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Focused Health History
Family history of defects / early cardiac disease / siblings with defects
Maternal history of stillborns or miscarriages Congenital anomalies / genetic anomalies /
fetal alcohol syndrome / Down Syndrome and Turner Syndrome
Maternal exposure to rubella
Focused Health History
Heart murmur Tires while eating Low weight for height Sweats while eating (diaphoretic) Cyanosis, worsens with feeding or activity
level Irritable weak cry
Focused Health History
In the older child additional symptoms may include: Chest pain Decreased activity level Syncope Slight of build
Focused Physical Assessment
General appearance Integumentary system Face, nose, and oral cavity Thorax and lung Cardiovascular system
Heart Murmurs
These sounds are produced by blood passing through a defective valve, great vessel, or other heart structure.
Murmurs are classified by: intensity, location, radiation, timing, and quality.
Pulses
Alert: Weaker pulses or lower blood pressure in the
lower extremities may indicate coarctation of the aorta (COA)
Bounding pulses can indicate a patent ductus arteriosus (PDA) or aortic insufficiency.
Vital Signs
Heart rate: tachycardia in the absence of fever, crying, or stress may indicate cardiac pathology.
Tachypnea, even with rest, chest retractions indicate respiratory distress, possibly resulting from congestive heart failure
Knee-chest Position
Child with a cyanotic heartdefect squats (assumes a knee-chest position) to relievecyanotic spells. Some times called “tet” spells. Ball & Bindler
Nurse puts infant in knee-chestposition. Whaley & Wong
First Breath
• Pulmonary alveoli open up• Pressure in pulmonary tissues decreases• Blood from the right heart rushes to fill the
alveolar capillaries• Pressure in right side of heart decreases• Pressure in left side of heart increases• Pressure increases in aorta
Diagnostic Test
Chest x-ray to define silhouette of the heart. Heart size, shape, pulmonary markings, and
cardiomegaly. Electrocardiogram ECG or EKG to define
electrical activity of the heart. Echo-cardiogram to visualize anatomic
structures.
Non-invasive
Cardiac Catheterization
An invasive test to diagnose or treat cardiac defects. Visualizes heart and vessels. Measures oxygen saturation of chambers. Measures intra-cardiac pressures. Determines muscle function and pumping action
of the heart.
Toxicity to Dye
Watch for signs of toxicity to the dye used during the procedure. Increased temperature Urticaria Wheezing Edema Dyspnea Headache
*Allergy response
Pre-cardiac Catheterization
Assess vital signs with blood pressure. Hemoglobin and hematocrit Pedal pulses NPO Hold digoxin IV if child is polycythemic
Post-cardiac Catheterization
Vital sign, with apical pulse, and blood pressure q 15 minutes for first hour.
Apical pulse for 1 minute to check for bradycardia or dysrhythmias.
Post-cardiac Catheterization
Assess pulses below the cath site. Record quality and symmetry of pulses. Assess temperature and color of affected
extremity. Check dressing for bleeding or hematoma
formation.
Home Care Instructions
Keep dressing in place for 24 hours. Keep site dry and clean. Observe site for redness, swelling, drainage,
or bleeding. Check temperature. Avoid strenuous exercise. Acetaminophen for pain. Keep follow-up appointment Pre-procedure medications as ordered.
Left to Right Shunt
Pressures on the left side of the heart are normally higher than the pressures in the right side of the heart. If there is an abnormal opening in the septum between the right and left sides, blood flows from left to the right.
Clinical Manifestations
The infant is not cyanotic.
Tachycardia due to pushing increased blood volume.
Cardiomegaly due to increased workload of the heart.
Clinical Manifestations
Dyspnea and pulmonary edema due to the lungs receiving blood under high pressure from the right ventricle.
Increased number of respiratory infections due to blood pooling in the the lungs promoting bacterial growth.
Right to Left Shunts
Occurs when pressure in the right side of the heart is greater than the left side of the heart. Resistance of the lungs in abnormally high Pulmonary artery is restricted
Deoxygenated blood from the right side shunts to the left side
Right to Left Shunt
Hole in septum + obstructive lesion =
Deoxygenated blood from the right side of the heart shunts to the left side of the heart and out into the body.
Clinical Manifestations
Hypoxemia = the result of decreased tissue oxygenation.
Polycythemia = increased red blood cell production due to the body’s attempt to compensate for the hypoxemia.
Increase viscosity of the blood = heart has to pump harder.
Potential Complications
Thrombus formation due to sluggish circulation.
