obtain a thorough nursing history.

 Discuss the care plan with the health care team (cardiologist, cardiac surgeon, nursing
case manager, social worker, nutritionist). Discuss the care plan with the patient, parents,
and other caregivers.
 Measure and record height and weight. Plot on a growth chart.
 Record vital signs and oxygen saturations.
o Measure vital signs at a time when the infant/child is quiet.
o Choose appropriate-size blood pressure (BP) cuff.
o Check four extremity BP Ã— 1.
 Assess and record:
o Skin color: pink, cyanotic, mottled.
o Mucous membranes: moist, dry, cyanotic.
o Extremities: check peripheral pulses for quality and symmetry; dependent edema;
capillary refill; color and temperature.
 Assess for clubbing (cyanotic heart disease).
 Assess chest wall for deformities; prominent precordial activity.
 Assess respiratory pattern.
o Before disturbing the child, stand back and count the respiratory rate.
o Loosen or remove clothing to directly observe chest movement.
o Assess for signs of respiratory distress: increased respiratory rate, grunting,
retractions, nasal flaring.
o Auscultate for crackles, wheezing, congestion, stridor.
 Assess heart sounds.
o Determine rate (bradycardia, tachycardia, or normal for age) and rhythm (regular
or irregular).
o Identify murmur (type, location, and grade).
 Assess fluid status.
o Daily weights.
o Strict intake and output (number of wet diapers; urine output).
 Assess and record the child's level of activity.
o Observe the infant while feeding. Does the infant need frequent breaks or does he
or she fall asleep during feeding? Assess for sweating, color change, or
respiratory distress while feeding.
o Observe the child at play. Is play interrupted to rest? Ask the parent if the child
keeps up with peers while at play.
o Assess and record findings relevant to the child's developmental level: age-
appropriate behavior, cognitive skills, gross and fine motor skills.
Nursing Diagnoses
 Impaired Gas Exchange related to altered pulmonary blood flow or pulmonary

congestion
 Decreased Cardiac Output related to decreased myocardial function
 Activity Intolerance related to hypoxia or decreased myocardial function
 Imbalanced Nutrition: Less Than Body Requirements related to excessive energy
demands required by increased cardiac workload
 Risk for Infection related to chronic illness
 Fear and Anxiety related to life-threatening illness
Nursing Interventions
Relieving Respiratory Distress
 Position the child in a reclining, semi-upright position.

 Suction oral and nasal secretions as needed.
 Identify target oxygen saturations and administer oxygen as prescribed.
 Administer prescribed medications and document response to medications (improved, no
change, or worsening respiratory status).
o Diuretics.
o Bronchodilators.
 May need to change oral feedings to nasogastric feedings because of increased risk of
aspiration with respiratory distress.
Improving Cardiac Output
 Organize nursing care and medication schedule to provide periods of uninterrupted rest.
 Provide play or educational activities that can be done in bed with minimal exertion.
 Maintain normothermia.
 Administer medications as prescribed.
o Diuretics (furosemide, spironolactone):
 Give the medication at the same time each day. For older children, do not
give a dose right before bedtime.
 Monitor the effectiveness of the dose: measure and record urine output.
o Digoxin:
 Check heart rate for 1 minute. Withhold the dose and notify the physician
for bradycardia (heart rate less than 90 beats/minute [bpm]).
 Lead II rhythm strip may be ordered for PR interval monitoring.
Prolonged PR interval indicates first-degree heart block (dose of digoxin
may be withheld).
 Give medication at the same time each day. For infants and children,
digoxin is usually divided and given twice per day.
 Monitor serum electrolytes. Increased incidence of digoxin toxicity
associated with hypokalemia.
o Afterload-reducing medications (captopril, enalapril):
 When initiating medication for the first time: check BP immediately
before and 1 hour after dose.
 Monitor for signs of hypotension: syncope, light-headedness, faint pulses.
 Withhold medication and notify the physician according to ordered
parameters.
Improving Oxygenation and Activity Tolerance
 Place pulse oximeter probe (continuous monitoring or measure with vital signs) on
finger, earlobe, or toe.
 Administer oxygen as needed.
 Titrate amount of oxygen to reach target oxygen saturations.
 Assess response to oxygen therapy: increase in baseline oxygen saturations, improved
work of breathing, and change in patient comfort.
 Explain to the child how oxygen will help. If possible, give the child the choice for face
mask oxygen or nasal cannula oxygen.
Providing Adequate Nutrition
 For the infant:

o Small, frequent feedings.
o Fortified formula or breast milk (up to 30 cal/oz).
o Limit oral feeding time to 15 to 20 minutes.
o Supplement oral feeds with nasogastric feedings as needed to provide weight gain
(ie, continuous nasogastric feedings at night with ad-lib by-mouth feeds during
the day).
 For the child:
o Small, frequent meals.
o High-calorie, nutritional supplements.
o Determine child's likes and dislikes and plan meals accordingly.
o Allow the parents to bring the child's favorite foods to the hospital.
 Report feeding intolerance: nausea, vomiting, diarrhea.
 Document daily weight (same time of day, same scale, same clothing).
 Record accurate inputs and outputs; assess for fluid retention.
 Fluid restriction not usually needed for children; manage excess fluid with diuretics.
Preventing Infection
 Maintain routine childhood immunization schedule. With the exception of RSV (Synagis)
and influenza, immunizations should not be given for 6 weeks after cardiovascular
surgery.
 Administer yearly influenza vaccine.
 Administer RSV immunization for children younger than age 2 with complex CHD and
those at risk for CHF or pulmonary hypertension.
 Prevent exposure to communicable diseases.
 Good hand washing.
 Report fevers.
 Report signs of URI: runny nose, cough, increase in nasal secretions.
 Report signs of GI illness: diarrhea, abdominal pain, irritability.
Reducing Fear and Anxiety
 Educate the patient and family.

