1102 Test 4 Child With Cardiovascular

Front 1

Acyanotic Heart Disease-

Back 1

a broad term for any congenital heart defect in which all of the blood returning to the right side of the heart passes through the lungs and pulmonary vasculature in the normal fashion. The common forms of acyanotic congenital heart defects are those where there is a defect in one of the walls separating the chambers of the heart, or obstruction to one valve or artery.

The main types of acyanotic heard disease are: Patent Ductus Arteriosus (PDA), Coarctation of the Aorta, Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD), Atrioventricular Septal Defect (AVSD), Aortic Stenosis (AS), Pulmonary Stenosis (PS), Tetralogy of Fallot, Transposition of the Great Vessels (TGA), Total Anomalous Pulmonary Venous Return (TAPVR), Truncus Arteriosus, Tricuspid Atresia, and Hypoplastic Left Heart Syndrome (HLHS).

Front 2

Afterload:

Back 2

Afterload is what comes after ventricular contraction or the resistance against which the heart must pump blood. Afterload is determined by two conditions; the blood volume ejected from the ventricle and the compliance of the vascular space into which the blood is ejected. Think of afterload as a hose nozzle. If the hose nozzle is wide open, afterload is decreased due to decreased compliance. If the hose nozzle is almost closed, afterload will increase because the water has so much resistance to push against. Now, if you increase or decrease the amount of water that comes from the nozzle, there will be a further effect on the afterload. Think of afterload as blood pressure. Increased blood pressure is increased afterload, while decreased blood pressure is decreased afterload.

Front 3

Balloon angioplasty:

Back 3

was introduced in the late 1970's. PTCA is a non-surgical procedure that relieves narrowing and obstruction of the arteries to the muscle of the heart (coronary arteries). This allows more blood and oxygen to be delivered to the heart muscle. PTCA, is now referred to as percutaneous coronary intervention, or PCI, as this term includes the use of balloons, stents, and atherectomy devices.

Front 4

Cardiac catheterization

Back 4

is a medical procedure used to diagnose and treat certain heart conditions.

A long, thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck and threaded to your heart. Through the catheter, doctors can do diagnostic tests and treatments on your heart.

For example, your doctor may put a special dye in the catheter. This dye will flow through your bloodstream to your heart. Once the dye reaches your heart, it will make the inside of your coronary (heart) arteries show up on an x ray. This test is called coronary angiography (an-jee-OG-ra-fee).

Front 5

Contractility

Back 5

s the intrinsic ability of the heart to contract independent of preload and afterload. Changes in the ability to produce force during contraction result from different degrees of binding between myosin (thick) and actin (thin) filaments. The degree of binding that occurs depends on concentration of calcium ions in the cell; in an intact heart, it is usually the action of the sympathetic nervous system (through catecholamines) that determines the concentration of calcium ions in the cytosol of cardiac muscle cells. All factors that cause an increase in contractility work by causing an increase in intracellular [Ca++] during contraction

Front 6

Cyanosis:

Back 6

is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen.

Front 7

Cyanotic heart disease:

Back 7

is a heart defect, present at birth (congenital), that results in low blood oxygen levels. There may be more than one defect.
Causes, incidence, and risk factors

Normally, blood returns from the body and flows through the heart and lungs. It will then leave the heart with enough oxygen to supply the body's tissues.

Heart defects can change the way blood flows through the heart and lungs. This abnormal blood flow (called right-to-left shunt) can result in too little oxygen in the blood moving through the rest of the body.

Front 8

Diastole:

Back 8

s the period of time when the heart fills with blood after systole (contraction). Ventricular diastole is the period during which the ventricles are relaxing, while atrial diastole is the period during which the atria are relaxing.

Front 9

Echocardiography:

Back 9

often referred to in the medical community as a cardiac ECHO or simply an ECHO, is a sonogram of the heart (it is not abbreviated as ECG, which in medicine usually refers to an electrocardiogram)

Front 10

Electrocardiography:

Back 10

is a transthoracic interpretation of the electrical activity of the heart over time captured and externally recorded by skin electrodes.[1] It is a noninvasive recording produced by an electrocardiographic device. The etymology of the word is derived from the Greek electro, because it is related to electrical activity, cardio, Greek for heart, and graph, a Greek root meaning "to write". In English speaking countries, medical professionals often write EKG (the abbreviation for the German word elektrokardiogramm) in order to avoid confusion with EEG.[citation needed]

The ECG works mostly by detecting and amplifying the tiny electrical changes on the skin that are caused when the heart muscle "depolarizes" during each heart beat. At rest, each heart muscle cell has a charge across its outer wall, or cell membrane. Reducing this charge towards zero is called de-polarization, which activates the mechanisms in the cell that cause it to contract. During each heartbeat a healthy heart will have an orderly progression of a wave of depolarisation that is triggered by the cells in the sinoatrial node, spreads out through the atrium, passes through "intrinsic conduction pathways" and then spreads all over the ventricles. This is detected as tiny rises and falls in the voltage between two electrodes placed either side of the heart which is displayed as a wavy line either on a screen or on paper. This display indicates the overall rhythm of the heart and weaknesses in different parts of the heart muscle.

Front 11

Fluoroscopy:

Back 11

is an imaging technique commonly used by physicians to obtain real-time moving images of the internal structures of a patient through the use of a fluoroscope. In its simplest form, a fluoroscope consists of an X-ray source and fluorescent screen between which a patient is placed. However, modern fluoroscopes couple the screen to an X-ray image intensifier and CCD video camera allowing the images to be recorded and played on a monitor.

