congenital heart defects

assessment of cardiac function in children

• history

  • parental concerns

  • mother’s health and pregnancy

  • family history

• inspection

  • nutritional status

  • color

  • chest deformities

  • unusual pulsations

  • respiratory excursion

  • digital clubbing

assessment of a child with possible cardiovascular disorder

• palpation and percussion

  • chest

  • abdomen

  • peripheral pulses

• auscultation

  • heart rhythm (full minute)

  • blood pressure (both arms, one leg)

  • character of heart sounds

diagnostic evaluation of heart abnormalities

• chest xray

• ECG

• CBC (for polycythemia)

• echocardiogram

• arterial blood gas

• cardiac catheterization

cardiac catheterization

• diagnostic

• interventional

• electrophysiology results

• right side more common in kids because it is safer and structural defects allow access to the left side of the heart

pre-procedural care for cardiac catheterization

• nursing assessment (skin, pulses, weight, height)

• NPO 4-6 hours, clarify AM meds

• IV fluids?

• developmentally appropriate psychological prep

• sedation?

post-procedural care for cardiac catheterization

• observation for complications

  • color and level of consciousness

  • vital signs (take HR for one full minute, pay attention to BP) and respiratory status

  • distal extremities (pulses distal to site can be weaker first few hours)

  • dressing for bleeding (hold pressure 1 inch above site if bleeding)

  • fluid intake; both IV and PO

  • hypoglycemia

  • strokes

    • confusion, face or arm weakness, slurred speech, loss of balance

discharge planning for cardiac catheterization

• pressure dressing for 24 hours

• no tub baths for 3 days, may shower next day

• rest and quiet activities for 3 days then can go back to school, but avoid strenuous activity until cardiologist releases

• regular diet

• ibuprofen or tylenol for pain

• teach signs and symptoms of infection

developmental considerations: heart size

• ventricles are equal in size at birth

• during infancy, muscle fibers of heart are less developed and less organized resulting in limited functional capacity

developmental considerations: O2 saturation

normal is 95-100%

developmental considerations: fat and muscle

infants and small children have thin chest walls with little to no subcutaneous fat and muscle

how does fetal circulation differ from adult circulation

• liver and lungs are bypassed via shunts

  • ductus arteriosus - bypass lungs

  • foramen ovale - bypass lungs

  • ductus venosus - bypass liver

• oxygenation and filtration of impurities are conducted by mother

• fetal circulation ensure that the most vital organs and tissues receive the max concentration of oxygenated blood

fetal shunts

all close at birth or shortly after in response to

• decreased maternal hormone prostaglandin E

• increased O2 saturations

• pressure changes within the heart

general clinical findings for cardiac defects

• dyspnea

• feeding difficulty and failure to thrive

• stridor or choking spells

• HR over 200; respiratory rate about 60 in infant

• recurrent respiratory tract infections

• in the older child - poor physical development, delayed milestones, and decreased exercise tolerance

• cyanosis and clubbing of fingers and toes

• squatting or knee chest position

  • blood meets resistance in legs, keeps oxygenated blood circulating in vital organs

• heart murmurs

• excessive perspiration

• signs of heart failure

cardiac anatomy

acyanotic heart defects

• atrial septal defects

• ventricular septal defects

• patent ductus arteriosus

• coarctation of aorta

• aortic stenosis

• pulmonic stenosis

atrial septal defects, ventricular septal defects, and patent ductus arteriosis

increased pulmonary blood flow

coarctation of aorta, aortic stenosis, pulmonic stenosis

• obstructive lesions

• decreased blood flow to areas of body

two principal congenital heart defect clinical consequences can occur

1. caused by defects that result in left to right shunting of blood

2. caused by defects that result in decreased pulmonary blood flow

clinical consequences of defects with increased pulmonary blood flow

• systemic pressure is greater than the pulmonary pressure so left to right shunting occurs

• increased blood volume on right side of heart increases pulmonary blood flow at the expense of systemic blood flow (more going to lungs, less going to body)

• s/s of CHF

congestive heart failure (CHF)

chronic condition where the heart muscle can’t pump enough blood to meet the body’s needs, which causes buildup of fluid in lungs and other organs

causes of congestive heart failure

• volume overload

• pressure overload

• decreased contractility

• high cardiac ?