 Provide the family with contact phone numbers: how to schedule a follow-up visit; how
to reach a cardiologist during the work week, evenings, weekends, and holidays.
Family Education and Health Maintenance
 Instruct the family in necessary measures to maintain the child's health:

o Complete immunization.
o Adequate diet and rest.
o Prevention and control of infections.
o Regular medical and dental checkups. The child should be protected against
infective endocarditis when undergoing certain dental procedures.
o Regular cardiac checkups.
 Teach the family about the defect and its treatment.
o Provide patients and families with written and verbal information regarding the
CHD. Offer appropriate Internet resources for information about CHD and
medical and surgical treatment options.
o Signs and symptoms of CHF (see page 1477).
o Signs of hypercyanotic spells associated with cyanotic defects and need to place
child in knee-chest position.
o Need to prevent dehydration, which increases risk of thrombotic complications.
o Emergency precautions related to hypercyanotic spells, pulmonary edema, cardiac
arrest (if appropriate).
o Special home care equipment, monitors, oxygen.
 Encourage the parents and other people (teachers, peers) to treat the child in as normal a
manner as possible.
o Avoid overprotection and overindulgence.
o Avoid rejection.
o Promote growth and development with modifications. Facilitate performance of
the usual developmental tasks within the limits of the child's physiologic state.
o Prevent adults from projecting their fears and anxieties onto the child.
o Help family deal with its anger, guilt, and concerns related to the disabled child.
 Initiate a community health nursing referral if indicated.
 Stress the need for follow-up care.
 Encourage attendance in support groups for patients and families.
Evaluation: Expected Outcomes
 Improved oxygenation evidenced by easy, comfortable respirations

 Improved cardiac output demonstrated by stable vital signs, adequate peripheral
perfusion, and adequate urine output
 Increased activity level
 Maximal nutritional status demonstrated by weight gain and increase in growth curve
percentile
 No signs or symptoms of infection
 Parents discuss diagnosis and treatment together and with child
CONGESTIVE HEART FAILURE

CHF occurs when the cardiac output cannot meet the metabolic demands of the body.
 May result from the following:

o CHDs with a volume or pressure overload (moderate to large left-to-right shunt
[PDA, VSD] or AV valve insufficiency).
o Acquired heart disease: myocarditis, cardiomyopathy, acute rheumatic fever.
o Chronic pulmonary disease (cor pulmonale, bronchopulmonary dysplasia).
o Arrhythmias (prolonged SVT, complete heart block).
o Anemia.
o Iatrogenic fluid overload.
 In an attempt to meet the metabolic needs of the body, the heart rate increases to increase
cardiac output.
o Cardiac output = heart rate Ã— stroke volume.
o Stroke volume is the amount of blood (mL) ejected from the heart with each
heartbeat; it depends on preload and vascular resistance.
 Preload (CVP) increases as the failing heart contracts poorly.
 With decreased cardiac output, the systemic vascular resistance increases to maintain BP.
This increase in afterload limits cardiac output.
 With decreased blood flow to the kidneys, the glomerular filtration rate decreases as
tubular reabsorption increases sodium and water retention, resulting in a decreased urine
output.
 Long term, these compensatory mechanisms are detrimental to the failing myocardium.
The chronic increase in preload and afterload contributes to chamber dilation and
myocardial hypertrophy, leading to progressive CHF.
 Impaired myocardial function.

o Tachycardia, S3 gallop.
o Poor peripheral perfusion: weak peripheral pulses, cool extremities, delayed
capillary refill.
o Pallor.
o Exercise or activity intolerance.
 Pulmonary congestion.
o Tachypnea.
o Cyanosis.
o Retractions, nasal flaring, grunting.
o Cough.
 Systemic venous congestion.
o Hepatomegaly.
o Peripheral edema: scrotal and orbital.
o Water weight gain.
o Decreased urine output.
 Characteristic physical examination findings.

 Chest X-ray shows cardiomegaly and pulmonary congestion.
Management
 Diuretics to reduce intravascular volume (furosemide, spironolactone).

 Digoxin to increase myocardial contractility.
 Afterload reduction to decrease the workload of the ailing myocardium (angiotensin-
converting enzyme inhibitors: captopril, enalapril, lisinopril).
 Beta-adrenergic blockers to counteract the increased sympathetic activity and reduce
systemic vascular resistance (metoprolol, carvedilol).
Complications
 Pulmonary edema.
 Failure to thrive.
 URIs.
 Arrhythmia.
 Death.
Nursing Assessment
 Assess response to medical treatment plan.

 Document vital signs and oxygen saturations.
 Observe infant or child during feeding or activity. Assess for diaphoresis, need for
frequent rest periods, and inability to keep up with peers.
 Follow growth curve.
Nursing Diagnoses
 Decreased Cardiac Output related to myocardial dysfunction

 Excess Fluid Volume related to decreased cardiac contractility and decreased excretion
from the kidney
 Impaired Gas Exchange related to pulmonary venous congestion
 Activity Intolerance
 Risk for Infection related to pulmonary congestion
 Imbalanced Nutrition: Less Than Body Requirements related to increased metabolic
demands with decreased caloric intake
 Anxiety related to child's diagnosis and prognosis
Improving Myocardial Efficiency
 Administer digoxin as prescribed.

o Measure heart rate. Hold medication and notify health care provider for heart rate
less than 90 bpm.
o Check most recent potassium level. Hold medication and notify health care
provider for potassium less than 3.5.
o Run lead II ECG if ordered to monitor PR interval. If first-degree AV block
occurs, notify health care provider and hold medication as directed.
o Report signs of possible digoxin toxicity: vomiting, nausea, visual changes,
bradycardia.
o Double-check dose of digoxin with another nurse before administering the dose.
Make sure the digoxin order has two signatures.
 Administer afterload reduction medications as prescribed.
o Measure BP before and after giving the patient the medication. Hold the
medication and notify the health care provider for low BP (greater than a 15-mm
Hg drop from baseline).
o Observe for other signs of hypotension: dizziness, light-headedness, syncope.
Maintaining Fluid and Electrolyte Balance

 Administer diuretics as prescribed.
o Obtain daily weights.
o Keep strict intake and output record.
o Monitor serum electrolytes. Provide potassium supplements as needed.
 Sodium restriction: not usually needed in children; provide dietary assistance as needed.
 Fluid restriction: not usually needed in children.
Relieving Respiratory Distress
 Administer oxygen therapy as prescribed.

