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1

fetal development

  • ↑↑  Pulmonary Vascular Resistance (lungs not open)

  • ↓↓ Systemic vascular resistance

  • Heart and other components of circulatory system are formed by 8th week

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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

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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

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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)

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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 ^^

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Congenital Heart Disease

anatomic defects of the heart that prevent normal blood flow to the pulmonary and/or systemic system

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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

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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

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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

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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)

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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

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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

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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

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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

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ASD treatment/therapies

  • Closure during cardiac catheterization

  • Diuretics

  • Low dose aspirin 6 months after procedure

  • Patch closure

  • Cardiopulmonary bypass

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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

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PDA treatment/therapies

  • Administer indomethacin to allow for closure

  • Diuretics (Lasix)

  • Extra calories for infants

  • Thoracoscopic repair

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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

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Tetralogy of Fallot treatment/therapies

  • Surgical procedures: shunt placement until able to undergo primary repair

  • Complete repair within first year of life

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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

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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

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Pulmonary stenosis treatment/therapies

  • Balloon angioplasty

  • Infants: brock procedure

  • Children: valvotomy

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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

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Aortic Stenosis treatment/therapies

  • Balloon dilation

  • Administer beta blockers, Ca channel blockers

  • Norwood procedure (replacing aortic valve)

  • Aortic valvotomy

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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)

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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

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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

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Transposition of the Great Arteries SWAP

S: severe cyanosis (low O2, increased heart rate, increased respiratory rate, poor feeding)

W: Watch rhythm, O2

AAlprostadil (med that keeps shunts open)

P:  Procedures to correct

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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

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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

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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

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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

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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

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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

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Signs of chronic hypoxemia

  • Clubbing

  • Polycythemia

    • body will start making more RBC to try to get more oxygen and nutrients to rest of body

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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

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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

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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

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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

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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

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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

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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

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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

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HF in infants symp.

  • Tachypnea

  • Diaphoresis during feedings

  • Irritable

  • Decreased volume of feeds

  • Poor weight gain

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HF in younger children symp.

  • Abdominal pain

  • Nausea /vomiting

  • Poor appetite

  • Cough/wheeze

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HF in older child symp.

  • Anorexia

  • Abdominal pain

  • Edema

  • Palpitations

  • Chest pain

  • Dizziness

  • Exercise intolerance

  • Wheezing

  • Dyspnea

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HF labs

  • BNP: elevated

  • Troponin: elevated

  • CBC: check for anemia

  • CMP: hyponatremia

  • BUN & creatinine: may be elevated

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HF diag.

  • Based on clinical & lab findings

  • Also get EKG, Echo

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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)

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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

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cardiomyopathy

Disease of the heart muscle

classifications

  • dilated

  • hypertrophic

  • restrictive

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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

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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

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cardiomyopathy: restrictive

  • rare

  • prevents filling of ventricles (USUAL AMOUNT OF BLOOD NOT ABLE TO GET IN)

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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

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cardiomyopathy treatment/therapies

  • Beta blockers, calcium channel blockers, ACE inhibitors, anticoagulants

  • Heart transplant

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cardiomyopathy diag.

  • Chest x-ray

  • ECG

  • Echo

  • KIDS THAT DROP DEAD: HYPERTROPHIC CARDIOMYOPATHY

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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

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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

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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

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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

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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

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Rheumatic Fever nursing considerations

  • Education on prevention * (anyone that has strep needs to take full course of antibiotics)

  • Prevention

  • Bed rest

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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

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endocarditis

Infection of the inner lining of the heart and valves that can enter the bloodstream

  • Common causes: strep viridans, candida albicans, staph aureus

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endocarditis risk factors

  • congenital or acquired heart disease

  • indwelling catheters

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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)

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endocarditis labs

  • CBC, ESR, urinalysis, blood cultures (+ for diagnosis)

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endocarditis diag.

  • EKG, echo, blood culture, CBC, ESR, CRP

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endocarditis nursing interventions

  • Comfort measures

  • Prophylactic antibiotics: before dental procedures

  • Treated with long course of antibiotics

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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