2. Fetal Circulation
Before birth, blood from the placenta,
about 80% saturated with oxygen, returns
to the fetus by way of the umbilical vein.
On approaching the liver, most of this
blood flows through the ductus venosus
directly into the inferior vena cava,
bypassing the liver.
After a short course in the inferior vena
cava, where placental blood mixes with
deoxygenated blood returning from the
lower limbs, it enters the right atrium.
3. Fetal Circulation
Here it is guided toward the oval foramen by
the valve of the inferior vena cava, and most
of the blood passes directly into the left
atrium.
From the left atrium, where it mixes with a
small amount of desaturated blood returning
from the lungs, blood enters the left ventricle
and ascending aorta.
Since the coronary and carotid arteries are
the first branches of the ascending aorta, the
heart musculature and the brain are supplied
with well-oxygenated blood.
4. Fetal Circulation
A small amount from the IVC is prevented
from entering the left atrium and remains
in the right atrium.
It mixes with desaturated blood returning
from the head and arms by way of the
superior vena cava.
Desaturated blood from the superior vena
cava flows by way of the right ventricle
into the pulmonary trunk.
5. Fetal Circulation
During fetal life, resistance in the
pulmonary vessels is high, such that most
of this blood passes directly through the
ductus arteriosus into the descending
aorta, where it mixes with blood from the
proximal aorta.
After coursing through the descending
aorta, blood flows toward the placenta by
way of the two umbilical arteries.
The oxygen saturation in the umbilical
arteries is approximately 58%.
6. Fetal Circulation
N.B.
A. A small amount of blood enters the liver sinusoids
and mixes with blood from the portal circulation.
B. A sphincter mechanism in the ductus venosus,
close to the entrance of the umbilical vein, regulates
flow of umbilical blood through the liver sinusoids.
This sphincter closes when a uterine contraction
renders the venous return too high, preventing a
sudden overloading of the heart.
During its course from the placenta to the organs of
the fetus, blood in the umbilical vein gradually loses
its high oxygen content as it mixes with desaturated
blood.
7. Fetal Circulation
Theoretically, mixing may occur in the following
places :
1) In the liver by mixture with a small amount of
blood returning from the portal system.
2) In the inferior vena cava which carries
deoxygenated blood returning from the lower
extremities, pelvis, and kidneys.
3) In the right atrium by mixture with blood returning
from the head and limbs.
4) In the left atrium by mixture with blood returning
from the lungs.
5) At the entrance of the ductus arteriosus into the
descending aorta.
8.
9. Congenital heart disease (CHD)
Congenital heart disease is a defect in the
structure of the heart and
great vessels which is present at birth
• CHD are the main cause of defect-related deaths
• Incidence is 8-9/1000 live births
• More common in premature infants
• May be associated with a significant musculoskeletal
defect
(e.g. diaphragmatic hernia, tracheo-oesophageal fistula,
imperforate anus)
10. Congenital heart disease (CHD)
• Causes are multifactorial and
include maternal illness
(diabetes mellitus,
phenylketonuria, and systemic
lupus erythematosus),
maternal infections (Rubella),
drugs (lithium, thalidomide),
known teratogens, harmful
habits (alcohol, hydantoin)
and associations with
chromosomal abnormality or
other recognized patterns of
malformation or syndrome; Trisomy 18
100% have CHD
11. Central cyanosis
• noted in the trunk, tongue, mucous membranes
• due to reduced oxygen saturation
Peripheral cyanosis
• noted in the hands and feet, around mouth
• due to reduced local blood flow
Recognition of Cyanosis
12.
13. ASSESSMENT OF HEART
DISORDERS IN CHILDREN
History
Physical
assessment
general
appearance
pulse, blood
pressure, &
respirations
15. Atrial Septal Defect
ASD is an opening in the atrial septum permitting
free communication of blood between the atria.
Seen in 10% of all CHD.
16. Atrial Septal Defect
Clinical Signs & Symptoms
Rarely presents with signs of CHF or other
cardiovascular symptoms.
• Most are asymptomatic but may have easy
fatigability or mild growth failure.
• Cyanosis does not occur unless pulmonary HTN
is present.
17. Atrial Septal Defect pathophysiology
1. Oxygenated blood is shunted
from left to right side of the heart
via defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs
congestive heart failure
18. Treatment
Medical Management
Medications – digoxin
Cardiac Catheterizaton -
Amplatzer septal occluder
Open-heart Surgery
19. Atrial Septal Defect
Treatment:
Surgical or catherization laboratory closure is
generally recommended.
