DEFECTS THAT INCREASE PULMONARY BLOOD FLOW
Defects Description
Ventricular Septal Defect ● Acyanotic Disorder
● Most common defect found in
children
● VSD occurs when a portion of the
ventricular septum does not
completely close
Assessment Therapeutic Management
● Allows oxygenated blood to flow
from the left side of the heart to the
right side of the heart
● Increases pulmonary blood flow and
ultimately increases the volume of
blood returning to the left heart,
causing left heart dilation over time.
● Harsh, holosystolic murmur noted at
the left lower sternal border
● VSD may not be heard at birth
because the pulmonary vascular
resistance is still high
● Fatigue
● Diagnosed by:
○ X-ray
○ ECG
○ Echocardiography with
Doppler/MRI
● Administer diuretic or digoxin
○ Help prevent fluid from
accumulating in the lungs
● Some types of VSDs can be closed
in the catheterization lab (using
septal occluder device); others are
closed surgically
● If a child has a small defect that is
causing no clinical concerns, the
defect does not need to be closed
● Postoperatively, listen for arrhythmia
complication
Atrial Septal Defect ● Acyanotic Disorder
● Abnormal communication between
the two atria, allowing blood to shift
from the left to the right atrium
● Blood flow is from left to right
(oxygenated to deoxygenated)
● Two types of ASDs:
○ ostium primum (ASD1):
where the opening is at the
lower end of the septum
○ ostium secundum (ASD2):
where the opening is near
the center of the septum
● Harsh systolic murmur is heard over
the second or third interspace (the
pulmonic area)
● Echocardiography with color flow
Doppler: reveal enlarged right side
of the heart and increased
pulmonary circulation
● Cardiac catheterization: reveal the
separation in the atrial septum and
the increased oxygen saturation in
the right atrium
● Surgery to close the defect is done
electively between 1 and 3 years of
age.
● Closure is important because
without it, a child is at risk for
infectious endocarditis and heart
failure.
● Important to repair in girls because
they can cause emboli during
pregnancy.
● If the defect is very large, a Silastic
or Dacron patch may be sutured into
place to occlude the space
Atrioventricular Canal ● Also called endocardial cushion
Defect defect
● Results from incomplete fusion of
the endocardial cushion
● Blood may flow between all four
heart chambers with this defect
● ECG often will reveal first degree ● Pulmonary artery banding: increases
heart block pressure in the pulmonary artery
● Echocardiography will confirm the and right side of the heart, reducing
diagnosis the amount of shunting
● Surgery: necessary for a final repair
because these defects are too large
to close spontaneously
● Postoperatively, closely observe
children for jaundice resulting from
red blood cell destruction as red
cells are destroyed by the newly
constructed valves
Patent Ductus Arteriosus ● Acyanotic Disorder: blood flowing
from the aorta is fully oxygenated
● Accessory fetal structure that
connects the pulmonary artery to the
aorta
● Fetal shunt fails to close after
several days of life
● Blood will shunt from the aorta
(oxygenated blood) to the pulmonary
artery (deoxygenated blood)
because of the increased pressure
in the aorta
● Occurs often in infants who are
preterm of difficult respirations at
birth
● Physical examination, the child has
a wide pulse pressure (the
difference between systolic and
diastolic blood pressures)
● Continuous (systolic and diastolic)
“machinery” murmur can be heard at
the upper left sternal border or under
the left clavicle in older children
● Prostaglandins: stimulate the ductus
arteriosus to remain open in fetal life
● Prescribed IV indomethacin or
ibuprofen and prostaglandin
inhibitors to effect closure
● If indomethacin is given side effects
may be: Headache, dizziness,
somnolence,nausea, etc.
● Insertion of Dacron-coated
stainless-steel coils by
cardiac catheterization when the
child is 6 months to 1 year of age
● Large defects can be closed
surgically by ductal ligation
 DEFECTS WITH OBSTRUCTION TO BLOOD FLOW
Defects Description
Pulmonary Stenosis ● Narrowing of the pulmonary valve or
the pulmonary artery just distal to
the valve
● Inability of the right ventricle to
evacuate blood by way of the
pulmonary artery because of the
obstruction
Assessment Therapeutic Management
● Asymptomatic or have signs of mild ● Management of the defect depends
(right-sided) heart failure on the severity of the stenosis and
● If the narrowing is severe: present the child’s age.
cyanosis ● Balloon angioplasty by way of
● typical systolic ejection murmur, cardiac catheterization
grade IV or V
crescendo–decrescendo in quality,
can be heard, usually loudest at the
upper left sternal border.
● ECG or echocardiography: reveal
right ventricular hypertrophy
● Cardiac catheterization:used for
interventional enlargement
Aortic Stenosis ● Stenosis, or stricture, of the aortic
valve prevents blood from passing
freely from the left ventricle of the
heart into the aorta
● increased pressure and hypertrophy
of the left ventricle occur
● systolic heart murmur at the right
second intercostal space (aortic
space), but the child may be
otherwise asymptomatic
● If severe, decreased cardiac output
evidenced by faint pulses,
hypotension, tachycardia, and
inability to suck for long periods
● Sudden death can occur when the
amount of oxygen needed by the
heart muscle on exertion exceeds
what is available
● ECG or echocardiography: reveal
left ventricular hypertrophy
● Stabilization with a beta-blocker or
calcium channel blocker may be
necessary to reduce cardiac
hypertrophy
● Artificial valve replacement for
correction: need exercise testing
● If prosthetic valve is used: receive
anticoagulation or antiplatelet
therapy and antibiotic prophylaxis
Coarctation of the Aorta ● Narrowing of the lumen of the aorta
due to a constricting band
● Two locations:
○ “Preductal” - constriction
between subclavian artery
and ductus arteriosus.
○ “Postductal” - constriction is
distal to the ductus
arteriosus.
● Increased blood pressure in the
heart and upper portions of the body
● Irritability, nosebleed, headache and
vertigo
○
● History and physical assessment ● Interventional angiography (a
● Absence of palpable femoral pulses balloon catheter) or surgery
● Absent brachial pulses ● Digoxin and diuretics: reduce the
● Leg pain on exertion because of the severity of congestive heart failure
diminished blood supply to their from hypertension
lower extremities
● Collateral arteries enlarge
● Echocardiography, ECG, MRI, or
x-ray examination: reveal left-sided
heart enlargement
 DEFECTS WITH MIXED BLOOD FLOW
Defects Description
Transposition of the ● aorta arises from the right ventricle
Great Arteries instead of the left
● pulmonary artery arises from the left
ventricle instead of the right
● Blood enters the heart from the vena
cava > right atrium > right ventricle >
aorta to body completely
deoxygenated
● This severe a defect is incompatible
with life
● Occur in large newborns (9 to 10 lb)
and more often in boys
Total Anomalous ● Pulmonary veins return to the right
Pulmonary Venous atrium or the superior vena cava
Return instead of to the left atrium
Assessment
● Cyanosis
● ECG may or may not reveal heart
changes
● Cardiac catheterization will reveal
the low oxygen saturation
● An absent spleen is often
associated with the disorder
● Infants are mildly cyanotic and tire
easily.
Therapeutic Management
● Prostaglandin: keep the ductus
arteriosus patent
● Balloon atrial septal pull-through
operation: enlarge the septal
opening
● Surgical correction of transposition
of the great vessel in infant (1 week
to 3 months of age)
● Surgery involves reimplanting the
pulmonary veins into the left atrium
● balloon atrial septal pull-through
procedure: enlarge a small foramen
ovale.
● The child may be maintained on a
continuous IV infusion
Truncus Arteriosus ● One major artery or “trunk” arises
from the left and right ventricles in
place of separate aorta and
pulmonary artery vessel
● Cyanotic ● Repair needs restructuring the
● Typical VSD murmur common trunk to create separate
vessels
● Some need a second surgical
procedure by school age
Hypoplastic Left Heart ● Left ventricle is nonfunctional
Syndrome ● Accompanying mitral or aortic valve
atresia
● Causes: right ventricle to
hypertrophy
● Detected by ultrasound prenatally
● mild to moderate cyanosis
● Echocardiography
● Prostaglandin therapy
● Inhaled nitrogen combined with
oxygen may be prescribed to
decrease PO
● Heart transplantation