Front 12

Heart failure:

Back 12

is generally defined as inability of the heart to supply sufficient blood flow to meet the body's needs.[1][2][3] It has various diagnostic criteria, and the term heart failure is often incorrectly used to describe other cardiac-related illnesses, such as myocardial infarction (heart attack) or cardiac arrest

Common causes of heart failure include myocardial infarction and other forms of ischemic heart disease, hypertension, valvular heart disease, and cardiomyopathy.

Front 13

Innocent heart murmur:

Back 13

This diagnosis means the murmur is produced by a normal, healthy heart. It can come and go throughout childhood. It usually goes away on its own as the child gets older and doesn't pose any health threat

Front 14

Left-to-right shunt:

Back 14

Left to right shunts are characterized by a "back-leak" of blood from the systemic to the pulmonary circulation. As a consequence, the pulmonary circulation carries not only the blood that legitimately entered the right atrium and ventricle through the superior and inferior vena cava, but also the additional blood entering through an ASD, VSD, or a PDA. Blood volume and pressure in the pulmonary circulation become abnormally high. If the shunt is significant, there is progressive damage to the pulmonary vasculature and gradual development of irreversible pulmonary hypertension. The pressure in the pulmonary circuit may ultimately exceed the systemic pressure with reversal of blood flow from the right side of the circulation to the left (Eisenmenger syndrome).

Front 15

Organic heart murmur:

Back 15

Organic murmurs are produced by heart problems and thus indicate a lesion.

Front 16

Phonocardiogram:

Back 16

s a plot of high fidelity recording of the sounds and murmurs made by the heart with the help of the machine called phonocardiograph, or "Recording of the sounds made by the heart during a cardiac cycle." The sounds are thought to result from vibrations created by closure of the heart valves.

Front 17

Polycythemia:

Back 17

is a disease state in which the proportion of blood volume that is occupied by red blood cells increases. Blood volume proportions can be measured as hematocrit level

Front 18

Postcardiac surgery syndrome:

Back 18

Dressler’s Syndrome occurs in 5%-6% of patients with acute myocardial infarction4. It
characteristically presents with fever, malaise and pleuropericardial chest pain, the onset of which
is 2-3 weeks after the acute event7. About 28% develop pleural or pericardial effusion2,6. The
acute event can also be anything causing inflammation or penetration of the pericardium, cardiac
operation, a stab wound, a non-penetrating blow to the chest, or perforation of the heart with a
heart catheter, or post-operative cytomegalovirus (CMV) infection. In these cases it is called
post-pericardotomy5 or post cardiac injury syndrome1.

Front 19

Postperfusion syndrome

Back 19

A condition seen in 2% of Pts who undergo cardiac surgery, 3-7 wks after cardiopulmonary bypass, resembles infectious mononucleosis or hepatitis, and is attributed to viruses transfused with
the blood during surgery Clinical Fever, splenomegaly, lymphadenopathy, maculopapular rash, anemia, atypical lymphocytes Prognosis Benign; resolves spontaneously without therapy. See Post-perfusion lung. Cf Post-resuscitation syndrome.

Front 20

Preload:

Back 20

is the amount of blood in the heart (again usually the left ventricle is the reference point) that is available to be pumped out - think of the heart as a primed pump and the preload is the amount of fluid that the pump is primed with.

Front 21

Afterload:

Back 21

is essentially referring to the diameter of the vasculature that the heart has to pump the blood through - think of a straw and a section of hose pipe. If you have a mouth full of water and blow it through a section of hose pipe, it doesn't take much effort. It takes more effort to blow a mouth full of water through the straw. If course, you can generate more water pressure through the narrower straw than you can through the wide section of hose pipe. It is the same with the heart. If our blood vessels are constricted, then the LV has to work harder to pump out the blood.

Front 22

IF the left side of the heart is failing:

Back 22

then pressure backs up into the pulmonary system.

Front 23

Frank-Starling law says:

Back 23

that the greater the preload the greater the contractility of the heart.....up to a point. You can stretch the cardiac fibers too much and you then can't get a good pressure anymore - no more bang for your buck. For this think of a new vs a used rubber band. After a rubber band has been stretched and used for a while, it never returns to its original shape, it is all stretched out. That can happen to the heart as well. If the heart has to work too hard because it is too full, then it becomes overstretched and fails.

heart cath can measure ejection fraction. (the quality of heart beat). Heart fibers are comparable to a balloon filled to full doesn’t pop as well as balloon filled just right. Cardiomyopathy Cardiomegaly

Front 24

EF should be around:

Back 24

60%

Front 25

A person with CHF will have an EF of around

Back 25

20-25%

Front 26

A person doesn't get on the heart transplant list until the EF gets to around:

Back 26

10%.

Front 27

EF:

Back 27

ejection fraction

Front 28

The child with a cyanotic heart defect must have enough

Back 28

fluid in the vasculature to help prevent clots. However, if the child has too much fluid, he/she can go into heart failure.

Front 29

If you have a left-to-right shunt:

Back 29

you have oxygenated blood going from the left side of the heart over to the right side of the heart. Whatever blood is leaving the left side of the heart for the body is oxygenated, so the body doesn't get into trouble unless the blood pressure is too low. However, the flow of blood is increased to the lungs. That makes the right side of the heart have to work harder. That result in right-sided heart failure and right-sided hypertrophy (either RV, RA or both depending on the defect and amount of time the right side of the heart is having to deal with the increased volume).

