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Module 5 - Cardiovascular Disorders
I. Etiology and Pathophysiology of Fetal to Newborn Circulation:
A. Three Fetal Circulation Shunts
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
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Ductus venosus – a structure that shunts blood past the portal
circulation
Foramen ovale – an opening between the right and left atria of the
heart
Ductus arteriosus – a vessel between the aorta and the pulmonary
artery that shunts blood from the PA to aorta
* Shunts should close in several days after delivery.
B. Fetal Circulation:
1. Main blood flow:
Placenta  Umbilical Vein  Liver  Ductus Venosus  Inferior
VenaCava  Right Atrium Foramen Ovale (by pass going to lungs
for oxygenation)Left Atrium  Left Ventricle  Aorta  Body
2. Secondary blood flow:
Right Atrium  Right Ventricle  Pulmonary Artery 
Ductus Arteriosus (bypass going to the lungs because the blood is
already oxygenated)  Aorta  Body
3. Third route of blood flow – very minimal amount
Right Atrium  Right Ventricle  Pulmonary Artery  Lungs (needs
to perfuse the lungs and upper body with oxygen) Left Atrium 
Left Ventricle  Aorta  Body
C. Changes in Circulation at Birth



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The umbilical arteries and vein and the ductus venosus become
non-functional
Decreased pulmonary vascular resistance and increased pulmonary
blood flow
Increase in pressure of the left atrium, decrease pressure in right
atrium, causing closure of foramen ovale.
Pulmonary resistance is less than systematic resistance so there is leftto-right shunting resulting in closure of the ductus arteriosus.
II. Congestive Heart Failure:
Definition:
CHF is inability of the myocardium to circulate enough oxygenated blood to meet
the demands of the body. When the heart fails, cardiac output is diminished.
Heart rate, preload, contractitility, and afterload are affected. Peripheral tissue is
not adequately perfused. Congestion in lungs and periphery develops.
Etiology and Pathophysiology:
 Congenital defects – allow blood to flow from the left side of the heart to
the right so that extra blood is pumped to the pulmonary system rather
than through the aorta when the ventricle contracts.
 Obstructive congenital defects – restricts the flow of blood so the heart
hypertrophies to work harder to force blood through the narrowed
structures. The hypertrophied muscle becomes ineffective.
 Other defects which weaken the heart muscle.
Compensatory Mechanisms:
 Stimulation of the sympathetic nervous system which releases
norepinephrine from the adrenals. This stimulates blood vessels to
constrict and an increase in the heart rate.
 Tachycardia increases venous return to the heart which stretches the
myocardial fibers and increases preload. Only successful for short period of
time.
 Increased renin and ADH secretion caused by decrease renal
perfusion. Resultant increase in Na and H2O retention to increase
fluid to the heart and leading to edema.
Assessment:
Clinical Manifestations related to cause:
I. Pulmonary Congestion
 Infant tires easily especially with feedings
 Tachypnea, dyspnea, orthopnea
 Retractions with respiratory distress, grunting, nasal flaring
 Wheezing, rales, rhonchi
 Easy fatigue – especially during feedings, tires with play
 Restlessness, apprehension, irritable
* earliest signs are mild resting tachpnea, difficulty with feeding
II. Impaired Cardiac Output
 Tachycardia, diminished pulses
 Extremities cool, mottled, pallor
 Capillary refill > 2 seconds
 Diaphoretic / sweating
 Hypotension
III. Systemic venous congestion
 Hepatomegaly,
 Edema - appears first in face and eyes (periorbital); then peripheral
 Weight gain
IV. High metabolic rate
 FTH (failure to thrive)
 Slow weight gain
Goals of treatment:
 Improve cardiac function - increase contractility, make the heart work
more efficiently/ Reduce afterload
 Reduce volume overload, Remove accumulated fluid and Na