• Closure is performed electively between ages 2 &
5 yrs to avoid late complications.
• Surgical correction is done earlier in children w/
CHF or significant Pulm HTN.
20. Treatment
Device Closure – Amplatzer septal occluder
During cardiac catheterization the occluder is placed in the
Defect
21. Cardiac Catheterization
Pre-care:
History and Physical
Lab work – EKG, ECHO cardiogram, CBC
NPO
Preprocedural teaching
Post Care:
Monitor vital signs
Monitor extremity distal to the catheter instertion,
Keep leg immobilized
Vital signs
Check for bleeding at insertion site
Measure I&O
22. Ventricular Septal Defect
VSD – is an abnormal opening in the ventricular
septum, which allows free communication
between the Rt & Lt ventricles. Accounts for 25%
of CHD.
23. Ventricular Septal Defect
Hemodynamics
The left to right shunt occurs secondary to
PVR being < SVR, not the higher pressure in
the LV.
This leads to elevated RV & pulmonary
pressures & volume hypertrophy of the LA &
LV.
24. Ventricular Septal Defect
Clinical Signs & Symptoms
• Small - moderate VSD, 3-6mm, are usually
asymptomatic and 50% will close
spontaneously
by age 2yrs.
• Moderate – large VSD, almost always have
symptoms and will require surgical repair.
26. Ventricle Septal Defect
pathophysiology
1. Oxygenated blood is shunted
from left to right side of the
heart via defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs
congestive heart failure
27. Ventricular Septal Defect
Treatment
• Small VSD - no surgical intervention, no
physical restrictions, just reassurance and
periodic follow-up and endocarditis prophylaxis.
• Symptomatic VSD - Medical treatment
initially with afterload reducers & diuretics.
28. Ventricular Septal Defect
Treatment
Indications for Surgical Closure:
Large VSD with medically uncontrolled
symptomatology & continued FTT.
Ages 6-12 mo with large VSD & Pulm. HTN
30. Patent Ductus Arteriosus
PDA – Persistence of the normal fetal vessel
that joins the PA to the Aorta.
Normally closes in the 1st wk of life.
Accounts for 10% of all CHD, seen in 10% of
other congenital hrt lesions and can often play
a critical role in some lesions.
Female : Male ratio of 2:1
Often associated with coarctation & VSD.
31. Patent Ductus Arteriosus
Hemodynamics
As a result of higher aortic pressure, blood
shunts L to R through the ductus from Aorta
to PA.
Extent of the shunt depends on size of the
ductus & PVR:SVR.
Small PDA, pressures in PA, RV, RA are
normal.
32. Patent Ductus Arteriosus
Clinical Signs & Symptoms
Small PDA’s are usually asymptomatic
Large PDA’s can result in symptoms of CHF,
growth restriction, FTT.
Bounding arterial pulses
Widened pulse pressure
Enlarged heart, prominent apical impulse
Classic continuous machinary systolic murmur
Mid-diastolic murmur at the apex
33. Patent Ductus Arteriosus pathophysiology
1. Blood shunts from
aorta (left) to the
pulmonary artery
(right)
2. Returns to the
lungs causing
increase pressure
in the lung
3. Congestive heart
failure
34. Patent Ductus Arteriosus
Treatment
Indomethacin, inhibitor of prostaglandin synthesis
can be used in premature infants.
PDA requires surgical or catheter closure.
Closure is required treatment heart failure & to
prevent pulmonary vascular disease.
Usually done by ligation & division or intra
vascular coil.
Mortality is < 1%
36. Treatment for PDA
Cardiac Catheterization
Insert coil – tiny fibers
occlude the ductus
arteriosus when a
thrombus forms in the
mass of fabric and wire
38. Pulmonary Stenosis
Pulmonary Stenosis is
obstruction in the region of
either the pulmonary valve or
the subpulmonary ventricular
outflow tract.
Accounts for 7-10% of all
CHD.
Most cases are isolated
lesions
Maybe biscuspid or fusion of
2 or more leaflets.
Can present w/or w/o an
intact ventricular septum.
39. Pulmonary Stenosis
Hemodynamics
RV pressure hypertrophy RV failure.
RV pressures maybe > systemic pressure.
Post-stenotic dilation of main PA.