 Elevate head of bed; infants may be more comfortable in an upright infant seat.
Promoting Activity Tolerance
 Organize nursing care to provide periods of uninterrupted sleep/rest.

 Avoid unnecessary activities.
 Respond efficiently to a crying infant. Provide comfort and treat the source of distress:
wet or dirty diaper, hunger.
 Provide diversional activities that require limited expenditure of energy.
 Provide small, frequent feedings.
Decreasing Risk of Infection
 Ensure good hand washing by everyone.

 Avoid exposure to ill children or caretakers.
 Monitor signs of infection: fever, cough, runny nose, diarrhea, vomiting.
Providing Adequate Nutrition
 For the older child: provide nutritious foods that the child likes, along with supplemental
high-calorie snacks (milkshake, pudding).
 For the infant:
o High-calorie formula (24 to 30 cal/oz).
o Supplement oral intake with nasogastric feedings. Allow ad-lib oral intake
through the day with continuous nasogastric feedings at night.
Reducing Anxiety and Fear
 Communicate the care plan to the child and family.

 Educate the family about CHF and provide home care nursing referral to reinforce
teaching after discharge.
 Encourage questions; answer questions as able or refer to another member of the health
care team.

 Teach the signs and symptoms of CHF.
 Teach medications: brand name and generic name, expected effects, adverse effects,
dose.
 Demonstrate medication administration.
 With the family, design a medication administration time schedule.
 Provide guidelines for when to seek medical help.
 Teach infant and child cardiopulmonary resuscitation (CPR) as needed.
 Reinforce dietary guidelines; provide a recipe to the parent on how to make high-calorie
formula.
 Reinforce ways to prevent infection.
 Make sure that follow-up visit with health care providers is scheduled.
 Educate the patient and family on infective endocarditis guidelines and provide them with
written materials. Standard general prophylaxis for children at risk: amoxicillin 50 mg/kg
(maximum dose = 2 g) given orally 1 hour before the procedure.
 Heart rate within normal range for age; adequate urine output
 No unexpected weight gain
 Clear lungs; normal respiratory rate and effort
 Participates in quiet diversional activities
 No signs or symptoms of infection
 Adequate intake of small, frequent feedings
 Parents express understanding of disease process and treatment
ACQUIRED HEART DISEASE

Note: Kawasaki disease is discussed in chapter 53.
ACUTE RHEUMATIC FEVER
Acute rheumatic fever (ARF) is an acute autoimmune disease that occurs as a sequelae of group
A beta-hemolytic streptococcal infection. It is characterized by inflammatory lesions of
connective tissue and endothelial tissue, primarily affecting the joints and heart.
 Most first attacks of ARF occur 1 to 5 weeks (average 3 weeks) after a streptococcal
infection of the throat or of the upper respiratory tract.
 Peak incidence occurs in children ages 6 to 15. Incidence after a mild streptococcal
pharyngeal infection is 0.3% and after a severe streptococcal infection is 1% to 3%.
 Family history of rheumatic fever is usually positive.
 Streptococcal infection abates with or without treatment; however, autoantibodies attack
the myocardium, pericardium, and cardiac valves.
o Aschoff's bodies (fibrin deposits) develop on the valves, possibly leading to
permanent valve dysfunction, especially of the mitral and aortic valves.
o Severe myocarditis may cause dilation of the heart and CHF.
 Inflammation of the large joints causes a painful arthritis that may last 6 to 8 weeks.
 Involvement of the nervous system causes chorea (sudden involuntary movements).
Documented or undocumented group A beta-hemolytic streptococcal infection is usually
followed in several weeks by fever, malaise, and anorexia. Major symptoms of ARF may appear
several weeks to several months after initial infection.
Major Manifestations
 Carditisâ€”manifested by systolic or diastolic murmur, prolonged PR and QT intervals on

ECG, and possibly by signs of CHF (see page 1477).
 Polyarthritisâ€”pain and limited movement of two or more joints; joints are swollen, red,
warm, and tender.
 Choreaâ€”purposeless, involuntary, rapid movements commonly associated with muscle
weakness, involuntary facial grimaces, speech disturbance, and emotional lability.
 Erythema marginatumâ€”nonpruritic pink, macular rash mostly of the trunk with pale
central areas; migratory.
 Subcutaneous nodulesâ€”firm, painless nodules over the scalp, extensor surface of joints,
such as wrists, elbows, knees, and vertebral column.
Minor Manifestations
 History of previous rheumatic fever or evidence of preexisting rheumatic heart disease.

 Arthralgiaâ€”pain in one or more joints without evidence of inflammation, tenderness, or
limited movement.
 Feverâ€”temperature greater than 100.4Â° F (38Â° C).
 Laboratory abnormalitiesâ€”elevated erythrocyte sedimentation rate (ESR), positive C-
reactive protein, elevated white blood cell count.
 ECG changesâ€”prolonged PR interval.
 Diagnosed clinically through use of the Jones criteria from the American Heart
Associationâ€”presence of two major manifestations or one major and two minor
manifestations (as listed above), with supporting evidence of a recent streptococcal
infection.
 ECG done to evaluate PR interval and other changes.
 Laboratory tests listed above. In addition, group A streptococcal culture and/or
antistreptolysin-O titer to detect streptococcal antibodies from recent infection.
 Chest X-ray for cardiomegaly, pulmonary congestion, or edema.
Management
 Course of antibiotic therapy to completely eradicate streptococcal infection (may be

