3. PDA: Pathophysiology, Manifestations,
and Clinical Therapy
• Pathophysiology • Therapy
– The Ductus Arteriosus Usually – EKG shows left ventricular
closes within the first hours hypertrophy
of life – PDA visualized on echo
– Common in preterm infants – Surgical Ligation
• Manifestations – Intravenous indomethicin
– Continuous “machine” like stimulates closure in
murmur premature infants but can
– Dyspnea not be used if CHF is present
– Tachycardia • Shunt
– Tachypnea – Left-to-right can be visualized
– Thrill in pulmonic area on echocardiogram
– May be asymptomatic – Increase in pulmonary blood
flow
5. ASD: Pathophysiology, Manifestations,
and Clinical Therapy
• Pathophysiology • Therapy
– Opening in the atrial septum – Dilated right ventricle on
– May occur alone or in echo secondary to blood
conjunction with a congenital overload and shunt size
defect – Spontaneous closure occurs
• Manifestations within the first 4 years of life
– Surgery or patch when
– Usually no symptoms in
closure does not occur or
infants and young children
when increased pulmonary
– With a large ASD, easy tiring, blood flow results in CHF
and poor growth occur
• Shunt
– Left-to-right resulting in
increased pulmonary blood
flow
7. VSD: Pathophysiology, Manifestations
and Clinical Therapy
• Pathophysiology • Therapy
– Opening in ventricular – If small x-ray and ekg reveal
septum little
• Manifestations – Echo establishes diagnosis if
– Only 15% are large enough to shunting is present
cause CHF or pulmonary – Most close within first 6
hypertension, or pulmonary months of life
infections – Surgical patching if poor
– Systolic murmur at third or growth is evident; otherwise
fourth left intercostal space treatment is conservative
• Shunt
– Left-to-right directly across
septum into pulmonary
artery
– Increased pulmonary blood
flow
8. Heart Defects with Decreased
Pulmonary Blood Flow
• Pulmonary Stenosis
• TOF-Tetralogy of Fallot
9. TOF: Pathophysiology, Manifestations,
• Pathophysiology
– Four defects
• Pulmonic Stenosis page 1371
• Right Ventricular Hypertrophy
• VSD
• Overriding Aorta
• Some kids have a fifth defect: ASD
• Manifestations
– Infant becomes hypoxic and cyanotic as the ductus arteriosus closes
– The degree of the pulmonary stenosis determines the severity of the
symptoms
– Systolic murmur in the pulmonic area and transmitted to suprasternal notch
– Polycythemia, hypercyanotic spell(tet spells), metabolic acidosis, poor
growth, clubbing, and exercise intolerance
– Knee chest squat of toddlers to decrease the return of systemic venous
blood to the heart
10. TOF: Pathophysiology, Manifestations,
and Clinical Therapy
• Therapy
– Diagnostic Tests
• X-ray shows “boot shaped”
heart due to large right
ventricle, prominent aorta
– Treatment
• Calm, give oxygen, and
morphine and propanolol to
decrease pulmonary vascular
resistance
• Modified BT shunt to delay
total correction surgery
• Shunt
– Right-to-left secondary to
elevated pressures on the right
side of the heart
13. TGA: Pathophysiology, Manifestations
• Pathophysiology- Parallel circulation
– Pulmonary Artery is the outflow tract for the left ventricle
– The Aorta is the outflow tract for the right ventricle
– Life threatening at birth, survival initially depends on an open ductus
arteriosus and foramen ovale
• Manifestations
– Cyanosis apparent soon after birth
• which does not improve with oxygen administration
• May be less apparent if VSD is present
– CHF may develop immediately, over days or weeks
– Tachypnea without retractions or other signs of dyspnea
– Long time to feed and need frequent rest periods b/c of rapid respiratory
rate and fatigue; growth failure may be seen as early as 2wks if not
corrected
– Systolic murmur if VSD present; otherwise none; S2 is loud
14. TGA: Clinical Therapy
• Therapy
– Diagnosis
• X-ray may reveal classis “egg-shaped heart” on a string
with enlarged ventricles
• Echo shows abnormal positioning of the great arteries
– Treatment
• Prostaglandin E1 is ordered to maintain a patent ductus
arteriosus until a palliative surgery can be performed
• Arterial switch performed before 1 week of life
• Balloon atrial septostomy can be performed to allow
mixing until surgery can be performed
18. Coarctation of the Aorta: Pathophysiology,
Manifestations
• Pathophysiology
– Narrowing or constriction of the descending aorta often near the
ductus arteriosus or subclavian artery which obstructs systemic flow
• Manifestations
– Many children are asymptomatic
– Severe constriction infants have cyanosis in the lower extremities
– Blood pressure lower in legs than arms
– Brachial and radial pulses are typically bounding and femoral weak or
absent
– Older children may complain of leg pain after exercising
– S2 is loud and single on auscultation
19. Coarctation of the Aorta: clinical
Therapy
• Therapy
– Diagnosis
• X-ray may show cardiomegaly
• Echo confirms narrowing and location
– Treatment
• In symptomatic newborns, PGE1 is given to reopen the
ductus arteriosus and promote blood flow to the lower
extremities
• Treatment to prevent CHF may include diuretics,
inotropic medications and oxygen
• Surgical correction is preferred
21. Congestive Heart Failure (CHF)
• Disorder in which circulation is inadequate to support the body’s
circulatory and metabolic needs.