  • children with CHF occur most frequently where structural abnormalities result in increased volume overload or increased pressure load on ventricles

clinical manifestations in CHF: pulmonary venous congestion

• tachypnea

• wheezing

• crackles

• retractions

• cough

• dyspnea on exertion

• grunting

• nasal flaring

• cyanosis

• feeding difficulties

• irritability

• fatigue with play

clinical manifestations in CHF: systemic venous congestion

• hepatomegaly

• ascites

• edema

• weight gain

• neck vein distension

clinical manifestations in CHF: impaired myocardial function (cardiac output)

• tachycardia

• weak peripheral pulses

• hypotension

• gallop rhythm

• extended capillary refill

• pallor

• cool extremities

• oliguria

• fatigue

• restlessness

• enlarged heart

• sweating

clinical manifestations in CHF: high metabolic rate

• failure to thrive or slow weight gain

• perspiration

therapeutic management of CHF

• improve cardiac function

• remove accumulated fluid and sodium (can also be limited on diet)

• decrease cardiac demands

• improve tissue oxygenation and decrease oxygen consumption

medications used in CHF

• furosemide (Lasix)

• ACE inhibitors

• Digitalis (digoxin)

furosemide

• diuretic

• give foods high in potassium

ACE inhibitors

• ex - captopril, lisinopril, enalapril

• reduces afterload, so monitor BP before and after giving

• most are potassium sparing

digitalis

• only oral med that increases contractility

• potassium and digoxin have inverse relationship (potassium low = digoxin more effective; potassium high = digoxin less effective)

rules for administration of digoxin

• given at regular intervals

• 1 hour before and 2 hours after eating

• check apical HR for 1 min before giving 

  • infants and young kids - hold is <90-110

  • older children - hold is <70

• do not mix with food or fluid

• behind teeth or brush after administering

• missed dose rules

  • < 4 hours - give missed dose

  • > 4 hours - withhold

  • if 2 doses are missed, notify practitioner

  • if child vomits, do not repeat dose

• check potassium levels prior to giving digoxin (hold if potassium levels are low)

digoxin toxicity symptoms

• nausea

• vomiting

• bradycardia

• anorexia

• neurological and visual disturbances

digoxin toxicity

• monitor child closely for dysrhythmias (digoxin toxicity can cause hyperkalemia)

• digibind (antidote; watch potassium levels)

  • binds to digoxin or other cardiac glycosides and is excreted by kidneys and removed from body

  • watch for rapidly dropping potassium levels

nursing considerations for CHF: activity intolerance

• promote adequate rest

• prevent crying

• group activities

• short intervals of play, cuddling

• provide neutral thermal environments

• supplemental oxygen

  • depends on orders

  • satting in 80’s can be okay

nursing considerations for CHF: altered nutrition

• infants will have higher metabolic rate bc of poor cardiac function and increased HR and RR

• anticipate hunger

• smaller, more frequent feedings (q 3 hours rather than q 2 or 4)

• feed no longer than 30 mins at a time and give remaining via NG

• feed in relaxed environment

• semi-erect position for feeding

• burp before, during, and after feeding

• formula with increased calories per ounce

• soft preemie nipple with moderately large opening

nursing considerations for CHF: ineffective breathing patterns

• assess rr, effort, and O2 saturations

  • > 60 = hold feeds

• position to encourage maximum chest expansion (not as important in infants)

• avoid constriction

• humidified supplemental oxygen; during stressful periods (crying or invasive procedures)

nursing considerations for CHF: potential for infections

• avoid crowded public spaces

• good handwashing

• screen visitors

nursing considerations for CHF: fluid volume excess

• accurate I&O

• weigh daily - same time, scale, and clothes

• assess for edema

• maintain fluid restriction if ordered

• provide good skin care

• change position frequently

nursing considerations for CHF: growth and development

• developmentally appropriate toys

• infants do catch up

nursing considerations for CHF: coping

• detailed teaching

• emotional support

family education and support for CHF

• teach s/s of worsening clinical status

• information on how to give meds

• stress importance of good nutrition

  • have high caloric requirements and get tired and tachypnic easily

• immunizations - stay up to date, if < 2 years need RSV prophylaxis

• promotion of growth and development

arterial septal defect

defect stems from patent foramen ovale of failure of a septum to completely develop between the right and left atria

