fetal development
↑↑ Pulmonary Vascular Resistance (lungs not open)
↓↓ Systemic vascular resistance
Heart and other components of circulatory system are formed by 8th week
transitions at birth
Umbilicus clamped causing ↑Systemic vascular resistance
LV wall thickens
BP rises
Lungs expand causing ↓ Pulmonary vascular resistance
Oxygen saturation increases
Foramen Ovale closes
Ductus Arteriosus closes
normal heart chambers
LV: pump again most pressure, most muscular, highest pressure
RA: blood just returning from body, lowest pressure
RA, unchanged to RV: blood back from body, lowest PaO2
LA, unchanged to LV: blood directly from lungs, highest PaO2
blood flows from high to low pressure
murmurs
An indicator of increased or turbulent flow
not an emergency but can indicate a problem
Most children will have one at some point
Normal during periods of increased cardiac output like anemia, fever, rapid growth
Can also be caused by congenital heart defects or defective heart valves
Has nothing to do with rhythm (just has to do with flow of blood)
Types of heart disease in children
Acyanotic
defect that allows blood to pass through opening
blood goes from left side of the heart to the right side of the heart, still have oxygenated blood going to the rest of the body
not necessarily going to see the signs of decreased oxygenation
^^LEFT TO RIGHT ^^
Cyanotic
unoxygenated blood going to rest of body
goes from right side to the left, signs of decreased oxygenation
^^RIGHT TO LEFT ^^
Congenital Heart Disease
anatomic defects of the heart that prevent normal blood flow to the pulmonary and/or systemic system
CHD risk factors
Maternal factors: alcohol or other substance use disorder during pregnancy, infection, diabetes mellitus
May have a genetic component
DiGeorge Syndrome
Noonan Syndrome
Trisomy 13, 18, 21*
May occur with other physical anomalies
Cleft lip / palate
CHD diag.
Chest x-ray: heart size
Echocardiogram: evaluate heart function and defects
MRI: more specific than echo
Cardiac Catheterization: measures pressures and oxygen levels in all 4 chambers; maps blood flow through the heart
Invasive test
Defects are categorized by blood flow patterns in the heart
defects in categories by blood flow
↑ pulmonary blood flow (acyanotic, left to right, more blood flows to the lungs)
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
↓ pulmonary blood flow (cyanotic, right to left, skipping pulmonary artery)
Tetralogy of Fallot
Tricuspid atresia
Obstruct (blood flow exiting heart meets area of stenosis, increased pressure when trying to leave the heart)
Coarctation of the Aorta
Pulmonary & aortic stenosis
Mixed
Transposition of the great arteries (TGA)
Hypoplastic left heart
Acyanotic: Ventricular Septal Defect (VSD)
↑ pulmonary blood flow (acyanotic, left to right, more blood flows to the lungs)
hole bw right and left ventricle
Loud, harsh murmur heard at left sternal border
Can use diuretics, ACE Inhibitors (decrease pressure and amount of blood flow from left to right side)
Backup of fluid in right ventricle, right sided heart failure, crackles in lungs, right ventricular hypertrophy
Untreated VSD: eventually causes both oxygenated AND unoxygenated blood to go into body (Eisenmenger Syndrome)
VSD s&s
^^H^^eart failure and pulmonary hypertension
^^O^^ften experiences lung infections (increased fluid in the lungs)
^^L^^ow growth rate/weight
^^E^^xtra heart sound (murmur)
^^S^^troke
Many will close spontaneously early in life, but if large will need surgery
VSD treatment/therapies
Closure during cardiac catheterization
Diuretics: watch K levels; help pull extra fluid
Complete repair with patch (risk for complete heart block)
Digoxin: will help heart contract stronger and slow it down
Acyanotic: Atrial Septal Defect (ASD)
↑ pulmonary blood flow (acyanotic, left to right, more blood flows to the lungs)
bw right and left atrium, blood going from left to right side of heart
lot of harsh murmur with fixed split second heart sound
Heart failure
Most will close on their own, treated as heart failure
May be asymptomatic if hole is small
ASD s&s
^^H^^eart failure and pulmonary hypertension
^^O^^ften experiences lung infections (increased fluid in the lungs)
^^L^^ow growth rate/weight
^^E^^xtra heart sound (murmur)
^^S^^troke
ASD treatment/therapies
Closure during cardiac catheterization
Diuretics
Low dose aspirin 6 months after procedure
Patch closure
Cardiopulmonary bypass
Cyanotic: Patent Ductus Arteriosus (PDA)
↑ pulmonary blood flow (acyanotic, left to right, more blood flows to the lungs)
Normal fetal circulation conduit between pulmonary artery and aorta
If it stays open, blood from aorta goes into pulmonary artery which will go into lungs and will increase pulmonary blood flow (acyanotic)
Fatigue easily, usually will need extra calories
Widened pulse pressure (bigger gap between systolic and diastolic) (decreased pressure during diastolic)
Systolic murmur (machine hum)
