PED Cardiac Anomalies
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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?
- Chromosomal/genetic: 10-12%
- 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)
- Multifactoral: 85%
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?
- Heart murmurs
- Delayed/decreased/absent pulses
- BP differences b/t extremities
- Wide Pulse pressure
- Clubbing of fingers
- Diaphoresis (on face esp)
What are some pediatric indicators of cardiac dysfunction?
- Poor feedings
- Tachycardia, tachypnea
- Failure to thrive, poor weight gain, activity intolerance
- Developmental delays
- 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)
- Cardiac Catheterization
- 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)
- Feeding difficulties
- 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
- Bounding pulses
- 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
- Surgical 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
- Tricuspid Atresia
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?
- Corrective surgery
- 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)
- Aortic Stenosis
- Pulmonic stenosis
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
- Chest pain
- 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
- Profound cyanosis
- 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
- Difficulty feeding
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
What are the s/s of CHF in children?
- Impaired myocardial function: tachycardia, fatigue, weakness, restlessness, pale, cool extremities, decreased blood pressure, decreased UO
- Pulmonary Congestion: tachypnea, dyspnea, respiratory distress, exercise intolerance, cyanosis
- Systemic Venous congestion: peripheral and periorbital edema, weight gain, ascites, hepatomegaly, neck vein distention
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