Decrease cardiac demands
Decrease oxygen consumption
Therapeutic Interventions and Nursing Care:
Improve Cardiac Function- increase contractility and reduce afterload
 Medication Therapy
a. Cardiac glycosides –improves ventricular contraction and decreases
heart rate and work load. Main drug given is Digoxin.
Nursing Care:
 Make sure have correct medication. Do not confuse with Digitoxin.
Usual type: Digoxin Elixir (0.05 mg/ml).
 Assess heart rate prior to administration.
Hold dose if: infant pulse < 100; child pulse is <80;
adolescent pulse is < 60
* If pulse is greater than 100 BPM – GIVE the medication.
 Two nurses check dosage
 Assess digoxin levels – normal is 1.0 – 2.0 mg./ml
 Parent teaching – see p. 1262
 Know signs of digitalis toxicity: (cardiac dysrrhythmia is first sign
in children. Bradycardia, anorexia, nausea and vomiting,
dizziness, weakness).
b. ACE inhibitors / afterload-reducing agents:
function by inhibiting conversion of angiotension I to II-results in
arterial relaxation. By relaxing the arterioles it reduces the
impedence to left ventricular ejection (afterload) and improve cardiac
output.
Capoten(Captoril)
Vasotec
 Nursing care
a. Promote rest
b. Maintain oxygen therapy and evaluate oxygen saturation
Remove Volume Overload / Accumulated Fluid & Na
 Medication Therapy
Diuretics - main stay of therapy. Diuretics enhance renal secretion
of sodium and water by reducing circulating blood volume and
decreasing preload.
o Furosemide (Lasix) – used for rapid diuresis
Lasix – give (1mg/kg) IM or IV,
check K+ level prior to IV administration because low K+ levels
lead to increase chance of developing toxicity, monitor
electrolytes, weigh daily, I&O
o Chlorothiazide (Diuril) – used for maintenance diuresis
o Zarozolyn (Thiazide type)
o Spironolactone (Aldactone)
 Diet Therapy - Fluid restriction may be required, but with caution to
prevent dehydration. Infants rarely require fluid restrict
 Nursing Care
a. Measure intake and output – weighing diapers
b. Observe for changes in peripheral edema and circulation
c. If ascites present – take serial abdominal measurements to
monitor changes.
d. Skin care
e. Turning schedule
Decrease Cardiac Demands
 Reduce metabolic needs
 Limit physical activity  Maintain body temperature
 Treat infections
 Minimize work of body
o Small frequent feedings
o If unable to consume an appropriate amount in 30 minutes of
feeding, stop feeding. NG tube feedings may be considered.
o Organize care
Improve Tissue Oxygenation
 All previous measures
 Supplemental cool humidified oxygen
Cardiac Disorders:
Cardiac disorders in children are divided into two major groups: congenital heart
disease and acquired heart disorders.
III. Congenital Heart Diseases
Main classifications of Congenital Heart Disease and Underlying Pathophysiology:
1. Defects that increase pulmonary blood flow
 Abnormal opening between cardiac chambers or greater arteries
 Increase volume overload on right side of heart
 Cardiac workload increases to compensate for volume overload
 Increased pulmonary blood flow
 Obstruction of blood flow from ventricles causes ventricular
hypertrophy, dilation
 Major problem is congestive heart failure
2. Defects with decrease blood flow and mixed defects
 Narrowing or constriction of an opening (valve or vessel) obstructing
blood flow
 Pressure rises in the area behind the obstruction
 Increase in cardiac workload
 Ventricular strain
 Obstruction on the left side of the heart can cause decrease perfusion to
the body
3. Defects obstructing systemic blood flow
 Obstruction on the right side of the heart decreasing the amount of
blood volume to the lungs
 Decreased oxygen saturation to the left side of the heart
 Cynanosis
Defects that increase pulmonary blood flow
1. Patent Ductus Arterious PDA:
Pathophysiology:
Failure of the fetal ductus arterious to close completely at birth. Stimuli for
closure include:
 Increased oxygen levels in the blood when the baby breaths normally
 Decreased prostaglandin levels
 Usually closes after birth
 Degenerates to a ligament
When the ductus fails to close  Oxygenated blood from the aorta returns through the PDA to the
pulmonary arteries to the lungs
 Left to right shunting
 Increased cardiac workload on left side of the heart
 Increased pulmonary blood flow – congestive heart failure
Signs and Symptoms:
 Continuous heart murmur – often the first indication of a congenital
heart defect.
 Signs of congestive heart failure
 Increase in respiratory infections
Therapeutic interventions
 Medication Therapy
a. Intravenous Indomethacin (Indocin) - a prostaglandin inhibitor, to
promote ductal constricture. 60% successful in 1st 14 days

Cardiac Catheterizaton / Transcatheter closure
a. Coil placed that promotes occlusion of the ductus arteriosus
b. Corrective measure that is less invasive than surgery
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 insertion,
Keep leg immobilized
Vital signs
Check for bleeding at insertion site, pressure dressing for first 24
hours.
Measure I&O – force fluids. Fluids increase elimination of the
contrast dye from the body.