Wide pulse pressure
Bounding pulses
May be asymptomatic
Heart failure
Rales
PDA treatment/therapies
Administer indomethacin to allow for closure
Diuretics (Lasix)
Extra calories for infants
Thoracoscopic repair
Cyanotic: Tetralogy of Fallot (TET)
↓ pulmonary blood flow (cyanotic, right to left, skipping pulmonary artery)
4 components:
Right ventricular hypertrophy
Aortic displacement (overriding)
Pulmonary stenosis
Septal defect (ventricular)
Cyanosis at birth: progressive cyanosis over first year of life
Systolic murmur
Clubbing of fingers, trouble breathing
Tetralogy of Fallot treatment/therapies
Surgical procedures: shunt placement until able to undergo primary repair
Complete repair within first year of life
TET spell
Acute episode of cyanosis: crying, screaming
Sudden increase of pressure in the lungs forces blood to the left side & out to the system leading to sudden cyanosis
Brought on by coughing, feeding, stooling, crying
Resolved by increasing systemic resistance to match pressure
Children will have trouble feeding, fingernail changes (clubbing)
treatment
Put them in knee chest position
help increase systemic pressure which will increase the blood flow and oxygenation to the body
Obstructive: Pulmonary stenosis
narrowing of the pulmonary valve or pulmonary artery that results in obstruction of blood flow from the ventricles
Systolic ejection murmur
Symptoms depend on severity of obstruction
Cyanosis varies; worse with severe narrowing
Cardiomegaly
Heart failure
Right side of heart will have to work harder to get blood flow to the lungs
tachypnea
increased work of breathing
shortness of breath
tachycardia
edema (decreased blood flow going to the lungs)
will lead to right ventricular hypertrophy
Pulmonary stenosis treatment/therapies
Balloon angioplasty
Infants: brock procedure
Children: valvotomy
Obstructive: Aortic Stenosis
narrowing of the aortic valve = systemic blood flow affected
Infants: faint pulses, hypotension, tachycardia, poor feeding tolerance, poor weight gain
Children: intolerance to exercise, dizziness, chest pain, possible murmur
Blood flow up to aorta will be compromised
hypotension
weak pulses
exercise intolerance
dizziness
beta blockers or calcium channel blockers can be used for treatment
Aortic Stenosis treatment/therapies
Balloon dilation
Administer beta blockers, Ca channel blockers
Norwood procedure (replacing aortic valve)
Aortic valvotomy
Obstructive: Coarctation of the aorta
narrowing of the lumen of aorta, usually at ductus arteriosus → obstruction of blood flow from the ventricle
Systemic blood flow drops dramatically as shunt closes
Stenosis/ atresia of valves on left side
Infants: heart failure
Children: headaches, dizziness, fainting, nosebleeds
low narrowing would cause higher blood pressure in upper extremities (bounding pulses, headache, stroke) and lower blood pressure in lower extremities (cold, diminished pulse)
Coarctation of the aorta treatment/therapies
May give prostaglandins to keep PDA open
Balloon angioplasty or shunts
Anyone less than 6 months should have repair
mixed: Transposition of the Great Arteries
Backwards heart 🙁
aorta is connected to right ventricle
pulmonary artery is connected to left ventricle
not sustainable to life
two closed systems that are not connected (nothing going to the body gets oxygenated)
septal defect or PDA must exist in order to oxygenate the blood; VSD & ASD are common
Murmur
Cardiomegaly
Heart failure
Transposition of the Great Arteries SWAP
S: severe cyanosis (low O2, increased heart rate, increased respiratory rate, poor feeding)
W: Watch rhythm, O2
A: Alprostadil (med that keeps shunts open)
P: Procedures to correct
Transposition of the Great Arteries treatment/therapies
surgery must be done to switch arteries within first 2 weeks of life
IV prostaglandin E to keep ducts open
Infants with CHD
Fail to make the transition
Usually are tachypneic
Are poor feeders
Are sweaty
May be cyanotic
Have pale/cool extremities with weak pulses
May have a murmur
Children with CHD
Have exercise intolerance
Have respiratory problems
May have edema
May complain of chest pain
May faint or have headaches
May complain of palpitations
May have a murmur
nursing managment of infant with CHD
Monitor fluid balance
Monitor perfusion
Monitor work of breathing, particularly during feedings
Ensure periods of rest
Monitor growth
Reassurance / education for families
Administer medications as ordered
Digoxin
Furosemide (lasix)
Spironolactone
ACE inhibitors
Sildenafil Citrate
Feeding tubes as needed
feeding in CHD
Follow baby cues, don’t wait for cry
Feed for limited period, gavage remainder
NG/OG tubes common; GT tubes also
Limit calorie expenditure for feedings
Allow rest while still receiving nourishment
May need calorically dense formula to get more calories in less fluid
High calorie formulas, fortified breast milk
Position important: upright, side-lying
When would patient be cyanotic?