Brain abscess or stroke due to the un-oxygenated blood bypassing the filtering system of the lungs.
Heart Failure
Major manifestation of cardiac disease
Under 1 year of age due to congenital anomaly
Over 1 year with no congenital anomaly may be due to acquired heart disease
Clinical Manifestations of HF
Systemic Venous Congestion Weight gain, hepatomegaly, edema, jugular vein
distension Pulmonary Venous Congestion
Tachypnea, dyspnea, cough, wheezes Compensatory Response
Tachycardia, cardiomegaly, diaphoretic, fatigue, failure to grow
Digoxin Therapy
Digoxin increases the force of the myocardial contraction. Take an apical pulse with a stethoscope for 1 full
minute before every dose of digoxin. If bradycardia is detected. < 100 beats / min for infant and toddler < 80 beats in the older child < 60 beats in the adolescent
* Call physician before administering the drug.
Signs of Digoxin Toxicity
Bradycardia Arrhythmia Nausea, vomiting, anorexia Dizziness, headache Weakness and fatigue
Interventions
Fluid restriction Diuretics – Lasix (potassium wasting) or
Aldactone (potassium sparing) Bed rest Oxygen Small frequent feedings – soft nipple with
supplemental NG for adequate calorie intake Pulse oximeter Sedatives if needed
Feeding
Small frequent feedings Soft nipple to easy energy needed to suck 24 calorie formula for added calories NG feed if not taking in adequate calories to
gain weight
Patent Ductus Arteriosus
PDA Incidence 10% One of the most common benign defects Ductus normally closes within hours of birth Connection between the pulmonary artery
(low pressure) and aorta (high pressure) High risk for pulmonary hypertension
Diagnosis and Treatment
Diagnosis by Chest x-ray – enlarged heart and dilated
pulmonary artery Echo-cardiogram – show the opening between
pulmonary artery and aorta
Treatment
Indomethocin given po – constricts the muscle in the wall of the PDA and promotes closure
Cardiac Catheterization – coil is placed in the open duct and acts like a plug
Closed heart surgery – small incision made between ribs on left hand side and PDA is ligated or tied and cut
Atrial Septal Defect
ASD 10% of defects Blood in left atrium flows into right atrium Pulmonary hypertension Reduced blood volume in systemic
circulation If left untreated may lead to pulmonary
hypertension, congestive heart failure or stroke as an adult.
Diagnosis and Treatment
Diagnosis: heart murmur may be heard in the pulmonary valve area because the heart is forcing an unusually large amount of blood through a normal sized valve.
Echocardiogram is the primary method used to diagnose the defect – it can show the hole and its size and any enlargement of the right atrium and ventricle in response to the extra work they are doing.
Treatment
Surgical closure of the atrial septal defect After closure in childhood the heart size will
return to normal over a period of four to six months.
No restrictions to physical activity post closure
Ventricular Septal Defect
VSD 30% of defects Opening in the ventricular septum Left-to-right shunt Right ventricular hypertrophy Deficient systemic blood flow
VSD
Small holes generally are asymptomatic Medium to moderate holes will cause
problems when the pressure in the right side of the heart decreases and blood will start to flow to the path of least resistance (from the left ventricle through the VSD to the right ventricle and into the lungs)
This will generally lead to CHF
Diagnosis and Treatment
Diagnosis – heart murmur – clinical pearl a louder murmur may indicate a smaller hole due to the force that is needed for the blood to get through the hole.
Electrocardiogram – to see if there is a strain on the heart
Chest x-ray – size of heart Echocardiogram – shows size of the hole and
size of heart chambers
Treatment VSD
CHF: diuretics of help get rid of extra fluid in the lungs
Digoxin if additional force needed to squeeze the heart
FTT or failure to grow may need higher calorie concentration
Will need prophylactic antibiotics before dental procedures if defect is not repaired
Surgical Repair
Over a period of years the vessels in the lungs will develop thicker walls – the pressure in the lungs will increase and pulmonary vascular disease
If pressure in the lungs becomes too high the un-oxygenated blood with cross over to the left side of the heart and un-oxygenated blood with enter the circulatory system.
If the large VSD is repaired these changes will not occur.
Coarctation of Aorta
COA 7 % of defects Congenital narrowing of the descending aorta 80% have aortic-valve anomalies Difference in BP in arms and legs (severe
obstruction)
Diagnosis and Treatment
In 50% the narrowing is not severe enough to cause symptoms in the first days of life.
When the PDA closes a higher resistance develops and heart failure can develop.