W/intact septum & severe stenosis R-L shunt
cyanosis.
Cyanosis is indicative of Critical PS.
40. Pulmonary Stenosis
Clinical Signs & Symptoms
Depends on the severity of obstruction.
Asymptomatic w/ mild PS < 30mmHg.
Mod-severe: 30-60mmHg, > 60mmHg
Prominent jugular wave.
Split 2nd hrt sound w/ a delay
Heart failure & cyanosis seen in severe cases.
41. Pulmonary Stenosis
Treatment
Mild PS no intervention required, close follow-
up.
Mod-severe – require relieve of stenosis.
Balloon valvuloplasty, treatment of choice.
Surgical valvotomy is also a consideration.
42. Pulmonic Stenosis
Treatment:
Medications – Prostaglandins to keep the PDA open
Cardiac Catheterization
Baloon Valvuloplasty
Surgery
Valvotomy
43. Aortic Stenosis
Aortic Stenosis is an obstruction to the
outflow from the left ventricle at or near the
aortic valve that causes a systolic pressure
gradient of more than 10mmHg. Accounts for
7% of CHD.
3 Types
Valvular – Most common.
Subvalvular(subaortic) – involves the left
outflow tract.
Supravalvular – involves the ascending aorta
is the least common.
44. Aortic Stenosis
Hemodynamics
Pressure hypertrophy of the LV and
LA with obstruction to flow from the
LV.
Mild AS 0-25mmHG
Moderate AS 25-50mmHg
Severe AS 50-75mmHg
Critical AS > 75mmHg
45. Aortic Stenosis
Clinical Signs & Symptoms
Mild AS may present with exercise
intolerance, easy fatigabiltity, but usually
asymptomatic.
Moderate AS – Chest pain, dypsnea on
exertion, dizziness & syncope.
Severe AS – Weak pulses, left sided heart
failure, Sudden Death.
LV thrust at the Apex.
Systolic thrill @ rt base/suprasternal notch
46. Aortic Stenosis
Treatment
Balloon valvuloplasty is the standard of
treatment. Aortic insufficiency & re-stenosis is
likely after surgery and may require valve
replacement.
Activity should not be restricted in Mild AS.
Mod-severe AS, no competitive sports.
47. Coarctation of the Aorta
Coarctation- is narrowing of the aorta at
varying points anywhere from the transverse
arch to the iliac bifurcation.
98% of coarctations are juxtaductal
Male: Female ratio 3:1.
Accounts for 7 % of all CHD.
48. Coarctation of the Aorta
Hemodynamics
Obstruction of left ventricular outflow pressure
hypertrophy of the LV.
49. Coarctation of the Aorta
Clinical Signs & Symptoms
Classic signs of coarctation are diminution or
absence of femoral pulses.
Higher BP in the upper extremities as compared
to the lower extremities.
90% have systolic hypertension of the upper
extremities.
Pulse discrepancy between rt & lt arms.
50. Coarctation of the Aorta
Clinical Signs & Symptoms
With severe coarc LE hypoperfusion, acidosis,
HF and shock.
Differential cyanosis if ductus is still open
Cardiomegaly, rib notching on X-ray.
absence of femoral pulses
1. Radial pulses full/bounding and femoral or
popliteal pulses weak or absent
2. Leg pains, fatigue
3. Nose bleeds
52. Coarctation of the Aorta
Treatment
With severe coarctation maintaining the
ductus with prostaglandin E is essential.
Surgical intervention, to prevent LV
dysfunction.
Angioplasty is used by some centers.
Re-coarctation can occur, balloon angioplasty
is the procedure of choice.
57. Cyanotic Spell
most signif prob to develop in infants and toddlers
with cyanotic heart disease
brought on by crying, feeding, exercise, warm
bath, or straining to defecate
during a hypoxic spell, child will often squat knee
to chest to decrease venous return (by incr
systemic vascular resistance) from LE which decr
CO and relieves the cyanotic spell.
58. Tetralogy of Fallot
combination of four defects
pulmonary stenosis: degree determines severity
VSD
over-riding of the aorta
RVH
accounts for 10% of CHD
elevated R sided pressures: R to L shunt
xray: boot shaped heart d/t RVH
risk for metabolic acidosis and syncope.