Hole that allows mixing of unoxygenated and oxygenated blood that is pumped to the rest of the body
Right side is under higher pressure than the left
Signs of chronic hypoxemia
Clubbing
Polycythemia
body will start making more RBC to try to get more oxygen and nutrients to rest of body
Nursing Management for patients needing cardiac catheter
May be via femoral vein
Frequent VS
Assess for hemorrhage
Monitor rhythm
Strict bed rest for several hours
Strict I&O
surgeries
Palliation (band / shunt)
To protect lungs from too much flow
To allow mixing
Repair, may need to do in stages
Transplant
Post-operative nursing care:
Pressors
Pain management
Wound care
Meticulous assessment of perfusion/ ventilation/ renal function/ conductivity
Speech therapy for oral aversion
Reconnecting with parents
Dysrhythmias: Typically secondary
Bradycardia
Vagal
Disrupted conduction
Digoxin toxicity
Tachycardia
SVT (supraventricular tachycardia) most common; treated with cardioversion or adenosine
Stable (not symptomatic) : vagal maneuvers (put icepack on infant’s face, blow as hard as they can through a straw, turn baby upside down)
Unstable (sweaty, dizzy, not feeling well, pale): cardioversion or adenosine
Wolff Parkinson White Syndrome
heart beats abnormally fast for periods of time; have increased risk for sudden death
Acquired Cardiovascular Disorders
Cardiovascular disease the child is NOT born with. They develop it secondary to a condition
Interventions for all acquired heart diseases
Maintain adequate oxygenation
Maintain adequate cardiac output and tissue perfusion
Maintain hydration and nutrition
Promote pain relief, comfort, rest
Administer and manage medications
Monitor for and prevent complications
Promote growth and development
Provide emotional and psychosocial support for child and parents
HF
inability of the heart to pump an adequate amount of blood to the systemic circulation to meet body’s demands
In children, most commonly related to congenital heart disease
HF causes can be classified as
Ventricular dysfunction:
Normal structured heart: cardiomyopathy, myocarditis, myocardial ischemia, drugs & toxins; also sepsis
CHD: congenital heart disease
Volume overload with normal ventricular contractility: may be due to cardiac or noncardiac causes
Cardiac: VSD, PDA, AV septal defect, aortic stenosis, mitral regurg
Noncardiac: oliguric renal failure
Pressure overload with normal ventricular contractility
Often results from ventricular outflow obstruction
Aortic stenosis, coarc of aorta, pulmonary stenosis
HR types
Right sided HF: RV function is reduced
End diastolic pressure rises causing increased central venous pressure and systemic venous engorgement
Can lead to hypertension, hepatomegaly, and edema
Left sided HF: LV dysfunction occurs and end-diastolic pressure rises
Result= increased pressure in LA and pulmonary veins
Lungs become congested with blood à pulmonary edema & elevated pulmonary pressures
HR compensatory
Heart initially tries to meet body’s demand → hypertrophy and stimulation of sympathetic nervous system
Hypertrophy is from heart working harder to try to get that blood out to the body
HF in infants symp.
Tachypnea
Diaphoresis during feedings
Irritable
Decreased volume of feeds
Poor weight gain
HF in younger children symp.
Abdominal pain
Nausea /vomiting
Poor appetite
Cough/wheeze
HF in older child symp.