Surgical Intervention
a. Open heart surgery / Surgical ligation of ductus via left thoracotomy
incision.
Pre-operative Nursing Care:
1. Extensively prepare the child and the parents for the experience demonstrate tubes and bandages and describe the scar the
operation leaves
2. Teach coughing and deep breathing to the child
3. Conduct the child and the parents on a tour of the intensive care
unit and introduce them to the staff
4. Observe the child for signs of infection
5. Make sure all laboratory tests are completed
Post-operative Nursing Care
1. Maintain adequate pulmonary function
o Keep patent airway
o Patient should deep breathe and cough. Monitor use of IPPB.
o Suction if necessary
o Oxygen - adequate to cells
o Chest suction for refilling lungs. Care of chest tubes
o Check rate and depth of respirations
o Check water-seal chest drainage
2. Maintain adequate circulatory functioning
o Check vital signs
o Replace blood where necessary
o Check intake and output every hour
3. Provide for rest through organized care
4. Establish adequate hydration and nutrition; fluid and electrolyte
balance
5. Take measures to prevent post-operative complications
o Turn patient frequently
o Skin care
o Check extremities for occlusions of major vessels with blood
clots: cyanosis, paleness of extremity, or coldness to the touch
o Passive range of motion
o Check dressing for signs of hemorrhage
6. Prepare for discharge
2. Atrial Septal Defect ASD:
Pathophysiology:
 Involves defects that occur during the development of the atrioventricular
canal with an opening located between the atriums.
o A Patent foramen ovale happens in 20 percent of all births, a slit-like
opening remains in the atrial septum. This defect is usually functional
murmur and requires no surgical intervention.
 Left to right shunting through the defect
 Enlarged right atrium
 Increased pulmonary blood flow – congestive heart failure
Signs and Symptoms:
 Frequently asymptomatic if there are no other abnormalities
 Signs of congestive heart failure may develop later in life
 Increased respiratory infections
Therapeutic Interventions and Nursing care:
 Medical management
a. Watched closely by cardiologist – assess for spontaneous
closure in first year of life.

Cardiac catheterization
a. Amplatzer Septal Occluder. Small round mesh device loaded into
the tip of a special catheter placed in heart via cardiac
catheterization. Once it is in place the device is deployed and
attaches to rim of tissue around defect. Tissue will eventually
cover the device and close the defect.

Surgical Intervention
a. Surgical closure with sutures or patch
3. Ventricular Septal Defects VSD:
Pathophysiology:
 Abnormal opening between the ventricles
 The majority of defects occur in the membranous septum of the ventricles,
and severity is related to the size of the defect and amount of pulmonary
blood flow.
 Left to right shunting. Blood flows from Left ventricle through defect to the
right ventricle and re-circulated via pulmonary artery to the lungs causing
increase in pulmonary congestion.
Signs and Symptoms:
 Depend on the severity
 Tachypneic, diaphoretic, fatigues easily, underweight for age, tires before
feeding completed.
 More severe have congestive heart failure
Therapeutic Interventions and Nursing care:
 Usually requires no treatment – closes first few years of life.
 Treat the congestive heart failure
 Surgical
a. Closure with sutures or patch via cardiopulmonary bypass
surgery
Defects with decrease blood flow and mixed defects
1. Pulmonic Stenosis
Pathophysiology:
 Pulmonic stenosis - Lesion that obstructs the flow of blood from the right
ventricle usually by narrowing of entrance to the pulmonary artery leads to
right ventricular hypertrophy
 Aortic - Lesion that obstructs the flow of blood from the left ventricle
usually by narrowing of entrance to the aorta which can lead to left
ventricle hypertrophy.
Signs and Symptoms
 Depend on severity
 Usually asymptomatic and have normal growth and development
 Can manifest EKG abnormalities with exertion
 Murmur
Therapeutic Interventions:
 Medications
Prostaglandins to keep the PDA open so some oxygenation can occur
 Cardiac catheterization
a. Baloon valvuloplasty – special catheter with balloon end is
passed through the heart into the defective valve and then
balloon dilates stenotic valve. Stents may be placed also.

