What is the difference between a congenital heart disease and acquired heart disease?
Congenital: anatomic, resulting in abnormal function
Acquired: through infection, autoimmune response, environmental factors, or familial tendencies
What is the most common CHD?
What is the major cause of death within the first year of life?
The most common CHD s Ventricular Septal Disease
CHD is the major cause of death within the first year of life
Management is primarily surgical
Most causes of CHD cannot be identified and 1/3 of kids never experience handicaps
What are the most common causes of CHD?
Maternal or environmental: 1-2% (Fetal alcohol syndrome leads to a 50% chance of anomaly along with Rubella. Other viruses include cytomegalovirus, toxoplasmosis, and others)
How does the fetal heart change after birth?
Foramen Ovale closes within the first few hours or days of life
The clamping of the umbilical cord functionally stops the ductus venosis and then it atrophies
Ductus arterisosis persists for 15-30 hrs after birth
How does the cardiovascular system adjust to extrauterine life?
O2 exchange and waste removal goes from placental responsibility to a function of the lungs
Lungs fully aerated by 2 wks of life
Aorta no longer receives
What basis assessments are done at birth to check for CHD?
General appearance and color
Approp height and weight on growth chart
Assess for clubbing of the fingers
Assess resp status
Periph pulses and presence of heart murmurs
BP of all extemities
What are the clinical manifestations of CHD?
BP differences b/t extremities
Wide Pulse pressure
Clubbing of fingers
Diaphoresis (on face esp)
What are some pediatric indicators of cardiac dysfunction?
Failure to thrive, poor weight gain, activity intolerance
Positive prenatal history (exposed to infection, virus, teratogens during pregnancy)
Positive family history of cardiac disease
How is CHD diagnosed?
MRI (for structural defects)
Pulse oximetry (should be >94%)
Hematologic testing (hyperoxia will fix a pulmonary problem, but not a cardiac)
Chest xray (for enlarged heart)
What common medications are used for CHD?
Digoxin (Lanoxin): Do not give to infant if HR <90-110 or young child <70 bpm. Sweet syrup placed in buccal pocket in back of mouth-brush teeth or risk with water afterwards. Monitor for signs of toxicity and hypokalemia. Desired therapeutic level is around 2nmol/L
Furosemide (Lasix): loop diuretic that can waste potassium and sodium
Propranolol (Inderall): Beta blocker
Spinonolactone (Aldactone): potassium sparing diuretic, watch for hyperkalemia
Enalapril & Captopril: ACE inhib
What parent teaching is important with digoxin use?
Proper administration and taking hr prior to admin
Do not skip or double doses, even if missed
Do not repeat dose if infant vomits
Signs of toxicity include nausea, vomiting, poor feeding, lethargy, bradycardia and skin rash
Which CHD disorders cause increased pulmonary flow (shunting from left to right)(acyanotic disease)?
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosis (PDA)
Atrial Canal Defect (ACD)
What are the s/s of ASD?
Right atrial enlargement (may lead to right ventricular hypertrophy)
Pulmonary HTN-SOB on exertion, may have recurrent resp infections
Fixed split heart sound due to right ventricular overload
Increased risk of embolism & stroke
How is ASD treated?
With digoxin & Diuretics
Cardiac cath closure across defect if it does not close on its own
When does VSD occur during fetal development?
between the 4-8 week of gestation
What are the s/s of VSD?
Often asymptomatic with heart murmur (loud)
Systolic thrill in left sternal border
Recurrent Resp Infec
CHF is common
What complications will VSD cause? Who is VSD very common in?
Right ventricular hypertrophy
Pulmonary HTN leading to a decrease in pulmonary compliance and stiffening of the lungs leading to ineffective ventilation
*VSD is the most common CHD and is common in Down Syndrome and Fetal Alcohol Syndrome
*may close on its own within first year, if not, it may be surgically patched by cardiac cath
What are the s/s of a patent ductus arteriosis? What risks are involved?
May have a systolic heart murmur sounding like a washing machine
"Wet" breath sounds
Apnea or resp difficulty
Poor Feeding with diaphoresis
Poor weight gain or growth
Excessive fluid weight gain
Prolonged cap refill
*high risk of bacterial endocarditis
How is PDA treated?