DEFECTS WITH DECREASED PULMONARY BLOOD FLOW

Defects Description Assessment Therapeutic Management

Tricuspid Atresia ● Extremely serious disorder because
the tricuspid valve is completely
closed
● No blood to flow from the right
atrium to the right ventricle
● Extreme cyanosis, tachycardia,
dyspnea
● IV infusion of PGE1: ductus remains
open
● Surgery: construction of vena cava
to pulmonary artery shunt
● Fontan procedure: restructure the
right side of the heart

Tetralogy of Fallot ● 4 anomalies are present:
○ Pulmonary stenosis
○ VSD
○ dextroposition (overriding) of
the aorta
● Not exhibit a high degree of
cyanosis after birth
● Polycythemia: increase in the
number of red blood cells as the
body attempts to provide enough
● Surgery: done at 1 to 2 years of age
● Keep hypercyanotic episodes to a
minimum during this waiting time
● Administering:
○ oxygen, placing the baby in a
○ Hypertrophy of the right
ventricle
○ Deletion abnormality of
chromosome 22
RBC to supply oxygen to all body knee–chest position
parts ○ morphine sulfate
● If not corrected: severe dyspnea, ○ Propranolol (Inderal, a
growth restriction, clubbing of fingers beta-blocker)
will develop ○ Blalock-Taussig procedure:
● Squatting, knee chest position when temporary or palliative
resting surgical repair
● Develop syncope (fainting) and ○ Brock procedure: Full repair
hypercyanotic episodes (tet spells)
caused by decreased blood and
oxygen supply
● Develop cognitive challenge
● A loud, harsh, widely transmitted
murmur or a soft, scratchy, localized
systolic murmur in the left second,
third, or fourth parasternal
interspace may be present
● Diagnosed with:
○ History
○ physical symptoms
○ Echocardiography
○ ECG
○ Cardiac catheterization
○ Laboratory finding
 ACQUIRED HEART DISEASE

Defects Description Assessment Therapeutic Management

Congestive Heart Failure ● A result of:
○ congenital heart disorder
○ rheumatic fever
○ Kawasaki disease
○ infectious endocarditis
● Occurs because:
○ The myocardium of the heart
cannot pump and circulate
enough blood to supply
oxygen and nutrients to body
cells
○ Blood pools in the heart
(excessive preload)
○ After cardiac surgery or
rheumatic fever
● Occur in:
○ children under 1 year of age
● Tachycardia: heart attempts to beat
faster to move blood forward more
effectively
● Tachypnea/rapid breathing
● Hepatomegaly
● Irritable and restless: abdominal
pain caused by the liver distention
● Lower extremity edema
● Dyspnea
● Presence of rales and bloody
sputum on coughing
● Cyanotic
● Breathless,tired, and difficulty
feeding
● Enlarged liver
● Ascites present in peritoneal space
● As a rule, if the width of the heart is
more than half the width of the chest
(in a child over 1 year of age), the
heart is enlarged
● Echocardiography: heart failure
● ECG: Ventricular hypertrophy
● Diuretics: reducing workload of
heart, evacuating the accumulated
fluid (reduces preload)
○ furosemide (Lasix)
○ spironolactone (Aldactone)
● Digoxin: increase contractility and
slow tachycardia
● Hydralazine: decrease afterload
(arterial vasodilator)
● Nifedipine: calcium channel blocker
● Nitroprusside: direct-acting
vasodilator
● Captopril: angiotensin converting
enzyme (ACE) inhibitor
Persistent Pulmonary ● Results when:
Hypertension ○ pulmonary vascular
resistance present at birth
because of unopened alveoli
fails to fall to normal
● Occurs most often in:
○ full-term infants who have
perinatal asphyxia
● Occurs because:
○ hypoxia and acidosis from
respiratory difficulty cause
vasoconstriction of the
pulmonary artery
● Tachypnea ● Supportive therapy”
● Pulse oximetry: shows a low PO2 ○ Oxygen
from inability of blood to perfuse the ○ high-frequency oscillatory
lungs ventilation
● Echocardiogram: shows right-to-left ○ IV glucose
shunting across the patent ductus or ○ antibiotics
foramen ovale ○ medications to reduce
pulmonary resistance
○ low-dose dopamine: elevate
systemic blood pressure
● Sildenafil citrate:vasodilation and
reduced resistance
● Sodium bicarbonate: relieve
acidosis and reverse pulmonary
vasoconstriction
● Inhaled nitric oxide: promote
pulmonary vasodilatation
● Infants who do not respond to
common therapy may need ECMO