• Etiology
– Most common cause
• May be caused by congenital heart defects that obstruct systemic blood
outflow tract or cause increased pulmonary blood flow
• children with uncorrected defects develop CHF with 6 to 12 months of life
– Other causes
• Problems with heart contractility
• Pathologic conditions that require high cardiac output(severe anemia,
acidosis, or respiratory disease)
• Acquired heart disease(cardiomyopathy, rheumatic heart disease, and
Kawasaki disease)
• Disorders such as Duchenne muscular dystrophy
22. CHF: Pathophysiology
• Pathophysiology
– Left-to-right shunts
result in increased blood
to the pulmonary system
and can result in
pulmonary hypertension
– Obstructive defects
restrict the flow of blood
so the heart muscle
hypertrophies to work
harder to force blood
through the structures
23. CHF: Manifestations in Infants
• Initial Manifestations • Later Manifestations
– Tiring easily, especially – Tachypnea
during feedings – Tachycardia
– Weight loss or lack of – Pallor
normal weight gain – Cyanosis
– Diaphoresis – Nasal Flaring
– Irritability – Grunting
– Frequent Respiratory – Retractions
infections
– Cough or crackles
– Third heart sound
24. CHF: Manifestations in Older
Children
• Initial Manifestations
– Exercise Intolerance
– Dyspnea
– Abdominal pain or
distention
– Peripheral edema
• Later Manifestations
– Generalized fluid volume
overload
– Jugular vein distention
25. CHF: Nursing Management
• Assessment
– Physiologic assessment
– Developmental assessment
• Planning and Implementation
– Administer and monitor
prescribed medications
• Lasix and digoxin see page 1388
– Maintain oxygen and myocardial
function
– Promote rest
– Foster development
– Provide adequate nutrition
– Provide emotional support
– Discharge planning and home
care teaching
• Evaluation
27. Kawasaki Disease
• Acute febrile, systemic
vascular inflammatory
disorder that affects the
small and midsize arteries,
including the coronary
arteries
• Leading cause of acquired
heart disease in children in
the US
• Etiology unknown, thought
to be caused by an
unidentified infectious
agent
28. Kawasaki Disease: Manifestations
and Clinical Therapy
• Manifestations
– Acute 1-2 weeks
• Irritability, high fever that persists more than 5 days, hyperemic
conjunctivae, red throat, swollen, hands and feet, rash on trunk and
perineal area, cervical lymph node enlargement(unilateral)
– Subacute 2-4 weeks
• Cracking lips and fissures, desquamation of the skin on the tips of toes
and fingers and toes, joint pain, cardiac disease, and thrombocytosis
– Convalescent stage 6-8 weeks
• Child appears normal but lingering signs of inflammation may be present
• Clinical Therapy
– Intravenous Immunoglobulin
– High does of aspirin for fever
– Hospitalization
29. Kawasaki Disease: nursing
management
• Assessment • Comfort
– Temperature, skin, eyes, I&O, – Skin clean and dry
weight, cardiac – Cool compresses, tepid baths
• Medication – Small frequent feeding
– Aspiring administration • Exercise
• Monitor for side – Passive ROM
effects(bleeding, GI • Discharge planning
upset) – Teaching on aspiring therapy
– Immune Globulin – Postpone live virus vaccines
• Treat and administer like (measles and varicella) for 11
a blood product months after immune
globulin administration,
others may be given on
schedule
30. Rheumatic Heart Disease
• Inflammatory disorder of the connective
tissue that results from an autoimmune
response to some strains of A beta-hemolytic
streptococci
• Affects joints, brain, and skin tissue
• Children between 5 and early adolescent are
more commonly infected
• Rheumatic heart disease develops in 10% of
individuals with rheumatic fever
31. Rheumatic Heart Disease:
Manifestations and Clinical Therapy
• Hallmark signs
– Occur three weeks after an untreated streptococcal infection
• Carditis involving the mitral or aortic valve is detected by presence
of a new murmur
• Chest pain
• Two or more large joints become inflamed with pain, welling,
tenderness, erythema, and heat
• Non pruritic skin rash with pink macules and blanching in the
middle of the lesions on the trunk
• Clinical Therapy
– Antibiotics (penicillin, to eradicate strep infection)
– Corticosteroids (to reduce inflammation)
32. Rheumatic Heart Disease: Nursing
Management
• Prevention • Medications
– Children with possible throat – Antibiotics and aspirin
infections would be cultured • Discharge Planning
for strep
– Recovery phase occurs at
• Hospitalization home
– Children with rheumatic fever • Activity limitations
should be hospitalized • Antibiotic prescribed
– Bed rest for monitoring – Teaching
carditis
• Reoccurrence of sore
– 4weeks bed rest if carditis
throat need for culture
develops
• Follow up care to monitor
heart function