Anorexia
Abdominal pain
Edema
Palpitations
Chest pain
Dizziness
Exercise intolerance
Wheezing
Dyspnea
HF labs
BNP: elevated
Troponin: elevated
CBC: check for anemia
CMP: hyponatremia
BUN & creatinine: may be elevated
HF diag.
Based on clinical & lab findings
Also get EKG, Echo
HF nursing care
Maintain adequate oxygenation,
maintain cardiac output and myocardial function
Monitoring fluid balance
Administer medications: Lasix, digoxin, enalapril, carvedilol
Can add polycose to infant’s bottles (feed every 3 hours no more than 30 minutes)
HF meds
Lasix: results sodium rich diuresis
must monitor urine output, potassium, daily weights
Digoxin: cardiac glycoside: (apical HR prior)
*need to get HR prior to administration**
Enalapril: ace inhibitor
monitor BP, watch for cough, nausea, diarrhea, headache
Carvedilol: decreases the excitability of the heart
check apical pulse, monitor bp
cardiomyopathy
Disease of the heart muscle
classifications
dilated
hypertrophic
restrictive
cardiomyopathy: dilated
most common – present with signs of heart failure (ABNORMAL PUMPING AND CONTRACTING)
Heart muscle becomes thin, LV becomes dilated & heart becomes unable to squeeze effectively
Most common cause = myocarditis (inflammation of the heart muscle)
Genetic forms: more than half the cases
cardiomyopathy: hypertropic
genetic increase in heart muscle mass; usually asymptomatic – cardiac arrest/sudden death (ABNORMALLY THICK)
Accounts for over 40% of cardiomyopathy cases
Enlargement of LV and septum, wall then becomes rigid
Then causes obstruction of blood outflow, diastolic dysfunction à myocardial ischemia
cardiomyopathy: restrictive
rare
prevents filling of ventricles (USUAL AMOUNT OF BLOOD NOT ABLE TO GET IN)
cardiomyopathy signs
Tachycardia and dysrhythmia
Dyspnea
Fatigue and poor growth
DCM (dilated cardiomyopathy): most present with signs of HF including tachypnea, cough, wheezing, tachycardia, weak pulses, and fatigue
Palpitations, syncope, poor infant feeding-respiratory distress
HCM (hypertrophic cardiomyopathy): symptoms depend on degree of obstruction
In previously healthy, may not have symptoms until cardiac arrest
cardiomyopathy treatment/therapies
Beta blockers, calcium channel blockers, ACE inhibitors, anticoagulants
Heart transplant
cardiomyopathy diag.
Chest x-ray
ECG
Echo
KIDS THAT DROP DEAD: HYPERTROPHIC CARDIOMYOPATHY
Rheumatic Fever
Autoimmune inflammatory reaction
worldwide , most common form of acquired heart diseases in all age groups
Develops 2-4 weeks after Group A step infection in throat
Affects heart (carditis), blood vessels, and joints
Most commonly affects heart valves
Can lead to rheumatic heart disease which leads to disability and death
Can recur
Rheumatic Fever: 2 theories
Cytotoxic theory
produces several enzymes that are cytotoxic including streptolysin O; has a direct cytotoxic effect on cells
Immunologic theory
some people have specific immune response to repeated strep infections and have increased cellular response to strep antigen
Rheumatic Fever findings
Hx of recurrent URI
Fever
Tachycardia, cardiomegaly, new or changed heart murmur, pericardial friction rub
Large joints* (knees, elbows, wrists, ankles, shoulders) with painful swelling indicating polyarthritis
Pink, nonpruritic macular rash on the trunk and inner surfaces of extremities that appears and disappears rapidly
CNS involvement: involuntary, purposeless muscle movements, muscle weakness, involuntary facial movements, random uncoordinated movements
Irritability, poor concentration, behavioral problems
Rheumatic Fever: Jones criteria
Major:
Carditis
Subcutaneous nodules
Polyarthritis (swelling of large joints, knees, elbows, angles)
Rash (erythema marginatum)
Chorea (CNS involvement) (meaningless movements)
Minor:
fever OR arthralgia
elevated CRP or ESR
prolonged PR
Rheumatic Fever diag.