Front 30

In a right-to-left shunt:

Back 30

some unoxygenated blood is going over to the left side of the heart and out to the body instead of going to the lungs to be oxygenated. This results in decreased oxygenated blood going out to the body. Depending on how much deoxygenated blood is going out, you will see drops in O2 saturation and/or cyanosis. Nurses and parents need to know what to do in the event of cyanotic episodes. Obviously, if blood is shunting from the right to the left, the defect has to be severe enough to cause the pressure to increase on the right side of the heart (remember, blood will go from the high pressure side to the low pressure side).

Front 31

Indomethacin decreases levels of:

Back 31

PGE1 and stimulates closure of the ductus arteriosus. If you have a patient who has a patent ductus arteriosus, the patient will be given indomethacin to close the ductus.

Front 32

PGE1 is given to:

Back 32

keep the ductus open so that we can get increased blood to the lungs for oxygenation

Front 33

Systole:

Back 33

is a phase of contraction during the cardiac cycle. Used alone, the term usually describes the phase of left ventricle contraction.[1] During systole, the myocardium contracts in a coordinated manner in response to a complex endogenous autonomic physiologic electrical stimulus. Systole results in positive fluid pressure generated within the chambers of the heart, driving blood flow out of the heart and into the body and lungs.

Front 34

Vasculitis:

Back 34

is an inflammation of the blood vessels. It happens when the body's immune system attacks the blood vessel by mistake. The cause is often unknown. Vasculitis can affect arteries, veins and capillaries. Arteries are vessels that carry blood from the heart to the body's organs. Veins are the vessels that carry blood back to the heart. Capillaries are tiny blood vessels that connect the small arteries and veins.

Front 35

CO =

Back 35

SV x HR

Front 36

CO is affected by:

Back 36

preload, afterload and contractility

Front 37

System Edema is first noticed as:

Back 37

Periorbital edema

Front 38

If preload is too high give:

Back 38

lasix

Front 39

To raise blood pressure give:

Back 39

dopamine

(but at the same time extra work)

Front 40

To increase contractility:

Back 40

give digoxin

Parents must know how to take pulse

Front 41

To induce vasodilation give:

Back 41

morpheine

Front 42

Health Promotion and Risk Management

Congenital -

Acquired -

Back 42

Women of child-bearing age should be immunized against rubella and varicella. Parents with family history of congenital defects need to have children screened

Children with hypertension, hyperlipidemia and rheumatic fever are at increased risk.

Rheumatic fever follows a group A beta-hemolytic streptococcus infection (otitis media, streptococcal pharyngitis and impetigo should have antibiotic therapy)
Avoid excessive salt, lack of exercise, obesity, diets high in saturated fats (reduce to 30% of calories by school age)

Front 43

Congestive Heart Failure:

Back 43

Usually occurs as a result of a congenital heart disorder or a disease such as rheumatic fever, Kawasaki’s disease or infectious endocarditis.
The heart can’t pump enough blood to supply oxygen and nutrients to the body
The body compensates for a while. Remember CO = SV x HR and the Frank-Starling principle. For children less than 5 y.o., increase in CO is mostly accomplished through increased HR
As renal blood flow decreases, GFR slows allowing retention of sodium and fluid. When the body senses decreased supply of oxygen, aldosterone is secreted which further promotes retention of sodium in an attempt to increase blood flow to the kidneys. ADH secretion is also increased to help retain fluid

Body compensates by increase red blood manufacture
Higher Hematotatic and Hemoglobin-blood is thick increased risk of clots. Blood clots form in the brain, lungs, extremities

Front 44

Symptoms Ride Sided Failure:

Back 44

back up of pressure into the portal system and venous system, hepatomegaly, abdominal pain, ascities (excess fluid in the space between the tissues lining the abdomen and abdominal organs (the peritoneal cavity).

Mosst common cause of right heart left failure is left side heart failure.

Front 45

Symptoms Left Sided Failure:

Back 45

- back up of pressure in pulmonary system, dyspnea, rales, bloody sputum on coughing, cyanosis
Left sided failure ultimately leads to right sided failure
Edema is a late sign for children. If present, it shows up as periorbital edema
In infants, signs can be breathlessness from rapid respirations, tiring easily and poor feeding related to exhaustion and dyspnea, may become diaphoretic when feeding, abrupt weight gain is the most obvious indication
Apical heart beat may be displaced laterally and downward. May have a third heart sound.

Front 46

Murmor is caused by:

Back 46

turbulent blood flow.

Murmor graded 1-6
4-5-6 are Thrill

Front 47

Heart Failure

Confirmed by:

Back 47

Confirmed by echocardiogram, chest x-ray and ECG

Front 48

Heart Failure

Treatment is aimed at:

Back 48

1) improving cardiac function, 2) removing accumulated fluid and sodium, 3)decrease cardiac demands, 4) improve tissue O2

Front 49

Heart Failure

Diuretics -

Back 49

Lasix, spironalactone, thiazides (may need K replacement)

Front 50

Heart Failure

Improve contractility -

Back 50

Digoxin (given a loading dose and then a maintenance dose, dig level should be 0.8 to 2.0 u/L, toxicity includes, nausea, vomiting, anorexia, slow heart rate, family teaching required, hold dose if HR < 100 for infants and toddlers, <80 for older children or <60 for adolescents)

