peds cv

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

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RA: blood just returning from body, lowest pressure

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RA, unchanged to RV: blood back from body, lowest PaO2

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LA, unchanged to LV: blood directly from lungs, highest PaO2

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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** ^^

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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)

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**↓ pulmonary blood flow** (^^cyanotic^^, right to left, skipping pulmonary artery)

* Tetralogy of Fallot
* Tricuspid atresia

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**Obstruct** (blood flow exiting heart meets area of stenosis, increased pressure when trying to leave the heart)

* Coarctation of the Aorta
* Pulmonary & aortic stenosis

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**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

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**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

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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:**

* **R**ight ventricular hypertrophy 
* **A**ortic displacement (overriding)
* **P**ulmonary stenosis
* **S**eptal defect (ventricular)

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* 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)

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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

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**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

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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

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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

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**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

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**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

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**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

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**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 b**p**

cardiomyopathy

Disease of the heart muscle

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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 

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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)

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**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

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* 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

Kawasaki Disease phases

Acute phase

* 1-2 wks; fever lasting >5 days
* conjunctival erythema without exudate
* swollen hands and feet
* cracked lips
* strawberry tongue

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Subacute phase

* fever subsided
* may see desquamation of fingers and toes
* high platelets
* joint pain
* anorexia

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Convalescent phase

* complete resolution of signs (3 months after onset)

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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**

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* **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