given despite previous treatment).
o Usually benzathine penicillin is given I.M. in a single dose.
o Oral erythromycin may be used for children who are allergic to penicillin.
 Oral salicylates (aspirin) or nonsteroidal anti-inflammatory drugs (naproxen sodium)
usually used to control pain and inflammation of arthritis. Aspirin is continued for 4 to 6
weeks for carditis.
 Corticosteroids used in severe cases to try to control cardiac inflammation.
 Phenobarbital, diazepam, or other neurologic agent to control chorea.
 Bed rest during the acute phase (until ESR decreases, C-reactive protein becomes
negative, and pulse rate returns to normal) to rest the heart. Bed rest may need to be
maintained for 2 to 4 months in cases of severe carditis.
 Mitral valve replacement may be necessary in some cases.
 Secondary prevention of recurrent ARF:
o Risk of recurrence greatest within first 5 years, with multiple episodes of ARF,
and with rheumatic heart disease. Prophylactic antibiotic treatment may be
lifelong.
o For those at low risk for recurrence, antibiotic prophylaxis may be continued for 5
years or longer.
o Antibiotic regimens may include the following:
 Benzathine penicillin I.M. every 28 days.
 Penicillin V or erythromycin 250 mg twice per day.
 Sulfisoxazole (Pediazole) 0.5 to 1 g (dosage calculated according to
patient's weight) once per day.
Complications
 CHF.
 Pericarditis, pericardial effusion.
 Permanent damage to the aortic or mitral valve, possibly requiring valve replacement.
Nursing Assessment
 Assess for signs of cardiac involvement by auscultation of the heart for murmur and
cardiac monitoring for prolonged PR interval.
 Monitor pulse for 1 full minute to determine heart rate.
 Assess temperature for elevation.
 Observe for involuntary movements: stick out tongue or smile; garbled or hesitant speech
when asked to recite numbers or the ABCs; hyperextension of the wrists and fingers
when trying to extend arms.
 Assess child's ability to feed self, dress, and do other activities if chorea or arthritis
present.
 Assess pain level using scale appropriate for child's age.
 Assess parents' ability to cope with illness and care for child.
 Assess need for home schooling while patient is on bed rest.
Nursing Diagnoses
 Decreased Cardiac Output related to carditis

 Acute and Chronic Pain related to arthritis
 Risk for Injury related to chorea
Improving Cardiac Output
 Explain to the child and family the need for bed rest during the acute phase
(approximately 2 weeks) and as long as CHF is present. In milder cases, light indoor
activity is allowed.
 In severe cases, organize care so that the child will not have to exert self and will have
hours of uninterrupted rest.
 Maintain cardiac monitoring if indicated.
 Administer course of antibiotics as directed. Be alert to adverse effects, such as nausea,
vomiting, and GI distress.
 Administer medications for CHF as directed. Monitor BP, intake and output, and heart
rate.
Relieving Pain
 Administer anti-inflammatory medication, analgesics, and antipyretics as directed.

o Monitor for signs of aspirin toxicity, such as tinnitus, nausea and vomiting, and
headache.
o Monitor for signs of corticosteroid useâ€”GI distress, acne, weight gain,
emotional disturbancesâ€”or long-term effects, such as rounded face, ulcer
formation, and decreased resistance to infection.
o Administer all anti-inflammatory medications with food to reduce GI injury.
o Be aware that anti-inflammatories may not alter the course of myocardial injury.
 Teach family the importance of maintaining dosage schedule, continuing medication until
all signs and symptoms of the ARF have gone, and tapering the dose as directed by health
care provider.
 Assist child with positioning for comfort and protecting inflamed joints.
 Suggest diversional activities that do not require use of painful joints.
Protecting the Child with Chorea
 Use padded side rails if chorea is severe.

 Assist with feeding and other fine-motor activities as needed.
 Assist with ambulation if weak.
 Avoid the use of straws and sharp utensils if chorea involves the face.
 Make sure that child consumes nutritious diet with recommended vitamins, protein, and
calories.
 Be patient if speech is affected, and offer emotional support.
 Protect the child from stress.
 Administer phenobarbital or other medication for chorea as directed. Observe for
drowsiness.
 Teach the appropriate administration of all medications, including prophylactic antibiotic.

 Encourage all family and household members to be screened for streptococcus and
receive the appropriate treatment.
 Instruct on additional prophylaxis for endocarditis with dental procedures and surgery as
indicated.
 Encourage following activity restrictions, resuming activity gradually, and resting
whenever tired.
 Encourage keeping appointments for follow-up evaluation by cardiologist and other
health care providers.
 Advise the parents that child cannot return to school until health care provider assesses
that all disease activity is gone. Parents may need to discuss with teachers how the child
can catch up with schoolwork.
 Instruct on follow-up with usual health care provider for immunizations, well-child
evaluations, hearing and vision screening, and other health maintenance needs.
 Provide general health education about early identification and treatment seeking for any
possible streptococcal infection (fever, sore throat). Compliance with 10 to 14 days of
antibiotics can greatly reduce the risk of ARF and other poststreptococcal sequelae.
 Heart rate and PR interval within normal range for age; no signs of CHF
 Compliant with anti-inflammatory therapy; reports pain as 1 to 2 on scale of 1 to 10
 Feeds self, washes face and hands, and ambulates to bathroom without injury
CARDIOMYOPATHY
According to the type of myocardial changes, cardiomyopathy can be classified into three
categories: dilated, restrictive, and hypertrophic. The most common type in children is dilated
cardiomyopathy.
 Familial (family history, genetic predisposition) tendency.

 Idiopathic in most cases.
 May be related to:
o Nutritional deficiency (carnitine or selenium).
o Viral infection (myocarditis), human immunodeficiency virus.
o Collagen vascular disease (systemic lupus erythematosus).
o Cardiotoxic drugs (doxorubicin [Adriamycin]).
o Cocaine abuse.
o Postpartum.
o Sustained tachycardia (ectopic atrial tachycardia).
o Catecholamine surge; hyperthyroidism.
 Dilated cardiomyopathy involves dilatation of one or both ventricles associated with
normal septal and LV free wall thickness.
 Decreased systolic function (contractility) results in CHF.
 Increasing end-systolic dimension results in AV valve insufficiency, further worsening
CHF.
 Signs of CHFâ€”tachycardia, tachypnea, dyspnea, crackles, hepatosplenomegaly.