Front 51

Heart Failure

Decrease afterload -

Back 51

hydralazine (vasodilator), nifedipine (Ca channel blocker), nipride (vasodilator), or captopril (ACE inhibitor)

Front 52

Diuretic affects-

Back 52

preload

Front 53

Decrease oxygen demands -

(treatment)

Back 53

Provide rest periods and uninterrupted sleep, place in Semi-Fowler’s position, space out activities/procedures, preserve body temperature, treat any infections

Front 54

Improve oxygenation -

Back 54

administer O2 as needed by hood, mask or nasal prongs. If prongs are used, check nostrils q 4hrs
Adequate nutrition is also important - may need six to eight small meals daily rather than 3 large meals, may need tube feedings

Front 55

Cardiovascular Disorders and the Nursing Process:

Back 55

Careful history taking and physical examination are most important
Common procedures for diagnosis are echocardiogram and cardiac catheterization

Front 56

Common nursing diagnoses:

Back 56

Decreased cardiac output related to congenital structural defect

Altered tissue perfusion related to inadequate cardiac output

Rubella, immmunizations.

Knowledge deficit related to care of the child pre- and postoperatively
Fear related to lack of knowledge about child’s disease

Altered family processes related to stresses of the diagnosis and care responsibilities

Ineffective individual or family coping related to lack of adequate support

Altered parenting related to inability to bond with critically ill newborn

Cardiac catheterization-cant get all the way to left but can get measurements from right.

Front 57

Important interventions include:

Back 57

teaching, providing opportunities to express fears, providing psychological support

Other interventions include comfort measures, oxygen administration, managing cardiac failure, pre-and postop care

Front 58

History Taking:

Back 58

Must be careful. Some of the symptoms are very subtle.
Some symptoms do not show up right after birth
Typical presentations of infants are tachycardia, tachypnea, and poor feeding
Older children may present with fatigue and frequent lower respiratory infections. Some children may perspire excessively
Edema is a late sign and usually presents first as periorbital edema.
May complain of decreased UOP
May have headaches, nose bleeds, high blood pressure in upper
extremities

Obtain a detailed prenatal history including exposure to infections (cytomegalovirus, toxoplasmosis, rubella or varicella), medication usage, drug and alcohol use, nutrition, exposure to radiation
Determine if there is any family history of congenital heart defects or heart disease, cognitive impairments, renal disease

Front 59

Coarctation-

Back 59

high blood pressure, (constriction of aorta) occurs in arch of Aorta. If pressure is backing up at constricted area this creates higher blood pressure in higher extremities. Headache, nose bleeds may be symptoms.

Front 60

Physical Assessment:

Assess:

Back 60

capillary refill presence of clubbing
cyanosis (pulmonary source vs. cardiac source)
ruddy complexion lethargy
rapid respirations tachycardia
abnormal body posture presence of a murmur

Front 61

Innocent murmurs vs. Organic murmurs
:

Back 61

With all murmurs, document position in cardiac cycle, duration, quality, pitch, intensity, location, whether there is a thrill and whether the murmur changes with position change

Organic murmurs are either systolic or diastolic, long, harsh or blowing, loud, constant and heard not matter what position the child is in

Clubbing-cyanosis over time
Murmur-grade, where, systolic-lush dub, diastolic,s2
Innocent murmur/organic murmur-some kids outgrow nothing wrong. Some wait to go away. No pathological reason
Foramen ovale-flap system
Ductus arteriosis-
EKG-takes electrical activity but not mechanical

Front 62

Diagnostic Tests:

ECG -

Back 62

- indicates HR, rhythm, presence or absence of hypertrophy, ischemia or necrosis, abnormalities in the conduction system, presence of electrolyte imbalances

Front 63

Diagnostic Tests:

Chest x-ray -

Back 63

shows heart size and shape, presence of CHF, prominence of pulmonary blood flow

Front 64

Diagnostic Tests:

Fluoroscopy:

Back 64

can be used to visualize the chambers of the heart, the great vessels, lungs, thoracic cage and diaphragm. Sometimes radioactive dye in injected. Sometimes contrast dye in used in conjunction with a cardiac cath

Front 65

Diagnostic Tests:

EKG:

Back 65

takes electrical activity but not mechanical

Front 66

Diagnositc Tests:

Echocardiogram -

Back 66

primary diagnostic test. Looks at the movement and dimensions of the cardiac structures using high-frequency sound waves

Front 67

Diagnostic Tests:

Phonocardiogram -

Back 67

heart sounds are recorded and displayed as a diagram

Front 68

Diagnostic Tests

MRI -

Back 68

used to evaluate heart structure, size or blood flow

Front 69

Diagnostic Tests

Treadmill:

Back 69

studies response to exercise

Front 70

Diagnostic Tests:

Lab tests -

Back 70

Hgb and Hct (polycythemia), ESR (rheumatic fever, Kawasaki disease, myocarditis), ABG (presence of a right to left shunt), O2 Sat, clotting times (PT, PTT) and platelet count, Na, K, dig level

Front 71

L to r:

Cyanotic or Acyanotic?

Back 71

Acyanotic

Front 72

R to L

Acyanotic or Cyanotic?