Rheumatic Fever ● Autoimmune disease that occurs as
a reaction to a group A
beta-hemolytic streptococcal
infection
● follows an attack of pharyngitis,
tonsillitis, scarlet fever, “streP
throat,” or impetigo
● children do not develop immunity:
streptococcal infections recur
● full course of rheumatic fever is 6 to
8 weeks
● maintained on bedrest only during
the acute phase of illness
● penicillin therapy & benzathine
penicillin: eliminate beta-hemolytic
streptococci
● Oral ibuprofen: reduce inflammation
and joint pain
● Corticosteroids: reduce in
● Flammation in children who are not
responding to ibuprofen
● Phenobarbital & diazepam: reducing
the purposeless movements of
chorea
● Mitral valve replacement to restore
heart function
Kawasaki Syndrome ● mucocutaneous lymph node
syndrome
● a febrile, multisystem disorder that
occurs almost exclusively in children
before the age of puberty
● incidence is higher in late winter and
spring
● Vasculitis (inflammation of blood
vessels) is the principal (and
life-threatening) finding
● begins with an acute phase (stage I) ● acetylsalicylic acid (aspirin) or
of high fever (102° to 104° F [39.0° ibuprofen decreases inflammation
to 40.0° C]) that does not respond to and blocks platelet aggregation
antipyretics ● Abciximab: specific for Kawasaki
● About 10 days after the onset, a disease
subacute phase begins ● IV immune globulin (IVIG): reduce
● The convalescent phase (stage II) the immune response
begins at about the 25th day and ● should not receive routine
lasts until 40 days. immunizations while taking IVIG:
● Stage III lasts from 40 days until the immunization will be ineffective
ESR returns to normal ● Steroids: contraindicated
● Criteria for Diagnosis of Kawasaki
Disease
○ Fever of 5 or more days’
duration
○ Bilateral congestion of ocular
conjunctiva
○ Changes of the mucous
membrane of the upper
respiratory tract: reddened
pharynx; red, dry, fissured
lips; or protuberance of
tongue papillae (“strawberry”
tongue)
○ Changes of the peripheral
extremities, such as
peripheral edema, peripheral
erythema, desquamation of
palms and soles
○ Rash
○ Cervical lymph node swelling
Endocarditis ● Inflammation and infection of the ● The onset of the illness is insidious
endocardium or valves of the heart ● Appear pale, anorexic and weight
● Caused by streptococci loss
● Arthralgia (pain in joints), malaise,
chills, or periods of sweating,
especially at night
● Petechiae
● Hemorrhages of the fingernails or
toenails
● left upper quadrant abdominal pain
● spleen may be enlarged
● Echocardiogram: shows vegetative
growths on the heart valves
● Diagnosis is confirmed by a blood
culture
Arrhythmias ● Holter monitors: detection of cardiac
arrhythmia
● Slowing of the heart rate during
inspiration
● normal rate resuming on expiration
● Ventricular tachycardia
● atrial fibrillation
Hypertension ● secondary manifestation of another ● 3 years of age, blood pressure
disease should be included as health
assessment
● Only when the blood pressure is still
elevated on a third occasion is
hypertension diagnosed
● common diseases associated with
hypertension in children are renal
and cardiac diseases
○ Cushing’s syndrome
○ primary hyperaldosteronism
○ adrenogenital syndrome
○ Pheochromocytoma (a tumor
of the adrenal gland)
● prophylactic administration of an
antibiotic before ear, nose, throat,
tonsils, or mouth surgery (and
before childbirth) to prevent
infectious endocarditis.
● nafcillin (Unipen) is prescribed
● Atropine: bradycardia
● Digoxin: decreasing and
strengthening the heart rate if
needed
● pacemakers implanted: steady heart
rhythm
● Cryo- or radioablation are
nonsurgical transvenous catheter
techniques: permanently disrupt an
abnormal arrhythmia
● Therapy for hypertension depends
on the underlying primary disease
● Essential hypertension (elevated
blood pressure for no identifiable
reason): reducing diet and urged to
increase the level of exercise to
reduce weight.
● not to use oral contraceptives:
elevate blood pressure
● Diuretic, vasodilators,
angiotensin-converting enzyme
(ACE) inhibitor
● Educate them and their parents
about the long-term effects of