59. Tetralogy of Fallot’s
Tetralogy of Fallot
Four anomalies
Pulmonary stenosis
VSD
Dextroposition of the
aorta
Hypertrophy of right
ventricle
60. Clinical manifestation of TOF
Symptoms are variable depending of
degree of obstruction
Cyanosis
Digital clubbing and hyperpnea at rest are
directly related to the degree of cyanosis
Tachycardia
Mental retardation
Retarded growth and development
RV heave
Systolic ejection murmur is heard along the left
sternal border
61. Polycythemia
Paroxymal dyspnea
Severe dyspnea on exertion
Squatting position for the relief of dyspnea
caused physical effort,
“Blue” spells, paroxysmal hypercyanotic attacks
– infant becomes hyperpnea, restless, cyanosis
increases, gasping respirations, syncope
62. Hypercyanotic Spells/Blue Spells/Tet
Spells
Clinical Manifestations
٭ Most often occurs in morning after feedings,
defecation, or crying
٭ Acute cyanosis
٭ Hyperpnea
٭ Inconsolable crying
٭ Hypoxia which leads to acidosis
64. Treatment of TOF
total repair is done by 6 mo if cyanotic spells
surgery is not necessarily curative, but most have
improved quality of life and improved longevity
residual problems: arrhythmias and RV
dysfunction
67. Surgical treatment
cardiac catheterization, which may include
procedural treatment in the cath lab
valve replacement
conduit placement
cardiac transplant
69. Treatment of the Child with TOF
Decrease cardiac workload
Prevention of intercurrent infection
Prevention of hemoconcentration
Surgical repair – palliative or corrective surgery
71. Nursing Care:
Monitor VS
I&O
Medications
Position
Metabolic rest
Assess and document child/family
interactions
Parent teaching
72. Transposition of Great Vessels
Aorta arises from the right
ventricle, and the pulmonary
artery arises from the left
ventricle - which is not
compatible with survival
unless there is a large
defect present in ventricular
or atrial septum.
artery
aorta
74. Transposition of the Great Arteries
Pathophysiology
Cyanosis due to failure of delivery of pulmonary venous
blood to the systemic circulation
Two parallel circulations with no mixing
Open atrial septum (fossa ovalis) allows some left-to-right
shunt, enhanced by a left-to-right ductus arteriosus shunt
Presence of ventricular septal defect facilitates mixing
75. Transposition of the Great
Arteries
Aorta from right ventricle, pulmonary artery from
left ventricle.
Cyanosis from birth, hypoxic spells sometimes
present.
Heart failure often present.
Cardiac enlargement and diminished pulmonary
artery segment on x-ray.
76. Transposition of the Great
Arteries
Anatomic communication must exist between
pulmonary and systemic circulation, VSD, ASD,
or PDA.
Untreated, the vast majority of these infants
would not survive the neonatal period.
77. Transposition of the Great Arteries
Clinical Manifestations
Cyanosis, tachypnea are most often recognized
within the 1st hrs or days of life.
Hypoxemia is usually moderate to severe,
depending on the degree of atrial level shunting and
whether the ductus is partially open or totally closed.
Physical findings, other than cyanosis, may be
remarkably nonspecific.
Murmurs may be absent, or a soft systolic ejection
murmur may be noted at the midleft sternal border.
78. Transposition of the Great Arteries
Chest film
Oval-shaped heart
Narrow mediastinum
Normal or increased pulmonary vascular markings
79. D-Transposition of the Great Arteries
This condition is a medical emergency,
and only early diagnosis and appropriate intervention can
avert the development of prolonged severe hypoxemia and
acidosis,
80. Treatment
When transposition is suspected, an infusion of prostaglandin
E1 should be initiated immediately to maintain patency of the
ductus arteriosus and improve oxygenation.
Endotracheal intubation
Infants who remain severely hypoxic or acidotic despite
prostaglandin infusion should undergo Rashkind balloon atrial
septostomy
A Rashkind atrial septostomy is also usually performed in all
patients in whom any significant delay in surgery is necessary.
81. Preventing Birth
Defects
Stop smoking
Avoid drinking alcohol while pregnant
Take a daily vitamin containing folic acid
Antenatal to make sure any medication (over-the-
counter or prescription) is safe to take during
pregnancy
Stop use of any illegal or "street" drugs
82. Nursing interventions pre and post
cardiac catheterization
Assessment pre-op for baselines
Assessment post-op:
Vital signs (which ones are priority?)
