Pediatric Nursing

Congenital defects of heart and Cardio-vascular dysfunction.

 Outlines:

• Understanding Fetal circulation

• Congenital malformations of heart.
• Pharmacology related treatment modalities for the
above (Indomethacin and prostaglandin therapy)
• Rheumatic heart disease.
• Nursing Care approaches while dealing with clients
 Fetal Circulation Structures
• Umbilical vein; umbilical arteries
• Foramen ovale
• Ductus arteriosus
• Ductus venosus
 Fetal Circulation
• The placenta accepts the blood without oxygen
from the fetus through blood vessels that leave the
fetus through the umbilical cord (umbilical arteries,
there are two of them).
• When blood goes through the placenta it picks up
oxygen. The oxygen rich blood then returns to the
fetus via the third vessel in the umbilical cord
(umbilical vein). The oxygen rich blood that enters
the fetus passes through the fetal liver and enters
the right side of the heart.
Conti..

• The hole between the top two heart chambers

(right and left atrium) is called a
patent foramen ovale (PFO). This hole allows
the oxygen rich blood to go from the right
atrium to left atrium and then to the left
ventricle and out the aorta. As a result the
blood with the most oxygen gets to the brain.
Conti…
• Blood coming back from the fetus’s body also
enters the right atrium, but the fetus is able to
send this oxygen poor blood from the right
atrium to the right ventricle (the chamber that
normally pumps blood to the lungs). Most of
the blood that leaves the right ventricle in the
fetus bypasses the lungs through the second
of the two extra fetal connections known as
the ductus arteriosus.
Conti..
• The ductus arteriosus sends the oxygen poor
blood to the organs in the lower half of the
fetal body. This also allows for the oxygen
poor blood to leave the fetus through the
umbilical arteries and get back to the placenta
to pick up oxygen.
Changes at Birth
 Pediatric Indicators
of Cardiac Dysfunction
• Poor feeding
• Tachypnea/ tachycardia
• Failure to thrive/poor weight gain/activity
intolerance
• Developmental delays
• + Prenatal history
• + Family history of cardiac disease
 Two Types of Cardiac Defects
• Congenital
– Anatomic>abnormal function
• Acquired
– Disease process
• Infection
• Autoimmune response
• Environmental factors
• Familial tendencies
 CHD
• Incidence: 5-8 per 1000 live births
– About 2-3 of these are symptomatic in first year of
life
– Major cause of death in first year of life (after
prematurity)
– Most common anomaly is VSD
– 28% of kids with CHD have another recognized
anomaly (trisomy 21, 13, 18, +++ )
 Older Classifications of CHD
• Acyanotic
– May become cyanotic
• Cyanotic
– May be pink
– May develop CHF
 Newer Classification of CHD
• Hemodynamic characteristics
– Increased pulmonary blood flow
– Decreased pulmonary blood flow
– Obstruction of blood flow out of the heart
– Mixed blood flow
 Increased Pulmonary
Blood Flow Defects
• Abnormal connection between two sides of
heart
– Either the septum or the great vessels
• Increased blood volume on right side of heart
• Increased pulmonary blood flow
• Decreased systemic blood flow
Increased Pulmonary Blood Flow Defects