 Decreased exercise tolerance, fatigue, sweating.
 Poor weight gain, nausea, abdominal tenderness.
 Ventricular arrhythmia.
 Chest pain.
 Syncope.
 Auscultation: systolic regurgitant murmur (if mitral or tricuspid insufficiency is present),

S2 is normal or narrowly split, prominent S3 gallop.
 ECG: tachycardia, abnormal ST segments, arrhythmia, ectopic atrial tachycardia.
 Chest X-ray: cardiomegaly, pulmonary congestion.
 Two-dimensional echocardiogram: poor ventricular systolic function, dilated heart
chambers; AV valve insufficiency.
 Cardiac catheterization: not needed for initial diagnosis; endomyocardial biopsy (to rule
out myocarditis); assess PVR.
Management
General Measures
 Identify and treat the underlying cause.

 Maximize caloric intake: fortify formula; supplemental nasogastric feedings.
 Supplemental oxygen as needed.
 Activity restriction (usually self-imposed by the younger child and infant). Restrict
participation in strenuous and competitive sports.
Treatment of Systolic Dysfunction with Dilated Cardiomyopathy
 Diuretics: furosemide, spironolactone.

 Inotropics: digoxin.
 Afterload reduction: captopril, enalapril, lisinopril.
 Anticoagulation: warfarin, low-molecular weight heparin (enoxaparin).
 Antiarrhythmics.
 Placement of an automatic implantable cardioverter-defibrillator.
 Biventricular pacing.
 Cardiac transplant.
Treatment of Diastolic Dysfunction with Hypertrophic Cardiomyopathy

 Beta-adrenergic blockers.
 Calcium channel blockers.
 AV sequential pacing.
 Myomectomy or myotomy.
Treatment of Diastolic Dysfunction with Restrictive Cardiomyopathy
 Diuretics.
 Anticoagulation.
 Permanent pacemaker for advanced heart block.
Complications
 Severe CHF.
 Increased pulmonary vascular resistance.
 Intracardiac thrombus.
 Embolus.
 Malignant arrhythmias.
 Sudden death.
Nursing Assessment
Perform a thorough nursing assessment as for CHD (see page 1475).
Nursing Diagnoses
 Decreased Cardiac Output related to impaired systolic or diastolic ventricular function

 Imbalanced Nutrition: Less Than Body Requirements related to increased metabolic
demands and poor feeding from dyspnea, fatigue, and poor appetite
 Ineffective Family Coping related to chronic illness
Maximizing Cardiac Output
 Monitor vital signs; notify physician for hypotension, tachycardia, arrhythmia; increasing
tachypnea.
 Administer oxygen therapy as prescribed.
 Administer medications as prescribed.
o Maintain bleeding precautions for anticoagulated patients.
o Document response to diuretics; monitor intake and output.
 Monitor electrolytes.
 Restrict level of activity.
Providing Maximal Nutritional Support
 Encourage frequent, small meals. Provide foods the child likes.

 Provide high-calorie supplements (milkshakes, pudding).
 Administer supplemental tube feedings if nutritional needs are not being met.
 Administer parenteral hyperalimentation and intralipids as directed (rarely needed).
Promoting Effective Coping and Control within the Family
 Organize a family meeting with various members of the health care team to review the
child's medical condition and to explain the treatment plan.
 Allow the child and family to express their questions, fears, and concerns.
 Identify support systems and services for the child and family: extended family members,
clergy, support groups, community resources.
 Teach child and family medication administration: purpose of the drug, drug dosage, drug
schedule, and adverse effects.
 Help family design a realistic medication schedule.
o Identify usual wake-up time and bedtime. Schedule medications accordingly.
o Do not give a diuretic right before bedtime or nap time.
o If possible, avoid having to give medications at school.
 Teach bleeding precautions if the child is on anticoagulation agents (coumadin).
o Monitor prothrombin International Normalized Ratio regularly.
o Observe for signs of bleeding.
o Counsel regarding menses and pregnancy.
o Instruct on foods and medications that interfere with coumadin.
 Give guidelines for notifying the physician.
o Worsening shortness of breath.
o Irregular pulse; palpitations.
o Syncope, dizziness, or light-headedness.
o Increasing fatigue, exercise intolerance.
 Teach infant and child CPR to family members and other caregivers.
 Stable vital signs

 Maximal nutritional status as evidenced by weight gain and growth
 Compliance with the treatment plan and follow-up visits
CARDIAC PROCEDURES
CARDIAC CATHETERIZATION
Cardiac catheterization is an invasive procedure used to identify cardiac anatomy; measure
intracardiac pressures, shunts, and oxygen saturations; and calculate systemic and pulmonary
vascular resistance.
Procedure
 Catheter insertion sites include femoral vein or artery, umbilical vein or artery, brachial
vein, or internal jugular vein.
 Under fluoroscopy, catheters are guided through the heart, collecting pressure
measurements and oxygen saturations.
 Contrast dye is injected through the catheters to visualize blood flow patterns and
structural abnormalities.
 Cardiac catheterization is usually an outpatient procedure for children who undergo an
elective procedure. After interventional procedures, some children are observed in the
hospital for 24 hours.
Indications
 To confirm or establish the diagnosis.

 To measure cardiac output.
 To measure pressures and oxygen saturations.
 To calculate intracardiac shunting, and pulmonary and systemic vascular resistance.
 To visualize coronary arteries.
 To assess for myocarditis or rejection following heart transplantation.
 To intervene:
o Balloon atrial septostomy (Rashkind) for restrictive atrial septum.
o Balloon valvuloplasty (AS, PS) and angioplasty (recurrent CoA).
o Endomyocardial biopsy.
o To occlude vessels (coil embolization) or defects (ASD, VSD, or PDA closure
devices).
o To stent vessels open (branch PA stenosis, recurrent CoA).
Complications
 Arrhythmias (usually catheter induced).