Back 72

Cyanotic

Front 73

Cardiac Catheterization

Can be either diagnostic or interventional

Back 73

Pressures in the heart can be measured
CO can be evaluated
Blood samples can be obtained and tested (O2 sat)
Electrical activity can be studied
Contrast can be injected to study blood flow, vessels and chambers
Balloon angioplasty can be performed to stretch stenosed areas or blockages in vessels

Pre-procedure - Patient teaching
How the test will be done, what to expect during the test, that afterward the child will have to lie flat and will have a bulky dressing over the catheter insertion site

Front 74

Cardiac Catheterization

Post-Procedure:

Back 74

Will have to lie flat several hours
VS every 15 minutes for the first few hours
Check site every 15 minutes for integrity of dressing, hematoma, redness, swelling
Check pulses distal to site. Also check extremity for capillary refill and warmth
Avoid dehydration – IVFs until taking PO
Avoid hypothermia
Check site daily for signs of infection
Avoid tub baths for 2-3 days and strenuous exercise for up to a week

Front 75

Important Points about Care of the Child with a Heart Defect

Back 75

Parents taking a child with a heart defect home should have discharge planning which includes name/number of a primary care provider, emergency numbers, home health visits, instruction in what to do if child becomes cyanotic, CPR training,
Should not allow a child with a cyanotic heart defect or severe aortic stenosis to cry for extended periods of time
Don’t usually have to restrict sodium intake (regulates water balance)
Need vitamin supplements and maybe an iron supplement
May need small feedings every 3-4 hours. May require feeding tubes or high calorie formulas/supplements.

Children usually limit their own activities, but parents should watch for respiratory distress when new activities are introduced
Children should receive their immunizations timely
Even minor illnesses should be treated promptly
Avoid dehydration
Receive prophylactic antibiotics prior to dental visits or oral surgery (Penicillin or Erythromycin)

Front 76

Congenital Heart Disease

Back 76

Occurs in about 6% of live births. Higher in pre-term infants or in infants who have a parent with aortic stenosis, ASD, VSD, or pulmonic stenosis
Children born with genetic defects have an increased risk of congenital heart defects (50%) as well as children with fetal alcohol syndrome (25-30%) and children born to diabetic mothers (3-5%)

Front 77

Congenital Heart Disease

Females are more likely to have:

Back 77

more prone to have PDA and ASD

Front 78

Congenital Heart Disease

Males are prone to have:

Back 78

more prone to have valvular aortic stenosis, coarctation of the aorta, TOF and transposition of the great vessels

The usual cause is failure of the heart to develop beyond an early stage of embryonic development

Front 79

Congenital Heart Disease

Maternal rubella is associated with:

Back 79

PDA, stenosis, ASD, VSD

Front 80

Acyanotic

Left to right Shunts:

Ventricular Septal Defect: (most common)

Back 80

opening in the septum between the two ventricles
results in right ventricular hypertrophy and increased pressure on the pulmonary artery
May not be evident at birth because high pulmonary resistance from incomplete opening of alveoli keeps the blood from coming across to the right ventricle
S&S (by age 4 to 8 weeks) - develops a loud, harsh, systolic murmur along the left sternal border 3rd or 4th ICS, widely transmitted, usually with a thrill
Diagnosed with echocardiography or MRI
RV hypertrophy may also be seen on ECG
20-60% close spontaneously - otherwise at risk of infectious endocarditis and cardiac failure
May require a Silastic or Dacron patch to close opening if edges can’t be approximated and sutured
Arrhythmias can be a complication post-op
Systolic murmur in ventricles between mitral triscupid
3rd and 4th is where you hear it the loudest.

Temporary arythmeas after surgery which may go away.
EP studies look at conduction system.

Front 81

Acyanotic

Left to right Shunts:

Atrial Septal Defect (ASD):

Back 81

Abnormal opening between the two atria
More frequent in girls than boys
Harsh systolic murmur over 2nd or 3rd ICS, fixed splitting of S2
Echo will show enlarged right side and increased pulmonary circulation
Cath will reveal separation in atrial septum and increased O2 sat in RA
At risk for infectious endocarditis and heart failure
Can cause emboli in pregnant females if not corrected
May require a patch if defect not able to be closed
May have arrhythmias post-op

Front 82

Acyanotic

Left to right Shunts:

Atrioventricular Canal (AVC) Defect:

Back 82

An incomplete fusion of the endocardial cushion. May be seen as a low ASD continuous with a high VSD. Mitral and tricuspid valves are usually distorted. Seen in 1 out of 9 children with Down syndrome
Blood flow is usually left to right, but may flow between all four chambers
Same symptoms of heart failure as ASD and VSD
Requires surgical repair and possible double valve replacement
Must be monitored post-op for jaundice from RBC destruction.
Increase release of bilirubin appears Jaundice

Front 83

Acyanotic

Left to right Shunts:

Patent Ductus Arteriosus (PDA)

Back 83

The ductus arteriosus between the pulmonary artery and the aorta fails to close at birth
May not close until 3 months causing blood to be shunted from the aorta to the pulmonary artery
Usually hear a continuous (systolic and diastolic) murmur at the upper left sternal border or under the clavicle of older children
ECG is usually normal, may show ventricular enlargement if the shunt is large
Cath is not usually needed unless to rule out other defects
Hear murmur for a while faint murmur. Heard when open stay
Endomethicin will take care of it.
May be given indomethacin, a prostaglandin inhibitor, to lower PGE1 levels and stimulate closure. Drug may be repeated as many as three times 12 to 24 hours apart
If medical management fails -
ligation of defect
visual assisted thoracoscopy (VAT) - a clip is placed on the ductus
If no surgery, child is a risk for heart failure related to the increased shunting, bacterial endocarditis from recirculating blood and potential stasis in the PA