• Atrial septal defect

• Ventricular septal defect
• Patent ductus arteriosus
ASD
Opening between the atria
• Because L atrial pressure is slightly higher than R atrial
pressure, blood flows from L to R.
• Causes increased flow of oxygenated blood into the R atria. R
atria becomes distended. Because there is low pulmonary
vascular resistance, blood backs up into the pulmonary
vessels and the R ventricle becomes distended as well.
• But because it is under low pressure, this is often tolerated
very well and the child may be asymptomatic.
• Rarely see CHF in uncomplicated ASD. Minimal symptoms of
Pulmonary vascular changes until several decades of
unrepaired ASD. Risk for atrial dysrhythmias and emboli
formation in later life if unrepaired.
• TX: may be closed in cardiac cath procedure; surgical repair
w/ patch—usually before age 6.
VSD
• Defect in ventricular septum—Error in early fetal development
• Can occur anywhere in muscle or membranous ventricular septum
• 20-25% of all CHD’s are VSD
• Manifestations—Depends on size of vsd and degree of shunting Can be pinhole
size to absence of entire septum. Small to moderated defects may close
spontaneously within first year of life.
• Patho-phys:
• Pressure is higher in L ventricle than in the R ventricle and systemic arterial
circulation offers more resistance than the pulmonary circulation, blood flows
through the defect and into the pulmonary artery.
• R Ventricle becomes enlarged (hypertrophied); over time the R atria may also
become distended.
• Symptoms: Characteristic Murmur; CHF is common; risk of Bact endocarditis; risk
of pulmonary vascular obstructive disease.
• Severe cases: very severe; resistance in pulm blood flow is >> than systemic
circulation. Reversal of blood flow through ventricles.
• REPAIRS:
• Cath repairs in clinical trials
• Surgical repair w/ bypass; Pulmonary artery banding (if not too large) or patch
PDA
• Ductus SHOULD close by about age 15 hours after birth. Some shunting of blood may
occur up to 24 hrs of life. DUCTUS closes because increase in arterial oxygen
concentration that follows initiation of pulmonary function. ALSO-- in Prostaglandin
E leads to closure of PDA.
• 5-10% of all CHDs are PDA. More common in females (abt 3:1)
• Patent DA allows blood to flow from left to right and  pulmonary blood flow
• Manifestations:
• Small PDA may be asymptomatic
• Large PDA may be CHF w/ tachypnea, dyspnea, and hoarse cry.
• Symptoms:
• BOUNDING Peripheral pulses
• Widened Pulse Pressure (>25)
• Murmur (“machinery murmur”) at upper left sternal border or in L
infraclavicular area.
• Murmur audible throughout cardiac cycle
• DEFINITIVE DX: ECHO
• Management of PDA:
• Medical>> Preterm kids= INDOMETHICIN to close PDA’s; surgical ligation if
meds fail
• prophylactic antibx to prevent bacterial endocarditis
• Surgery>> between age 1-2 yrs
 Obstructive Defects
• Coarctation of the aorta
• Aortic stenosis
• Pulmonic stenosis
Coarctation of the Aorta
• The aorta is narrowed near the insertion of the ductus arteriosus.
Increased pressure proximal to the defect. Causes high BP &
bounding pulses in arms; weak or absent femoral pulses, and cool
lower extremities with low BP.
• Signs of CHF in infants. Condition can deteriorate rapidly.
• Older kids may c/o dizziness, fainting and epistaxis from
hypertension.
• Patient at risk for ruptured aorta,
aortic aneurysm, or stroke