Pulses in the groin and leg will be diminished Echocardiogram will show the defect in the
aorta
Treatment
Prostaglandin may given to keep the PDA open to reduce the pressure changes
The most common repair is resection of the narrowed area with re-anastomosis of the two ends
Surgical complications – kidney damage due to clamping off of blood flow during surgery
High blood pressure post surgery – may need to be on antihypertensives
Antibiotic prophylactic need due to possible aortic valve abnormalities.
Tetralogy of Fallot (TOF)
6% of defects Most common cardiac malformation
responsible for cyanosis in a child over 1 year
TOF
Four Components VSD Pulmonary stenosis – narrowing of pulmonary
valve Overriding of the aorta – aortic valve is enlarged
and appears to arise from both the left and right ventricles instead of the left ventricle
Hypertrophy of right ventricle – thickening of the muscular walls because of the right ventricle pumping at high pressure
Clinical Manifestations
Dependent on degree of right ventricular outflow obstruction.
Right-to-left shunt Clubbing of digits “tet” spells - treated by flexing knees forward
and upward Severe irritability due to low oxygen levels
Diagnosis
Cyanosis Oxygen will have little effect on the cyanosis Loud heart murmur Echocardiogram – demonstrates the four
defects characteristic of tetralogy
Treatment
If oxygen levels are extremely low prostaglandins may be administered IV to keep the PDA open
Complete repair is done when the infant is about 6 months of age
Correction includes Closure of the VSD with dacron patch The narrowed pulmonary valve is enlarged Coronary arteries will be repaired Hypertrophy of right heart should remodel within a few
months when pressure in right side is reduced
Long Term Outcomes
Leaky pulmonary valve that can lead to pulmonary insufficiency
Arrhythmias after surgery Heart block – occasionally a pacemaker is
necessary Periodic echocardiogram and exercise stress
test or Holter evaluation
Aortic Stenosis
6% of defects Aortic valve: has two rather than three
leaflets. Leaflets are thickened or fused. Obstruction of blood flow from left ventricle Mild symptoms: dizziness, syncope, angina,
fatigue 30% incidence of sudden death
Aortic Stenosis
Causes obstruction to blood flow between the left ventricle and aorta.
Most common form is obstruction of the valve itself
When the aortic valve does not open properly the left ventricle must work harder to eject blood into the aorta.
Left ventricular muscle becomes hypertrophied.
Diagnosis
Heart murmur or AS is a turbulent noise caused by ejection of blood through the obstructed valve.
Electrocardiogram is usually normal Echocardiogram will show the obstruction
and rule out other heart anomalies Exercise stress test – provides information on
impact of the stenosis on heart function
Treatment
Cardiac catheterization – balloon dilation of the narrowed valve.
Surgical valvotomy if the closed procedure does not work – often done when patient is older when severe calcium deposits further obstruct the valve.
Recurrent valve obstruction is a complication and if valve replacement is done too early the child may outgrow the valve.
Antibiotic prophylaxis especially if valve replacement
Hypoplastic Left Heart
One of the most complex defects seen in the newborn and the most challenging of all the congenital defects
All the structures on the left side of the heart are severely underdeveloped.
Mitral and aortic valves are either completely closed or are very small – left ventricle is tiny – aorta is small and often only a few millimeters in diameter
HLH
Life threatening shock develops when the ductus arteriosis closes
Low oxygen saturations – will not increase with oxygen administration
Pulses will be weak in all extremities Plan to deliver infant in a hospital capable of
providing the aggressive treatment needed
Treatment
Three staged procedure to reconfigure the cardiovascular system Norwood – right ventricle becomes the systemic
ventricle pumping blood to the body Glenn done at 3-6 months Fontan done at 2 -3 years of age
Long Term Complications
Easily tiring when participating in sports or other exercises
Formation of blood clots – heparin or Coumadin use
Heart arrhythmias – pace maker Cardiac failure
Bacterial Endocarditis
Infection of endocardial surface of the heart History of CHD, Kawasaki Disease,
Rheumatic Fever, or prosthetic valves are more susceptible to infection
Prophylactic antibiotics with dental care, throat, intestinal, urinary or vaginal infections or surgery.
Kawasaki Disease
Acute-self limiting disease Generalized vasculitis Peak incidence 6 months to 2 years More common in males and Japanese
http://www.aafp.org/afp/990600ap/3093.html
Clinical Manifestations
High fever Conjunctivitis – no drainage Strawberry tongue Edema of hands and feed Reddening of palms and soles Lymph node swelling