• increased pulmonary blood flow

clinical manifestations of arterial septal defect

• may be asymptomatic

• heart murmur

• CHF

• increased risk for dysrythmias with pulmonary vascular obstructive disease and emboli later in life

treatment of arterial septal defect

• mild defects may close spontaneously

• open heart surgery and dacron patch closure

  • may be closed using devices (septal occluder) during cardiac catheterization

ventricular septal defect

the septum fails to completely form between the right and left ventricles

• increased pulmonary flow

clinical manifestations of ventricular septal defect

• CHF; moderate to severe

• cyanosis

• characteristic murmur

• right ventricular hypertrophy

• failure to thrive

• fatigue

• recurrent respiratory infections

therapeutic management of ventricular septal defect

• pulmonary artery banding

• may close spontaneously by age 3

• interventional heart cath with septal occluder

• surgical correction with patch and repair of AV valve tissue

patent ductus arteriosus (PDA)

the fetal structure fails to close - blood is shunted from aorta to pulmonary artery

• increased pulmonary blood flow

clinical manifestations of patent ductus arteriosus

• machine like murmur

• can be asymptomatic

• CHF

treatments for patent ductus arteriosus

• indomethacin (prostaglandin E inhibitor like ibuprofen) will cause defect to close on its own with a couple doses

• interventional heart cath with coil

• left thoracotomy or video assisted thoroscopic surgery (VATS) to place clip on ductus

clinical consequences of obstructive lesions

• blood exiting heart meets area of anatomic narrowing, causing obstruction to blood flow

• usually occurs near valve as in aortic and pulmonic stenosis

• either shunting (left to right) or backup of blood on right side

• increased pulmonary congestions

• signs of CHF

coarctation of the aorta

narrowing of aortic arch, usually distal to the ductus arteriosus and beyond the right subclavian artery

• obstructions of blood flow

• results in increased pressure proximal to defect (head and upper extremities) and decreased pressure distal to the defect (body and lower extremities)

two locations of coarctation

• preductal

• postductal

preductal coarctation of aorta

between subclavian artery before ductus arteriosus

postductal coarctation of aorta

collateral circulation develops during fetal life (distal to ductus arteriosus)

clinical consequences of coarctation of aorta

• L → R shunting, increased pulmonary blood flow leading to CHF

• increased blood flow to head and upper extremities

• decreased blood flow to trunk and lower extremities

therapeutic management of coarctation of aorta

surgery within first two years

long term complications can include

• recoarctation

• aortic aneurysm

• systemic hypertension

aortic stenosis

narrowing or fusion of aortic valves which interferes with left ventricle outflow; blood backs up into right side of heart and enlarges

• obstructions of blood flow

• results in decreased cardiac output, left ventricular hypertrophy (LVH), and pulmonary vascular congestion

aortic stenosis: serious defect

• obstruction tends to be progressive

• sudden episodes of myocardial ischemia, or lower cardiac output, can result in sudden death

• only defect where activity is limited

• surgical repair rarely results in normal valve

clinical manifestations of blood flow in infants

• faint pulses

• hypotension

• tachycardia

• poor feeding (decreased CO)

clinical manifestations of blood flow in children

• exercise intolerance

• chest pain

• dizziness when standing for long periods of time

treatment of aortic stenosis

• balloon dilation

• surgery; aortic valvotomy or replacement

• mortality high in NB, low for older kids

aortic stenosis and physical activity

• activity level is restricted in children even though chances of sudden death is very true

• children are not on bedrest but activity level is restricted

• curtail strenuous physical activities

pulmonic stenosis

defect involves narrowing or constriction of valves if the pulmonary artery interfering with right outflow