Surgery
a. Valvotomy
2. Tetralogy of Fallot:
Pathophysiology:
Most common cyanotic heart defect in children over 2 years of age. Contains
these 4 anomalies:
 Ventricular septal defect
 Dextroposition of aorta so that it overrides the defect
 Hypertrophy of right ventricle
 Stenosis of the pulmonary artery

Hemodynamics - a right to left shunt arises in this anomaly due to the
position of the aorta and the hypertrophied right ventricle thus, partially
unoxygenated blood is sent back to the systemic circulation through the
VSD
Signs and Symptoms:
1.
Retarded growth and failure to thrive
2.
Lack of energy
3.
Frequent infections
4.
Polycythemia
5.
Clubbing of fingers
6.
Squatting
7.
Cerebral abscess
8.
Blood pressure
9.
Cardiomegaly
10. Cyanosis
Therapeutic Interventions and Nursing Care: .
 Surgical Interventions
1. Palliative - Blalock-Taussig or Potts procedure, which increases blood flow
to the lungs. Creates an artificial PDA.
2. Open-heart surgery for corrective treatment of pulmonary stenosis and vsd
3. Survival depends upon mixing of blood from pulmonic and systemic
circulation
3. Transposition of the great arteries:
The 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.
Nursing Diagnosis and Goals:
Alternation in cardiac output: decrease R/T heart malformation
Child will maintain an adequate cardiac output AEB:
1.
RR (state specific highest and lowest range)
2.
B/P (state specific highest and lowest range)
3.
clear equal breath sounds
4.
5.
6.
7.
8.
9.
10.
O2 sat as specified
Monitor weight inappropriate weight gain (<30G/day), absence of
puffy eye lids
skin warm to touch
capillary refill 2-3 seconds
adequate urinary output (state amount, .5-2ml/kg/hr) or 0 > 60% of I
Maintain S.G. 1.005-1.018
Absence of S & S of CHF as sited earlier
Therapeutic Interventions and Nursing Care:
 V.S.
 Maintain Temp to avoid excessive 02 consumption
 Assess for S&S as listed above
 I&O, output > 60% of intake
 Assess Specific gravity of urine
 Administer Cardiac drugs
 Notify Dr. if any deterioration
 Elevate HOB 30%
 Check labs
 Organize care to provide periods of rest; provide outlets like dolls, reading
 Give small frequent feedings
 Assess and record child/family knowledge of participation in care including:
meds, HOB elevated, rest periods, I&O, S&S of decreased cardiac output
4. Truncus arteriosus:
A single arterial trunk arises from both ventricles that supplies the systemic,
pulmonary, and coronary circulations. A VSD and a single, defective, valve also
exist. Entire systemic circulation supplied from common trunk
Defects Obstructing Systemic blood flow:
1. Coarctation of the Aorta
Pathophysiology:
 Coarctation applies to any constriction /narrowing of the lumen of the
aorta.
 Obstruction to left ventricular output impeding systemic blood flow
 Left ventricular hypertrophy
 Pressure in ventricle and great artery increased before obstruction decreased beyond obstruction
 Pulmonary edema
Signs and Symptoms
 Increased B/P in upper extremities
 Decreased B/P in lower extremities.
 Radial pulses, full bounding pulses.
 Femoral or popliteal pulse weak or absent
 Leg pains occur under exertion, weakness or tingling in lower legs
 Fatigue
 Headache, nose bleeds
Therapeutic Interventions
 Baloon dilation with possible stent placement of the coarctation
 Open heart surgery with surgical reconstruction of the aorta
.
2. Hypoplastic left heart:
May have various left-sided defects, including coarctation of the aorta, aortic valve
& mitral valve stenosis or artresia
IV. Acquired Cardiac Diseases
A. Rheumatic Fever:
Etiology and Pathophysiology:
A systemic inflammatory (collagen) disease of connective tissue that usually follows a
group A beta-hemolytic streptococcus infection. This disorder causes changes in the
entire heart (especially the valves), joints, brain, and skin tissues.
Assessment:
Clinical Manifestations and Diagnostic tests:
Jones Criteria – utilized for diagnosis. There is no single clinical pattern.
 Two major criteria, or one minor criteria are necessary for a positive diagnosis
 Major criteria:
o Carditis, valvulitis
o Polyarthritis – knees, ankles, hips, shoulders
o Chorea
o Erthyema marginatum
o Subcutaneous nodules


Minor criteria:
o Fever
o Arthralgia-large joints, usually knees, wrists, elbows
o Previous rheumatic fever or rheumatic heart disease
o Elevated erythrocyte sedimentation rate
o Positive C-reaction protein
o P-R interval prolonged
o Antistreptolysin O titer - elevated
Supporting Evidence
o Recent scarlet fever
o Positive throat culture for group A streptococci
o Increased strep antibodies
Therapeutic Interventions:
 Medication Therapy
a. Antibiotic therapy for at least 5 years after attack with no residual heart
disease such as Penicillin or erythromycin
b. ASA for joint symptoms and relieve pain
c. Protection through use of antibacterial prophylaxis - penicillin