Front 84

Cyanotic

Right to Left Shunts:

Defects Causing Decreased Pulmonary Flow

Pulmonic Stenosis -

Back 84

May be asymptomatic or have mild heart failure
Usually a grade IV or V systolic murmur with a thrill. Heard loudest at the upper left sternal border. May have a split S2
ECG may show right ventricular hypertrophy. Cath can demonstrate degree of stenosis.
PGE1-keep ductus arteriosis and foramen ovale open. Avoiding pulmonic valve

Treatment depends on severity of stenosis and the child’s age
If severe, the pressure may reopen the foramen ovale allowing flow from right to left causing cyanosis
If severe, then given PGE1 to keep ductus arteriosus from closing so that the infant can get more blood oxygenated
Balloon angioplasty may be tried to break valve adhesions and relieve the stenosis
If there is a lesser degree of stenosis, the child can be allowed to wait until they are 4 or 5 years old so that there is less surgical risk


Heart failure will be on right side.

Heard best at 2nd intercostal space pulmonic




Involve an obstruction of pulmonary blood flow which increases pressure in the right side of the heart.
If an ASD and or VSD also exists, then deoxygenated blood shunts from the right side to the left side
Narrowing of a vessel or valve. Results in high pressure before the obstruction and lower after the obstruction. Prevents sufficient blood supply from reaching its intended site

Front 85

Cyanotic

Right to Left Shunts:

Defects Causing Decreased Pulmonary Flow

Tricuspid Atresia:

Back 85

Tricuspid valve is completely closed.
Blood can’t flow from RA to RV so goes through the patent foramen ovale to the LA.
Oxygenation occurs by blood being shunted through a patent ductus to the lungs
If the foramen ovale and ductus arteriosus close, the patient becomes profoundly cyanotic, tachycardic and dyspneic.
Kept on PGE1 until surgery
Surgery consists of creating a subclavian-pulmonary artery shunt or restructuring the right side of the heart with a baffle (Fontan procedure)

Front 86

Cyanotic

Right to Left Shunts:


Tetrology of Fallot

Back 86

Accounts for 10% of congenital defects.
Consists of 4 anomalies
Pulmonary stenosis
VSD (usually large)
Dextraposition (overriding) of the aorta
Hypertrophy of the RV (acquired from the increased pressure in the RV from trying to push blood through the stenosed pulmonary artery)
“Blue baby” although may not be dramatically cyanotic immediately after birth
Exhibit poor physical growth, clubbing, systolic murmur, hypoxic spells, polycythemia, activity intolerance and squatting.
Systolic murmur
Clubbing
Polycythemia-more red blood cells.

X-ray shows cardiomegaly. Echo and ECG show the enlarged RV. Echo also shows decreased size of the PA and reduced flow through the lungs. Extent of the defect is determined through cardiac cath. Lab values show increased RBC’s and reduced O2 sat
Usually wait until child is 1 or 2 years old. Some institutions perform surgery earlier to prevent hypoxic episodes
If having hypoxic episode, place infant in knee-to-chest position. May require Morphine to dilate vessels or propanolol to reduce heart spasm

X-ray shows cardiomegaly. Echo and ECG show the enlarged RV. Echo also shows decreased size of the PA and reduced flow through the lungs. Extent of the defect is determined through cardiac cath. Lab values show increased RBC’s and reduced O2 sat
Usually wait until child is 1 or 2 years old. Some institutions perform surgery earlier to prevent hypoxic episodes
If having hypoxic episode, place infant in knee-to-chest position. May require Morphine to dilate vessels or propanolol to reduce heart spasm
Can have a palliative repair in which the subclavian artery is used to create an artificial ductus arteriosus (Blalock-Taussig procedure) to allow blood to flow from the aorta to the lungs
After the Blalock-Taussig, the child will not have a palpable pulse in the right arm. The right arm should not be used for BP’s or blood sticks
Full repair includes relief of pulmonary stenosis, VSD repair and correction of the overriding aorta

Front 87

Cyanotic

Right to Left Shunts:

MIxed Defects:

Back 87

Transposition of the Great Arteries - The aorta comes off of the RV and the pulmonary artery comes off of the LV. (5% of anomalies)
Unless the infant also has an ASD and/or VSD this is incompatible with life because you have two closed systems
RA - RV - Aorta - body - vena cavae to RA
LA - LV - Pulmonary artery - lungs - pulmonary veins to LA
Usually cyanotic at birth, may have no murmur or various murmurs
Echo will show enlarged heart. Cath can show low O2 sat in the heart chambers. ECG may not show anything
Will be given PGE1 to try to keep the ductus open. Can also have balloon passed through foramen ovale in order to enlarge the opening
Surgical intervention involves switching the aorta and the pulmonary artery

Front 88

Cyanotic

Right to Left Shunts:

Mixed Defects:

Back 88

Total Anamalous Pulmonary Venous Return
Pulmonary veins return to RA or superior vena cava instead of the LA
Systemic circulation depends on a patent foramen ovale and/or patent ductus
Often these patients are also without a spleen as well
The infant is usually mildly cyanotic and tires easily
Will be kept on PGE1 until surgery to keep the ductus arteriosus open.
The pulmonary veins are reimplanted into the LA

Front 89

Cyanotic

Right to Left Shunts:

Truncus Arteriosus -

Back 89

the infant has a single vessel coming off the RV and LV instead of a separate pulmonary artery and aorta.
There is usually also a VSD
Child is cyanotic and has a typical VSD murmur
The common trunk in restructured surgically to create an aorta and a pulmonary artery

Front 90

Defects that Obstruct Systemic Blood Flow

Aortic Stenosis:

Back 90

Prevents blood from flowing freely from the LV to the aorta
Can lead to hypertrophy of the left ventricle, heart failure, and pulmonary edema
The child is usually asymptomatic. Will usually hear a rough systolic murmur at 2nd ICS right sternal border which may radiate to right shoulder, clavicle or neck. May see signs of decreased CO (faint pulses, hypotension, tachycardia, poor feeding). Child may develop chest pain with activity. Sudden death can occur when O2 demand far exceeds supply
ECG may show left ventricular hypertrophy. Cath can show degree of stenosis
Treated with balloon angioplasty or surgical repair to divide the stenotic valve or dilate a constrictive aortic ring

Murmur radiates to right shoulder

Front 91

Coarctation of the Aorta:

Back 91

A constrictive band causes narrowing of the aorta either between the subclavian vein and the ductus arteriosus or distal to the ductus arteriosus
Since BP is greatest in the subclavian vein, you will see higher pressures in the upper extremities (at least 20 mmHg) than in the lower extremities. The increased BP can cause headaches or nosebleeds. Can even cause a CVA
May only have a decreased femoral pulse. As child grows older, collateral circulation develops. Veins may become visible on the chest. Child may complain of leg pain on exertion. Child may or may not have a soft or moderately loud systolic murmur prominent at the base of the heart

After corrective surgery child will be hyptertensive..NIPRIDE drip for a few days.
Rest of body has to adapt to having normal blood flow..after surgery child may complain of abdominal pain.

Repaired either with balloon angioplasty or surgical removal of the narrowed portion. Try to let the child grow for a while. Usually scheduled around age 2. Has to be done prior to childbearing age in females
May have abdominal discomfort for a while post-op related to increased blood flow to lower part of the body.
Will usually still have an elevated BP for a while.

Front 92

Hypoplastic Left Heart Syndrome:

Back 92

The left ventricle doesn’t work.
The RV hypertrophies as it tries to do all of the work
Patient becomes increasingly cyanotic as more unoxygenated blood is shunted to the left side
Treatment is aimed to keep the ductus arteriosus open and increase flow of blood to the aorta
No surgical repair at this time
Best hope is heart transplant

Cyanotic

Right to left shunting

Front 93

Cardiac Surgery

Preop

Postop

Back 93

Pre-op
baseline vital signs at rest (count a full minute)
height and weight
hold dig for 24 hrs
enema

If valve replacement surgery - artificial valves are used more often, requires anticoagulation (Coumadin) or antiplatelet (aspirin, dipyridamole) therapy
young girls should avoid accidental pregnancy, should not use an estrogen-based OC nor an IUD
women desiring to become pregnant need to switch to Heparin therapy
prophylactic antibiotics need to be taken before dental work or oral surgery
may develop hemolytic anemia requiring blood transfusions



Complications - bleeding, shock, heart block or arrhythmias, heart failure, neuro changes, postcardiac surgery syndrome (febrile illness with pericarditis and pleurisy - one week post-op), postperfusion syndrome (3-12 weeks post-op - fever, splenomegaly, hepatomegaly, elevated WBC, malaise, maculopapular rash - CMV from donor blood)


Coumadin-pt, inr
Heparin-ptt

patient and family teaching (surgery, equipment, what to expect after surgery, tubes, the importance of deep breathing and coughing)

Post-op
VS every 15 minutes progressing to q 1 hr, ventilatory support, monitoring of heart rate and rhythm, BP, heart pressures, heart sounds, UOP, fluid status, ABG’s, PT, PTT, Plt, Hgb, Hct., K, Na, O2 sat, lung sounds, bowel sounds, measurement of chest tube integrity and drainage
Incentive spirometry (deep breathing) and coughing after extubated
Pain management
Explain procedures (extubation, chest tube removal, etc.)
Administer antibiotics
Observe for signs and symptoms of infection
May need warming immediately post-op, but watch for temperature elevation
Assess for hypervolemia
Advance activities as tolerated
Avoid arm pulling and pulling with arms

Heart Transplant - for hypoplastic left ventricle or cardiomyopathy
ECG’s will show two P waves
Post-op care is the same as with other heart surgeries
Patients will be placed on immunosuppresive therapy
Rejection is the #1 cause of death
Hyperacute rejection - immediately upon restoring circulation
Acute rejection - occurs in about 7 days, fever, tachycardia, ECG changes
Chronic rejection - may begin at about a year

Pacemaker - if difficulty with conduction system
Consists of a battery pack and lead wires
Leads are usually epicardial as opposed to endocardial in children
Parents and patient must be taught how to take the patient’s pulse
Batteries can last up to 15 years and they lose power slowly rather than stopping abruptly
If patient has hiccuping, lead wire may need to be repositioned
Magnets should be avoided

Front 94

Acquired Heart Disease

Rheumatic Fever:

Back 94

Rheumatic Fever
An autoimmune disease that is a reaction to a group-A beta-hemolytic streptococcus infection
Often follows an attack of pharyngitis, tonsillitis, scarlet fever, “strep” throat, or impetigo
Very important that antibiotics are taken to treat the initial infection to prevent rheumatic fever
Systematic inflammatory disease that affects primarily the heart, joints, brain and skin. Strep loves heart valves. The mitral valve is the most commonly affected.
Diagnosis – Modifications to the Jones criteria (p.893) and elevated antistreptolysin (ASO) titer