• TX: Non-surgical= balloon angioplasty. Usually effective

• Surgical: Does not require bypass since defect is outside
pericardium. Post-op complication is hypertension. Usually done
before age 2 yrs.
Aortic Stenosis
Narrowing of aortic valve Usually malformed in BI- rather than
TRI-cuspid valve.
• Causes increased resistance in left ventricle, decreased CO, L
ventricular hypertrophy and pulmonary vascular congestion.
• L Ventricular wall is hypertrophied>>increased pulmonary
vascular resistance and pulm HTN.
• L Ventricular hypertrophy >> decreased coronary artery
perfusion & increased risk of MI
• Clinical manifestations: Infants w/ severe defects demonstrate
signs of decreased CO. Faint pulses, hypotension, poor
feeding, tachycardia. Have a murmur. Exercise intolerance.
Chest pain, dizziness w/ standing.
• TX: Balloon Angioplasty to dilate the valve; or Surg: Konno
procedure [valve replacement]. May require repeat
procedures.
Pulmonic Stenosis and
Catheter Placement
• Pulmonary valve is stenosed. Narrowing @ entrance to
pulmonary artery. >>R Ventricular hypertrophy and
decreased pulm blood flow.
• Extreme form of PS is Pulmonary atresia (total fusion of
the commissures and no blood flow to lungs)
• PS>>R vent hypertrophy, R ventricular failure>> R atrial
pressure increases and may reopen foramen ovale.
Shunts un-oxygenaeted blood to L atrium>>systemic
cyanosis. May lead to CHF. Often have PDA as well.
• Cardiomegaly on CXR; TX: Balloon angioplasty to dilate
the valve.
• SURG TX—Brock procedure (Bypass to do valvotomy.
Usually can repair with catheterization.
Decreased Pulmonary
Blood Flow Defects
• Pulmonary blood flow is obstructed + have
defect of ASD or VSD.>>Blood backs up in R
side of heart. Causes desaturated blood to
shunt to the left, and into systemic circulation.
Usually hypoxemic and usually cyanotic. Most
common defects are TET & tricuspid atresia.
Tetralogy of Fallot
 Tetralogy of Fallot
• Tetra means 4. 4 Defects are:
• VSD
• Pulmonic stenosis
• Overriding aorta
• R Ventricular hypertrophy.
• Hemodynamics vary widely; depends on extent of pulmonic valve
stenosis & size of VSD. IF VSD is large pressures are = in R and L
ventricles. Blood is shunted in the direction of the least resistance
(pulm or systemic vascular resistance.
• If PVR is > than Systemic Vasc resistance, shunt with be right to left.
• Clinical manifestations: Vary with types of defect. “TET SPELLS” or “blue
spells” with acute episodes of cyanosis and hypoxia. May be anoxic
after feeding or w/ crying. RISK of emboli, LOC, Sudden death , Seizures.
• REPAIRS: usually indicated when tet spells and hypercyanotic spells
increase.
Tetralogy of fallot
Tricuspid Atresia
 Mixed Defects
• Transposition of great vessels
• Total anomalous pulmonary venous
connection
• Hypoplastic heart syndrome
– Right
– Left
• Blood is mixed from pulmonary and systemic
circulations within the heart chambers.
>>Relative desaturation of blood in systemic
blood flow. Cardiac Output decreases because
of volume load on ventricle. Signs of
desaturations, cyanosis, and CHF, but variable
depending on anatomy.
Transposition of Great Vessels
• Pulmonary artery leaves the L ventricle and the aorta
exits from the R ventricle. No communication
between the systemic and pulmonary circulations.
Must have PDA or Septal defect to permit blood flow.
Surgical TX of choice is Arterial switch procedure to
resect and reanastomose great vessels. Coronary
arteries have to be reimplanted to supply myocardial
circulation. Other procedures possible, depending on
defect.
Totally Anomalous Pulmonary Venous
Connection
• Rare Defect. Pulmonary veins fail to join L atrium.
Pulm veins drain into R atrium. Results in mixed
blood.
• Clinical manifestations: Usually cyanotic early on.
Condition rapidly deteriorates as pulmonary blood
flow increases and causes CHF. SURG TX: Common
pulmonary vein is anastomosed to the L atrium, ASD
is closed and anomalous venous connections ligated.
Success depends on specifics of anomalies.
Hypoplastic Left Heart
• L side of heart is underdeveloped. L ventricle
is small and aortic atresia. Most blood flows
across patent foramen ovale to R atrium to R
ventricle and out the pulmonary artery.
Descending aorta receives blood from the PDA
to supply the systemic circulation. PDA closure
>>rapid deterioration and CHF.
• TX: Keep ductus open w/ Prostaglandin E
infusion . SURG TX: #1 is Norwood procedure
to create a new aorta using the main
pulmonary artery and creation of large ASD.
• #2 is Bidirectional
Glenn Shunt @ 6-9
months age to
reduce volume load
on the R ventricle.
#3 is modified Fontan
procedure, similar to
Tricuspid atresia repair.
Transplant may be option
for some pts. Mortality rate
very high (30-50% mortality
rates).
 CHF in Children
• Impaired myocardial function
– Tachycardia, fatigue, weakness, restless, pale, cool
extremities, decreased BP, decreased urine output
• Pulmonary congestion
– Tachypnea, dyspnea, respiratory distress, exercise
intolerance, cyanosis
• Systemic venous congestion
– Peripheral and periorbital edema, weight gain, ascites,
hepatomegaly, neck vein distention
 Interventional Cardiac Catheter
Procedures in Children

• Transposition of great vessels

• Some complex single-ventricle defects
• ASD
• Pulmonary artery stenosis
 Rheumatic Fever
Rheumatic Heart Disease
• RF
– Inflammatory disease occurs after Group A
ß-hemolytic streptococcal pharyngitis/
tonsilitis
• Affects joints, skin, brain, serous surfaces, and
heart
• Rheumatic heart disease
– Most common complication of RF
– Damage to valves as result of RF
 Prevention of RHD
• Treatment of streptococcal tonsillitis/pharyngitis
– Penicillin G—IM X 1
– Penicillin V—Oral X 10 days
– Sulfa—Oral X 10 days
– Erythromycin (if allergic to above)—Oral X 10 days
• Treatment of recurrent RF
– Same as above