strep swab for those with sore throats
blood antistreptolysin o titer; most reliable diagnostic (blood test that shows if they have had strep)
increased CRP & ESR (both show inflammation)
echo: to see if pericardial effusions
Rheumatic Fever nursing considerations
Education on prevention * (anyone that has strep needs to take full course of antibiotics)
Prevention
Bed rest
Rheumatic Fever treatment
Administer antibiotics as prescribed to treat disease then prophylactically to prevent complications
Either penicillin BID or monthly IM injections of penicillin
Length of treatment depends on residual heart disease; anywhere from 5 years to indefinitely
Administer aspirin or NSAIDs as prescribed for anti-inflammatory and antiplatelet
endocarditis
Infection of the inner lining of the heart and valves that can enter the bloodstream
Common causes: strep viridans, candida albicans, staph aureus
endocarditis risk factors
congenital or acquired heart disease
indwelling catheters
endocarditis expected findings
some may only have fever/nonspecific symptoms
may present with high spiking fevers and appear very ill
Neonates: feeding problems, respiratory distress, tachycardia, heart failure, septicemia
Older children: fever, malaise, new murmur, myalgias, diaphoresis, weight loss, splinter hemorrhages under fingernails
Osler nodes: painful red bumps under skin hands and toes
Janeway lesions: nontender blanching lesions on soles of feet and hands
Inflammation of walls of blood vessels (thought to be related to infections)
endocarditis labs
CBC, ESR, urinalysis, blood cultures (+ for diagnosis)
endocarditis diag.
EKG, echo, blood culture, CBC, ESR, CRP
endocarditis nursing interventions
Comfort measures
Prophylactic antibiotics: before dental procedures
Treated with long course of antibiotics
endocarditis: duke criteria
Major:
+blood cultures
Evidence of endocardial involvement
Echo positive for vegetation, abscess
Minor
Presence of predisposed condition or IV drug use
Fever
Vascular condition
Microbiological evidence
Kawasaki Disease
Vascular inflammatory disorder
Etiology = unknown, infectious?
Strawberry tongue
Most common acquired Cardiovascular Disorder in US
Worse outcomes occur in those <1 years old or >5 years old
Complex presentation – has criteria for diagnostic work up & treatment
Kawasaki Disease phases
Acute phase
1-2 wks; fever lasting >5 days
conjunctival erythema without exudate
swollen hands and feet
cracked lips
strawberry tongue
Subacute phase
fever subsided
may see desquamation of fingers and toes
high platelets
joint pain
anorexia
Convalescent phase
complete resolution of signs (3 months after onset)
Coronary aneurysm may be present, MI remains a concern
Kawasaki Disease diag.
Acute phase: elevated ESR, CRP, antitrypsin, elevated WBC
Subacute: increased platelets (usually over 700,000)
May get EKG and echo to check for any changes
Kawasaki Disease nursing interventions
Administer aspirin as ordered
Ok to mix with soft food to assist young children
Administer IVIG (immunoglobulin) to decrease risk of developing coronary aneurysm
When is this repeated? 24 hours later if they are still febrile
Hydration & nutrition
Avoid live immunizations for about 1 year
MIS-C= Multisystem Inflammatory Syndrome in Children
Occurs within 4 weeks of COVID infection – even those who are asymptomatic
Multisystem (2 or more) organ involvement
Presentation very similar to Kawasaki
Labs will show elevated CRP & ESR
Some develop myocarditis & cardiac dysfunction
Kids will have elevated troponin and BNP
meds: aspirin
Only given to children!!
Post cardiac surgery or trauma to prevent clot
For treatment of rheumatic fever
For treatment of Kawasaki: high inflammation dose first, then lower antiplatelet dose second
Monitor hepatic function before and during therapy
meds: digoxin
Improves myocardial contractility
Need to monitor apical pulse and withhold medication if:
Heart rate of infants: less than 90
Heart rate of children: less than 70
Dig toxicity
Infants and children: bradycardia, anorexia, nausea, vomiting
Can also have neuro and visual disturbances
Has a very long half life (18-35 hours) so many take days before level back to normal range
Check potassium level before starting, if hypokalemic they will withhold the dose (watch potassium closely during therapy), withhold the dose if there are any signs of dig toxicity
Annual readings for blood pressure starting at age 3
hypertension
All children >3 years old should have blood pressures measured annually – diagnosis made on blood pressure readings from 3 separate visits
Primary: no identifiable cause; more likely to occur in school age children or adolescents who are overweight / have family hx
Secondary hypertension: has an identifiable cause; renal disease, endocrine disease, renovascular disease