• obstruction to blood flow

pulmonary atresia

extreme for of pulmonic stenosis; total fusion resulting in no blood flow to lungs

clinical manifestations of pulmonic stenosis

• may be asymptomatic

• some have mild cyanosis or CHF

• murmur

treatment of pulmonic stenosis

• balloon angioplasty

• surgery

summary of acyanotic defects

• shunting from left to right

• increased pulmonary congestion

• monitor for CHF

  • tachypnea

  • diaphoresis

  • eating problems

  • edema

  • rales, crackles

cyanotic heart defects

• tetralogy of fallot

• tricuspid atresia

• transposition of great vessels

• hypoplastic left heart

• truncus arteriosis

tetralogy of fallot, tricuspid atresia

decreased pulmonary blood flow

transposition of great vessels, hypoplastic left heart, truncus arteriosus

mixed blood flow

cyanotic defects

caused by defects that result in decreased pulmonary blood flow

• pressure is greater on pulmonic side so blood shunts L → R

• mixed oxygenated and deoxygenated blood flows to systemic circulation resulting in hypoxia

symptoms of cyanotic heart defects

• cyanosis

• polycythemia

• digital clubbing

• altered ABGs

general interventions for cyanotic heart defects

• provide good skin care

• supplemental oxygen

• monitor for and prevent dehydration

• developmentally appropriate preparation for tests and procedures

nursing considerations for cyanotic defects

• alteration in oxygentation

• anxiety caused by cyanosis

• dehydration

• prevention and accurate assessment of respiratory infections

tetralogy of fallot

combination of four defects (pulmonic stenosis, right ventricular hypertrophy, ventricular septal defect, overriding aorta

• decreased pulmonary blood flow

clinical manifestations of tetralogy of fallot

• heart murmur with a thrill

• polycythemia

• hypoxic episodes (squatting position)

• metabolic zcidosis

• poor growth

• clubbing

• exercise intolerance

therapeutic management of tetralogy of fallot

• improved quality of life and longevity with surgery done in stages

  • blalock-taussig shunt

  • VSD repair

  • pulmonary valvotomy

guidelines for hypercyanotic spells

• employ calm, comforting approach

• knee chest position

• 100% oxygen by face mask

• give morphine

• IV fluid replacement and volume expansion if needed

• repeat morphine if needed

tricuspid atresia

failure of tricuspid valve to develop resulting in no communication between right atrium and ventricle resulting in severe right hypoplasia or absence of right ventricle

• decreased pulmonary blood flow

clinical manifestations of tricuspid atresia

• cyanosis

treatment of tricuspid atresia

• prostaglandin E to maintain ductus arteriosus

• digoxin and diuretics

• palliative surgical repair to increase pulmonary blood flow

transposition of the great arteries

pulmonary artery arises from left ventricle and the aorta arises from the right ventricle; no communication between systemic and pulmonary circulations

• mixed blood flow

• must have associated defect that permits blood mixing to be compatible with life

s/s of transposition of great vessels

• increasing cyanosis as foramen ovale and ductus arteriosus closes

treatment of transposition of great vessels

• arterial switch procedure in the first few weeks of life

• IV prostaglandin E to keep ductus arteriosus open and/or balloon atrial septostomy to increase mixing of blood by opening the atrial septum

truncus arteriosus

pulmonary artery and aorta fail to divide during embryonic development; one single large vessel empties both ventricles

• mixed blood flow

clinical manifestations of truncus arteriosus

• cyanosis

• CHF

• heart murmur

treatment of truncus arteriosus

• surgical repair during first few months of life

• digoxin and diuretics

hypoplastic left heart syndrome (HLHS)

aortic valve atresia, mitral atresia or stenosis, small or absent left ventricle, sever hypoplasia of ascending aorta and aortic arch; underdevelopment of the left side of the heart

• mixed blood flow

• descending aorta receives blood via PDA

s/s of hypoplastic left heart syndrome

• cyanosis

• weak peripheral pulses

• cool extremities

• respiratory distress

• often no murmur

therapeutic management of HLHS

• prostaglandin E to keep PDA

• fontan procedure - direct blood flow to pulmonary artery from RA

• norwood procedure - anastomosis of main PA to aorta, shunt from RV to PA

• transplant

complications of heart surgery

• CHF
  dysrhythmias

• cardiac tamponade (outside of heart fills with fluid)

• cerebral edema 

• brain damage

• hemorrhage or anemia

s/s of cardiac tamponade

• atelectasis

• pneumothorax

• pulmonary edema

• pleural effusions

discharge planning after heart surgery

• wound care

• medication teaching

• bacterial endocarditis prophylaxis

• when to call practitioner

• self limit activity

• meet developmental needs