 Infection.
 Bleeding at catheter insertion site; large hematoma.
 Allergic reaction to contrast material.
 Loss of pulse in the extremity used for cannulation.
 Perforation of heart or vessels.
 Stroke.
 Dislodgment of coils, closure devices, or stents.
 Death.
Nursing Diagnoses
 Fear related to surgical procedure

 Deficient Knowledge regarding surgical procedure and associated nursing care
 Risk for Injury related to complications of cardiac catheterization
Preoperative
Reducing Fear in Child and Parents
 Provide specific instructions in nonthreatening manner:

o Day and time of the procedure.
o Nothing-by-mouth (NPO) guidelines.
o Sedation versus general anesthesia.
o Site of the planned arterial and venous puncture.
o Routine postprocedure care.
 Provide appropriate teaching geared toward the child's age and level of cognitive
development. Use diagrams and models as appropriate.
 Provide parents an opportunity, without the child present, to discuss the procedure, risks,
benefits, and alternative choices.
 Give child opportunity to express fears and ask questions.
 Provide tour of the cardiac catheterization laboratory if appropriate.
Explaining and Providing Nursing Care
 Obtain baseline set of vital signs: heart rate, BP, respiratory rate, and oxygen saturation.
 Measure and record child's height and weight.
 Note time of last oral intake: solids and liquids.
 Identify known allergies.
 List current medications and note time last taken.
 Help child change into a hospital gown.
 Start peripheral I.V. as needed.
 Administer sedation as prescribed.
Postoperative
Observe For and Prevent Complications
 Monitor and record routine vital signs (q 15 min Ã— 4, q 30 min Ã— 2, then q 1 hr);
extremity pulse check with vital signs.
 Notify health care provider for:
o Heart rate, respiratory rate, or BP outside normal parameters for age.
o Bleeding or increasing hematoma at puncture site.
o Change in oxygen saturations.
o Fever.
o Cool, pulseless extremity.
 Observe puncture site for redness, pain, swelling, or induration.
 Maintain the child in a reclining position for 4 to 6 hours after the procedure.
 Offer fluids as soon as the child is ready.

 Provide discharge information:
o Care of incision or puncture site (keep dry for 48 hours).
o Activity restrictions (usually for 48 hours, but may be up to 2 weeks following
placement of closure devices).
o Observe for and report late complications: redness, swelling, drainage from
puncture site.
o Follow-up medical care.
o Reinforce infective endocarditis precautions.
 If cardiac catheterization was a preoperative procedure, use the recovery time to teach the
child and family about upcoming hospital stay.
 Child describes procedure in own words; parents and child discuss procedure and ask
appropriate questions
 Child cooperative with preoperative nursing care
 Insertion site intact without drainage, redness, or hematoma
CARDIAC SURGERY
The ultimate goal of treatment of cardiovascular disease in children is to restore normal heart
structure and function. Most types of CHDs can be palliated or definitively repaired.
Procedures
Closed-Heart Surgery
 Surgical approach: lateral thoracotomy or mediastinal incision.

 Indications:
o PDA ligation.
o PA banding.
o CoA repair.
o Vascular ring repair.
o BT shunt placement.
o Occasionally, Glenn and Fontan procedures.
Open-Heart Surgery
 Surgery is done through a mediastinal incision.

 With the use of cardiopulmonary bypass, the surgeon can stop the heart and operate
inside to repair the defects.
 Deep hypothermia with circulatory arrest allows the surgeon to safely stop
cardiopulmonary bypass and remove arterial or venous cannulas to better visualize and
repair the defects.
 Indications:
o ASD, VSD, AV canal defect.
o Aortic stenosis, pulmonary stenosis.
o TOF.
o TGA.
o Tricuspid atresia.
o TAPVR or partial APVR.
o Truncus arteriosus.
o HLHS.
o Complex single ventricle.
Potential Complications of Specific Surgeries
 PDA ligation: laryngeal nerve damage, phrenic nerve damage, diaphragm paralysis,
thoracic duct injury.
 CoA: rebound hypertension, mesenteric arteritis (abdominal pain), coarctation restenosis.
 Aorta-pulmonary shunt (BT shunt): shunt occlusion, PA distortion, pulmonary
overcirculation.
 ASD: atrial arrhythmias, sinoatrial node dysfunction.
 VSD: transient or permanent heart block, residual VSD, ventricular dysfunction.
 TOF: low cardiac output, residual RVOTO or VSD, ectopic junctional tachycardia or
arrhythmias, thoracic duct injury.
 TGA: arterial switch operationâ€”coronary artery injury, ventricular dysfunction,
suprapulmonary stenosis.
 TGA: atrial switch operationâ€”baffle obstruction, RV failure, atrial arrhythmias.
 Valvotomy (for valve stenosis): valve insufficiency.
 Bidirectional Glenn shunt: SVC syndrome, low cardiac output, hypoxia, pleural
effusions.
 Fontan completion: low cardiac output, pleural effusions, ventricular dysfunction,
thrombus formation.
Cardiac Transplant Surgery

Cardiac transplantation is a treatment option for children with progressive CHF or certain cardiac
diseases not amenable to conventional medical-surgical therapy. Children who cannot grow and
meet developmental milestones or who have unacceptable quality-of-life issues may benefit from
cardiac transplant surgery. However, approximately one in four children die
P.1484
while waiting for an organ donor. Those children who receive a donor heart must take lifelong
immunosuppression medications to prevent organ rejection.
Indications for Cardiac Transplantation
 End-stage cardiomyopathy.
 Untreatable complex CHD.
 Malignant arrhythmia.
 Retransplant for cardiac graft failure.
Immunosuppressive Medications
 Cyclosporine (Neoral).
 Azathioprine (Imuran).
 Tacrolimus (FK506, Prograf).
 Prednisone.
 Muromonab-CD3.
Complications
 Organ rejection: routine surveillance endomyocardial biopsies are performed to assess for
rejection.
o With mild to moderate rejection, children may initially be asymptomatic.
o With severe rejection, children are usually symptomatic with hemodynamic
instability.
 Infection.
 Adverse effects of immunosuppressive agents.
o Cyclosporineâ€”hypertension and renal toxicity.
o Imuranâ€”bone marrow suppression.
o Tacrolimusâ€”hypertension, headache, electrolyte imbalance.
o Prednisoneâ€”hypertension, Cushing syndrome, growth retardation.
 Accelerated diffuse coronary artery disease.
 Posttransplant lymphoproliferative disease.
Routine Follow-Up
 Routine well-child care visits to primary care provider.

o Transplant children should not receive any live virus immunizations (oral polio,
measles-mumps-rubella, varicella). Monitor for adverse effects of chronic steroids
and immunosuppressive medications.
 Routine cardiology clinic visits.
o Laboratory studies: chemistry, hematology, therapeutic drug levels.
o Vital signs.
o ECG.
o Echocardiography.
 Serial cardiac catheterizations and endomyocardial biopsies.
 Yearly coronary angiography or dobutamine stress echocardiography.
Nursing Assessment
Baseline Assessment on Day of Surgery
 Measure and record height and weight.