Dyslipidemia ● increased lipids in blood serum
● Risk factors:
○ familial hypercholesterolemia
○ dominantly inherited disease
○ Obesity
○ sedentary lifestyle
○ high-fat diet
Cardiomyopathy ● structural or functional abnormality
of the ventricular myocardium
● impairs systolic function and leads
to heart failure.
● Idiopathic dilated cardiomyopathy
(IDC): viral respiratory or
gastrointestinal illness
● Hypertrophic cardiomyopathy
(HCM): common autosomal
dominant disease
○ brain tumor
● Screened for total serum cholesterol
● Acceptable level/borderline of total
cholesterol: 170 to 199 mg/dL
● LDL 110 to 129 mg/dL
● If total triglyceride, cholesterol, LDL
level is elevated: child’s diet should
be regulated
● hypercholesterolemia has no
symptoms
● Physical examination: reveals
ill-appearing child with severe
respiratory distress
● Weak peripheral pulses
● Decreased blood pressure
● Enlarged liver
● Chest radiograph echocardiogram,
and ECG reveal: enlarged heart
hypertension (increased risk of
heart and blood vessel disease)
● cholesterol-reducing agents
○ cholestyramine (Questran)
○ Side effects: large, bulky
stools
● HMG-CoA inhibitor
○ atorvastatin (Lipitor)
● Bed rest
● fluid restriction
● pharmacologic agents
○ Immune globulin