Nursing Interventions
** It is important that nurses teach measures in prevention of Rheumatic
fever. Teach parents that if child has a strep infection – emphasize the
importance of giving entire course of antibiotics and do not discontinue
because child feels better.
Assess compliance to medication therapy
Bed rest
Prevention further infection
2. Subacute Bacterial Endocarditis / Ineffective Endocarditis:
Etiology and Pathophysiology:
An infectious disease which involves abnormal heart tissue, particularly rheumatic
lesions or congenital defects. Microorganisms grow on the endocardium, forming
vegetations, deposits of fibrin, and platelet thrombi. The lesion may invade
adjacent tissues such as aortic and mitral valves.
Assessment:
Clinical Manifestations: Insidious onset of symptoms
 Fever
 Lethargy
 General malaise
 Anorexia
 Splenomegaly
 Retinal hemorrhages
 Heart murmur – 90%
Diagnosis:
 Blood cultures
Therapeutic Interventions and nursing care:
 Medication Therapy
1.
Large dose of Antibiotic therapy – Penicillin, Amoxicillin,
Clindmycin
 Nursing Care
1.
Bed rest
2.
Teach to notify dentist prior to dental work.
C. Kawasaki Disease:
Etiology and Pathophysiology:
Also known as mucocutaneous lymph node syndrome, is an acute systemic
inflammatory illness. Multisystem vasculitis – inflammation of blood vessels in the
body especially the coronary arteries with antigen-antibody complexes. Does not
appear to be contagious, however there is often a preceding upper respiratory
tract infection. Cause is unknown, although it is associated with an immunologic
response to infectious agents.
 Complications include: CHF, aneurysms, coronary thrombosis
Assessment:
Clinical Manifestations: Three phases of the disease:
1. ACUTE PHASE - 10 to 14 days- characterized by:
 Rapid onset of fever that persists for more than 5 days (generally 38.3-39.9
C or 101-104F) and does not respond to antibiotic treatment.
 Bilateral conjunctivitis lasting 3-5 weeks
 Rash on day 5 of illness, usually macular spreading from extremities to trunk
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Cervical lymphadenopathy
Irritability, lethargy
Anorexia, possibly; diarrhea, hepatic dysfunction
Acute pericarditis
Hands and feet are edematous and red.
Red throat
2. SUBACUTE PHASE - 10-25 days - fever disappears and most symptoms resolve,
characterized by
 Continued irritability
 Anorexia, diarrhea
 Arthritis and arthralgia
 Lip cracking and peeling – strawberry tongue
 Desquamation of the extremities palms and soles
 Cervical lymphadenopathy with large nodes
 Possible coronary aneurysms, with potential for thrombosis formation.
3. CONVALESCENT PHASE - 25-60 days.
 Disease is self-limiting, the signs of illness decrease and disappear.
 Transverse lines called Beau’s line form on the nails
 Lasts until the erythrocyte sedimentation rate returns to normal and all S&S
of the illness disappear
Diagnostic tests:
 ECG, Echocardiogram
 CBC, WBC increased
 PT- elevated
 ESR- elevated
 SGOT, SGPT- both elevated RT hepatic dysfunction
 IgA, IgG, and IgM elevated
Therapeutic Interventions and Nursing Care:
 Medication Therapy – given to decrease aneurysm formation and fever
a. Aspirin therapy – 100 mg/kg/day in four doses until fever resolved,
then doses decrease for 6-8 weeks or until aneurysms gone.
b. Gamma globulin - High dose IV (IVGG)

Nursing Interventions
1. Monitor VS including B/P
2. Assess oral mucous membranes and lubricate the lips, and give
small frequent feedings of soft, bland foods and liquids.
3. Assess body rash and extremities and provide skin care
4. Give antipyretics as ordered
5. Monitor I & O
6. Decrease stimulation
7. Provide comfort measures
- cool compresses and tepid sponge baths
- passive ROM to facilitate joint movement

Discharge Teaching
1. Aspirin for anticoagulant therapy, interventions for elevated temp.
2. Skin care and comfort measures
3. F/U for ECGs, echocardiograms, CXR every 3 months for 1 year
4. Call Dr. if: chest pain, SOB, dizziness, change in behavior, not
eating or drinking, illness, or any other concerns.
Summary for all heart disorders:

General Principles that apply to all heart conditions:
1.
2.
3.
4.
Encourage normal growth and development
Counsel parents to avoid overprotection
Deal with parents’ concerns and anxieties
Educate parents about conditions, tests, planned treatments,
medications
5. Assist parents in developing ability to assess child’s physical status