 Document vital signs: heart rate, respiratory rate, BP, and oxygen saturation.
 Assess for preoperative infection: fever, signs of URI (cough, runny nose, crackles),
vomiting, or diarrhea might necessitate delaying surgery.
 Document last oral intake.
 Void on call to the operating room.
Nursing Diagnoses
 Fear related to surgical procedure

 Risk for Injury related to complications of surgery
 Impaired Adjustment related to postsurgery care
Preoperative
Preparing the Child and Family and Reducing Fear
 Be honest and use nonthreatening language the child can understand.

 The following are frequently asked questions from parents. Address them with the
parents:
o What to tell the child.
o When to tell the child.
o What to bring to the hospital.
o Anticipated hospital course: how long in the operating room; how many days in
the intensive care unit (ICU); how many days on the general care unit; visiting
policy, rooming-in accommodations.
 Review preoperative instructions.
o NPO guidelines.
o Where to report on the day of the surgery.
o Time to arrive at the hospital; time of surgery.
o Preoperative medications (injection, liquid, inhalation).
o What the operating room looks like; what the people wear in the operating room
(hats, gowns, and masks).
 Explain the preoperative period.
o Change into a hospital gown.
o Parents stay with the child.
o Transportation to the operating room (walking, wheelchair, or stretcher).
 Explain the operative period.
o Operating room waiting room.
o Updates during the surgery will come from the surgical scrub nurses.
o Surgeon will meet the family after surgery to review the surgical findings and to
describe the operation.
 Explain the postoperative period. Use models and diagrams.
o Pediatric ICU routines and procedures.
o Monitoring lines and equipment.
o Ventilators, oxygen therapy.
o Protective restraints.
 Offer medical equipment to handle and play with (ECG leads, face mask, BP cuff), age-
appropriate books about surgery and hospital stays, and tour of ICU and surgical area as
available.
 Prehospital tour of the pediatric ICU and general pediatric care unit.
 Allow an opportunity for the child and family to ask questions, express their concern, or
to ask for more detail.
Postoperative
Observing For and Preventing Complications
 Assess respiratory status and maintain respiratory support.

o Maintain ventilatory support as needed.
o Maintain patent airway with routine endotracheal suctioning.
o Auscultate breath sounds frequently. Decreased breath sounds may indicate
pleural effusions, atelectasis, pneumothorax, hemothorax.
o Report results of routine chest X-ray.
o Perform frequent position changes: side-back-side.
o Monitor arterial blood gas levels and oxygen saturations.
o Assess chest tube drainage.
o Extubate when hemodynamically stable and when patient meets extubation
criteria.
o Administer oxygen therapy as needed after extubation.
 Assess cardiac status.
o Monitor vital signs and oxygen saturations.
o Maintain continuous ECG monitoring.
 Daily 12-lead ECG to assess rhythm.
 Temporary epicardial pacemaker wires available for pacing as needed.
o Continuous BP monitoring (arterial line).
o Monitor intracardiac pressures (CVP, LA, PA).
o Monitor peripheral perfusion, capillary refill, toe temperature.
o Titrate vasopressors (dopamine, dobutamine, milrinone) as prescribed.
 Assess fluid status.
o Record hourly intake (I.V. fluids, blood products, fluid boluses).
o Record hourly output (urine, chest tube drainage, nasogastric drainage).
o Perform daily weights.
o Administer diuretics as prescribed.
 Assess neurologic status.
o Monitor level of responsiveness, response to verbal commands, and response to
pain.
o Check pupil size and reactivity to light.
o Document movement of all extremities.
o Monitor all invasive lines for air bubbles and potential air embolism.
o Observe for signs of neurologic injury related to hypoperfusion or embolism.
 Monitor for potential specific complications related to particular surgery for CHD.
 Assess for postoperative pain.
o Monitor level of responsiveness, agitation.
o Implement pediatric pain scale rating to assist the child in identifying the severity
of pain.
o Administer pain medication as prescribed: continuous I.V. infusion and I.V.
boluses as necessary.
o Utilize patient-controlled analgesia continuous pump as appropriate.
 Assess serum electrolyte balance. Obtain blood tests and report results. Treat deficits with
supplements.
 Assess packed cell volume, platelet count, and coagulation studies.
o Low hematocrit (less than 30): consider directed donor transfusion or blood bank
transfusion.
o Low platelet count: continue to monitor; if bleeding persists, transfuse platelets.
o Prolonged coagulation studies: continue to monitor; if bleeding or oozing persists,
give protamine, fresh frozen plasma.
Enhancing Adjustment Postoperatively
 Provide continuity of care (primary nursing and consistent medical team).

 Explain all procedures and routines to the child and parents to minimize fear.
 Explain to the parents a child's typical reactions to stressful events.
o Regressionâ€”temporary loss of developmental milestones.
o Fear of any medical personnel (white coat anxiety).
o Sibling jealousy that one child is receiving a lot of attention.
o Withdrawalâ€”related to stimulation overload and lack of undisturbed sleep.
o Nightmares.
o Increased dependency; clinging behavior.
 Suggest parent and patient support groups, Child Life Therapy, community resources,
and counseling as needed.
 Provide the child and family with oral and written discharge instructions and
recommendations.
o Medications.
o Activity restrictions.
 No strenuous activity or contact sports for 6 to 8 weeks after surgery.
 Most children are ready to return to school at least part time about 2 weeks
after surgery.
 If child needs prolonged recovery time at home, may need to consider
home tutoring.
 No gym class until full recovery.
o Care of the incision.
o Dietary recommendations.
o Bathing or showering guidelines.
 Provide the child and family with a list of potential signs of complications and
instructions to notify the health care provider.
o Fever greater than or equal to 101.5Â° F (38.6Â° C).
o Any redness, swelling, or drainage from chest incision.
o Partial opening of the chest incision.
o Poor appetite, nausea, vomiting.
o Breathing difficulties, shortness of breath.
 Provide the family with the names and phone numbers of people to call for questions and
emergencies.
 Make follow-up appointments for the child to be seen by his or her primary care provider
2 to 3 days after discharge from the hospital and 10 to 14 days to be seen by their
pediatric cardiologist.
 Review American Heart Association's recommendations for infective endocarditis
prophylaxis: standard general prophylaxis for children at risk: amoxicillin 50 mg/kg
(maximum dose = 2 g) given orally 1 hour before the procedure.
Community and Home Care Considerations
 Arrange skilled home nursing visits as needed to:

o Review medications.
o Assess wound healing.
o Monitor vital signs and oxygen saturations.
o Assess oral intake; nutritional supplements.
o Resource for family.
 Make referral to community agencies as needed (infant and toddler program).
 Arrange for home medical equipment (oxygen, feeding pump, and supplies) as needed.
 Review safety precautions in the home:
o Childproof medication bottles.
o Poison control phone number for accidental medication overdose.
o Infant and child CPR techniques.
o Bleeding precautions for children on anticoagulation therapy. No aspirin or
ibuprofen products. Be sure to read the labels on over-the-counter cold and cough
syrups. Avoid activities with high risk of injury. All head injuries need to be
evaluated by a physician. Signs of bleeding:
 Blood in the urine.
 Black tarry stools.
 Prolonged nosebleeds.
 Bleeding gums.
 Bruising for no known trauma.
 Spitting or coughing up blood.
 Any unusual swelling or pain.
o MedicAlert bracelet or tags.
 Discuss developmental issues with the family.
o A child on diuretics may have difficulty with toilet training.
o Disciplining, establishing behavioral expectations, and setting limits in a child
with CHD should be similar to those for a child without CHD.
 Discuss the need for home schooling or tutoring during recovery time. Return to the
classroom as soon as the child is ready.
 Discuss infants with CHD in daycare situationsâ€”address each case individually.
Daycare programs usually have increased risk of URIs and other communicable diseases.
 Encourage routine dental visits to prevent dental caries (dental caries predispose to
bacteremia and endocarditis).
 Encourage heart-healthy eating and exercise routines.
 Encourage age-appropriate activities. A few children will need exercise restrictions.
Children with CHD should be allowed to participate in activities with rest periods as
needed. Children with pacemakers and children on anticoagulation therapy should refrain
from contact sports.
 Maintain standard childhood immunization schedule. Delay vaccines around the
perioperative time until fully recovered from surgery (no immunizations for 6 weeks
postoperatively).
 Encourage yearly influenza vaccine for children with unrepaired or complex CHD.
 Encourage RSV immunization for children younger than age 2 with complex CHD and
those at risk of CHF or pulmonary hypertension.
 Child describes procedure without fear; parents and child discuss procedure and ask
appropriate questions
 Vital signs stable, no signs of infection
 Parents offer support to child

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FIGURE 45-1 Congenital heart abnormalities

 CHD affects 8 to 12 of every 1,000 neonates.

COMMON CONGENITAL HEART MALFORMATIONS

(acyanotic lesions), and decreased pulmonary blood flow (cyanotic lesions).

BOX 45-1 ABBREVIATIONS USED FOR CONGENITAL HEART DISEASE

 Severe congestive heart failure (CHF).

Child and Adolescent

 Chest pain on exertion, decreased exercise tolerance.

Child and Adolescent

 CHF and pulmonary edema.

COARCTATION OF THE AORTA

 The neonate with critical CoA (ductal dependent lesion):

 Auscultationâ€”varies; nonspecific systolic ejection murmur.

Coarctation in the Child or Adolescent

Recurrent Coarctation in the Neonate or Child

 Balloon angioplasty in the cardiac catheterization laboratory.

Mild to Moderate PS in the Child and Adolescent

Child and Adolescent with PS

 Cyanosis (critical PS in the neonate).

PATENT DUCTUS ARTERIOSUS

 CHF, tachypnea, frequent respiratory tract infections.

 In the symptomatic premature neonate: indomethacin given I.V.

 CHF, pulmonary edema.

ATRIAL SEPTAL DEFECT

Pathophysiology and Etiology

VENTRICULAR SEPTAL DEFECT

Moderate to Large VSD

 Monitor ventricular function.

Pathophysiology and Etiology

 Assess RV outflow tract, monitor degree of pulmonary insufficiency.

TRANSPOSITION OF THE GREAT ARTERIES

 This defect results in two parallel circulations:

 Auscultation: varies; no murmur, or a murmur related to an associated defect, single S2.

 Stabilize with PGE1 infusion.

 Arterial switch operation (Jatene)â€”procedure of choice:

 Stabilize with PGE1 infusion.

HYPOPLASTIC LEFT HEART SYNDROME

 Critical mitral stenosis or atresia.

Pathophysiology and Etiology

 The left side of the heart is underdeveloped and essentially nonfunctional.

 Resuscitation and stabilization with PGE1 infusion.

 May need balloon atrial septostomy to allow unrestrictive LA to RA blood flow.

 Palliative, staged repair:

NURSING CARE OF THE CHILD WITH CONGENITAL HEART DISEASE

 Obtain a thorough nursing history.

 Impaired Gas Exchange related to altered pulmonary blood flow or pulmonary

 Position the child in a reclining, semi-upright position.

Improving Cardiac Output

Improving Oxygenation and Activity Tolerance

Providing Adequate Nutrition

 For the infant:

Reducing Fear and Anxiety

 Educate the patient and family.

Family Education and Health Maintenance

 Instruct the family in necessary measures to maintain the child's health:

Evaluation: Expected Outcomes

 Improved oxygenation evidenced by easy, comfortable respirations

CONGESTIVE HEART FAILURE

 May result from the following:

 Impaired myocardial function.

 Characteristic physical examination findings.

 Diuretics to reduce intravascular volume (furosemide, spironolactone).

 Assess response to medical treatment plan.