Extremities
Activity
Hydration
Medications
Comfort measures
83. Teaching after cardiac catheterization
Parental teaching
Watch for s/s of bleeding, bruising at site
Loss of sensation in foot on side of cath
When to call the physician
If any of above s/s noted within 1st 24 hrs
84. Rheumatic Fever
inflammatory connective tissue disorder that
follows initial infection by group A beta-hemolytic
streptococci
may lead to permanent mitral or aortic valve
damage
migratory polyarthritis, subcutaneous nodules,
fevers, chorea movements.
85. Rheumatic fever
S/S
Systolic murmur
Chorea (sudden involuntary movement of the limbs)
Macular rash on the trunk
Swollen and tender joints, Subcutaneous nodules
Positive ASO titer and increased ESR and C-reactive protein
Fever
Lethargy/general malaise
Anorexia
Splenomegaly
Retinal hemorrhages
86. Treatment for Rheumatic Fever
antibiotics to treat the strept infection: pcn,
erythromycin
Analgasic for joint pain and fever
monitored by cardiac echo (serial)
steroids for severe carditis with CHF
long term antibiotics until adulthood
1x/mo IM (Pen G)
Bedrest
Prognosis depends on how much heart involvement
87. Principles that apply to all cardiac
conditions:
Encourage normal growth and
development
Counsel parents to avoid overprotection
Address parents’ concerns and anxieties
Educate parents about conditions, tests,
planned treatments, medications
Assist parents in developing ability to
assess child’s physical status
88. Rheumatic heart disease
Rheumatic heart disease is a condition
in which the heart valves have been
permanently damaged by rheumatic
fever. The heart valve damage may start
shortly after untreated or under-treated
streptococcal infection such as strep
throat or scarlet fever. An immune
response causes an inflammatory
condition in the body which can result in
on-going valve damage
89. Sign and symptoms
THEORITICAL
These are the most common
sign and symptoms of
Rheumatic fever:
• Fever
• Swollen, tender, red and
extremely painful joints —
particularly the knees and
ankles
• Nodules (lumps under the skin)
• Red, raised, lattice-like rash,
usually on the chest, back, and
abdomen
• Shortness of breath and chest
discomfort
CLINICAL
Carditis
Arthritis
Chorea
Erythema marginatum
Arthralgia
90. CONTI
THEORITICAL
• Uncontrolled movements of
arms, legs, or facial
muscles
• Weakness
Symptoms of Rheumatic
heart disease depend on
the degree of valve
damage and may include:
• Shortness of breath
(especially with activity or
when lying down)
• Chest pain
• Swelling
CLINICAL
Subcutaneous
nodules
91. DIAGNOSIS
THEORITICAL
History
Physical examination
Tests.
chest x ray
ECG
ECHO
Cardiac MRI
Blood test
CLINICAL
Elevated ESR ,
Elevated C reactive
protein and leukocytosis
ECG P-R interval
prolong
Chest X rays shows
enlarged heart
92. TREATMENT
THEORITICAL
Treatment depends in
large part on how
much damage has
been done to the
heart valves. In
severe cases,
treatment may include
surgery to replace or
repair a badly
damaged valve.
CLINICAL
Benzyle penicillin
Aspirin
Erythromycin
94. COMPLICATIONS
THEORY
Some complications of rheumatic heart
disease include:
• Heart failure. This can occur from either
a severely narrowed or leaking heart
valve.
• Bacterial endocarditis. This is an
infection of the inner lining of the heart,
and may occur when rheumatic fever has
damaged the heart valves.
• Complications of pregnancy and
delivery due to heart damage. Women
with rheumatic heart disease should
discuss their condition with their
healthcare provider before getting
pregnant.
• Ruptured heart valve. This is a medical
emergency that must be treated with
surgery to replace or repair the heart
valve.
CLINICAL
Atrial hypertension
Heart failure
Atrial fibrillation
Recurrence of acute
rheumatic fever
Endocarditis
95. Nursing management
Assess the child’s pain perception using an
appropriate scale every 2 to 3 hours
. Provides information about the pain level
of the child.
Assess changes in behavior, such as high-
pitched cry, irritability , restlessness, refusal to
move, facial grimace, aggressive or
dependent behavior.
96. Nonverbal pain descriptions that are age-related
as child or infant may be unable to describe pain;
fear and anxiety associated with pain cause
changes in behavioral responses.
Examine affected joints, degree of joint pain, level
of joint movement.