Signs and Symptoms
Minor
fever
fatigue
joint tenderness
elevated ESR
Major
Aschoff bodies (hemorrhagic bullous lesions in the heart)
erythema marginatum
vegetation on valves
pericarditis
pericardial friction rub
muffled heart sounds
accentuated third heart sound
SC nodules
chorea (sudden involuntary movements of limbs)

2 major
Or
1 major and 2 minors

Diagnosis - If have two major symptoms or one major and two minor symptoms, rising or elevated ASO titer
Treatment
Bedrest until ESR decreases (degree of bedrest is based on degree of carditis)
Antibiotics (penicillin, erythromycin) x 10 days
Reduce inflammation (aspirin - watch for toxicity such as tinnitus, nausea, vomiting, headache, blurred vision)
Corticosteroids (if not responding to aspirin alone)
Phenobarbital for chorea
Treatment of heart failure
Prognosis depends on the amount of cardiac involvement
Kept on prophylactic antibiotics for 5 years or until 18 to prevent recurrence
Mitral valve replacement

Front 95

Kawasaki Disease:

Mucocutaneous Lymph Node Syndrome -

Back 95

an acute systemic vasculitis which leads to the formation of aneurysms and myocardial infarction
Actual cause is still unknown. Altered immune function occurs after an infection. There is an increase in antibody production. Antibody-antigen complexes apparently bind to the vascular endothelium and cause inflammation. The inflammation leads to platelet aggregation and the formation of thrombi or obstruction of heart and blood vessels


Acute Phase (Stage I)
High fever that doesn’t respond to antipyretics
Lethargic and irritable
May have red, swollen hands and feet, conjunctivitis, strawberry tongue and red, cracked lips, enlarged cervical lymph nodes, variety of rashes, abdominal pain as internal lymph nodes swell, anorexia, diarrhea, red and swollen joints
Elevated WBC and ESR
Subacute Phase - about 10 days after onset
Peeling of skin on palms and soles of feet
Platelet count rises (increases risk of clotting)
Aneurysms may form in coronary arteries - sudden death can occur


Convalescent Phase (Stage II) - begins at about the 25th day and lasts until 40 days
Stage III lasts from 40 days until the ESR returns to normal
Diagnosis is based on criteria (Box 25-11), blood studies and echocardiogram
Treatment
High dose aspirin – 80-100mg/kg/day in divided doses q6hr. After fever subsided give 3-5mg/kg/day
Anticoagulants are used to increase coronary dilation and prevent platelet aggregation
IV Gamma globulin - to reduce immune response
Observe child for signs of heart failure
Inspect and palpate extremities for warmth and capillary refill

Provide comfort measures
Protect edematous areas from pressure
May have dry, cracked lips
Monitor for dehydration, encourage fluids, but prevent fluid overload
Monitor nutritional status

Front 96

Bacterial Endocarditis:

Back 96

Inflammation and infection of the endocardium or valves of the heart
Generally caused by streptococcal viridans. Sometimes can be staphylococcal or fungal
Strep enters the blood stream during oral procedures, impetigo, UTI’s
Vegetation of bacteria, fibrin, and blood appear on the endocardium of the valves and heart chambers - destroys the endothelial lining
Signs and Symptoms - abrupt, unexplained low-grade intermittent fever, anorexia, malaise, weight loss, change in murmur, splenomegaly

Front 97

Endocarditis:

Diagnosis -

Treatment -

Back 97

blood culture, ECG, elevated ESR and C-reactive protein, anemias, leukocytosis, microscopic hematuria

antibiotics (PCN, Ampicillin, Gentamycin) for 2 to 8 weeks. Supportive therapy to prevent heart failure. Teach prevention with prophylactic antibiotics before procedures.

Front 98

Hypertension:

Primary HTN –

Back 98

Essential hypertension, idiopathic, rarely clinical evidence of disease, may have dizziness, HA, nose bleeds, visual disturbances, neuro deficits, extremity weakness and CVA

Front 99

Hypertension

Secondary HTN –

Back 99

symptom of underlying disease. Renal – weight loss, failure to gain weight, facial or pretibial edema, pale mucous membranes, unilateral or bilateral abdominal mass. Cardiac – absent or decreased femoral pulses, decreased in lower extremities, cardiomegaly, murmur, signs and symptoms of CHF
Children with HTN are usually taller and heavier than their peers. Obesity is a common concurrent condition
Coarctation of the aorta - the most common CV cause of HTN

Front 100

Hypertension

Diagnosis –

Back 100

BP on all extremities, CBC, UA, BUN, Cr, Uric Acid, Electrolytes, EKG, US of Kidneys, Arteriography
Therapeutic Management
Primary – focus on elimination of risk factors, weight reduction, exercise, sodium restriction, avoidance of alcohol and tobacco, relaxation techniques, alpha-and beta-adrenergic receptor blockers, diuretics, vasodilators, calcium channel blockers, ACE inhibitors
Secondary – treat the cause

Front 101

Hyperlipidemia:

Back 101

Identify kids at risk and treat early
Treatment: Dietary
Restrict intake of cholesterol and fats

Front 102

Hyperlipidemia:

Meds:

Back 102

If no response to diet, medications:
Colestipol (Colestid)
Cholestyramine (Questran)