Digoxin to decrease load on heart
Ibuprofen or Indomethicin to necrose the intima of the ductus arteriosis and encourage closure
How are ASD and ACD different?
Also common in down syndrome, atrial canal defect occurs both at the foramen ovale and where the atrium and ventricles meet
What nursing assessments will reveal disorders of increased pulmonary flow?
Many disorders are not visible at birth
Listen for persisting heart murmurs
S/S of CHF
FTT or abnormal water weight on growth chart
Family assessment for risk factors
Which CHD disorders cause decreased pulmonary flow (shunting from right to left)(cyanotic disease)?
Tetrology of Fallot
What is the tetrology of fallot?
Third most common legion
Combination of CHDs:
Pulmonary stenosis or obstruction
Overriding Aorta into VSD space leading to mixing of oxygenated blood to body
Secondary right ventricular hypertrophy due to restrictive blood flow
What are the s/s of the Tetrology of Fallot?
Tet spells (cyanotic or pink)
Increased cyanosis with irritability and crying
Clubbing of fingers
Poor growth and failure to gain weight
Describe Tet spells and how they are treated
Sudden, marked increased in cyanosis, syncope, and can lead to hypoxic brain injury
Put the child in a knee to chest position to promote circulation to trunk
Calm, comfort, and provide support
Admin 100% O2 by mask
IV fluid replacement to avoid stroke by polycythemia
Morphine subcut for agitation, but watch for resp depression
Continuous Prostaglandin E Drip: keeps PDA open by vasodilation. This increases pulmonic and systemic blood flow
What is tricuspid atresia? What are the s/s of tricuspid atresia?
Tricuspid valve is defective or missing
Common along with ASD and VSD
Decreased Pulmonary flow leads to chronic hypoxia, cyanosis, shortness of breath and slow growth
How is tricuspid atresia treated?
Prostaglandin E drip to maintain patent ductus arteriosis
May need heart transplant in serious cases
Which CHD disorders are considered obstructive defects?
Coarctation of the aorta (COA)
What s/s are associated with Coarctation of the Aorta (COA)? What are the risks involved?
Increased BP in head and upper extremities, decreased BP and pulses in lower extremities
Systolic ejection murmur
Decreased growth, weight gain, devel delays
S/S of CHF
*Increased risk of Aortic Rupture due to high pressure
*Common in Turner's Syndrome
How is COA treated?
CHF treated with digoxin and diuretics
Balloon dilation and stent placement by cardiac cath
What are the s/s of aortic stenosis?
Hypertrophy of the left ventricle and CHF
Systolic ejection murmur
Narrow pulse pressure with decrease in systolic pressures
Exercise intolerance which may lead to sudden death
What are the s/s of Pulmonic stenosis?
May be in conjunction with other CHD such as Tet of Fallot
How are aortic and pulmonic stenosis treated?
Cardiac Cath for balloon angioplasty or valvuplasty
Which CHD disorders cause mixing of the oxygenated and deoxygenated blood?
Transposition of the great vessels
Total anomalous pulmonary venous connection
Hypoplastic heart syndrome (either right or left sided)
What is the transposition of the great vessels, what are the s/s and how is it treated?
The pulmonic valve leads to the aorta and the aortic valve leads to the lungs
This sends deoxygenated blood to the brain and can cause serious damage
Murmur may be present
PDAs and VDS will be kept open to promote mixing of oxygen (with prostaglandin E or surgery)
Treated by surgically changing the arteries to their appropriate places
What is a total anomalous pulmonary venous connection? What are the s/s? How is it treated?
Pulmonary vein goes back into the right atrium, mixing oxygenated and deoxygenated blood
Increases pressure in right atrium
Once the PDA closes, profound cyanosis, severe chock and CHF results
Treated with prostglandin E to maintain PDA, corrective surgery or transplantation
What is hypoplastic heart syndrome?
Second most common CHD
Underdeveloped left side of the heart and valves lead to pulmonary congestion and edema
Symptoms appear when the PDA closes
What are the s/s of hypoplastic heart syndrome?
Skin ashen in color
Rapid onset difficulty of breathing
What is the prognosis for hypoplastic heart syndrome and what is the necessary treatment?
Usually fatal within the first days or months of life
Prostaglandin E to keep PDA open for surgical repair, but the only real treatment is a heart transplant