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Congenital Heart Disease

Terms in this set (94)

causes of congenital heart disease (CHD)
-multifactorial cause (genetics and environmental)
-maternal risk factors
-family history of CHD
-association with syndromes of chromosomal anomalies
maternal risk factors for congenital heart disease
-diabetes
-alcohol ingestion
-smoking
-exposure to toxins (lithium, hormones, amphetamines)
-exposure to infections (Rubella)
CHD is associated with which syndromes or chromosomal anomalies
Down Syndrome, Trisomy 13, Trisomy 18, Noonan Syndrome, Williams Syndrome, DiGeorge Syndrome, Marfan Syndrome, Turner Syndrome
How does a congenital heart disease form?
-the heart begins to develop at conception
-it is completely formed by 8 weeks
-congenital heart defects are often a result of one of many crucial steps not happening at the right time during those 8 weeks.
fetal circulation
In the fetus, the blood that enters the heart already contains oxygen, supplied from the mother by the placenta. Only a small amount of blood goes through the lungs (which do not contain air).
How does blood bypass the lungs in fetal circulation?
1. foramen ovale
2. ductus arteriosus
foramen ovale
a hole between the right and left atria
ductus arteriosus
a blood vessel that connects the pulmonary artery and the aorta.
fetal circulation (prenatal)
-Placenta provides oxygen rich blood to the fetus so that most of the blood bypasses the lungs through the PDA & FO
-There is a high Pulmonary Vascular Resistance (PVR) and low Systemic Vascular Resistance (SVR) (therefore, blood wants to go to circulatory system as opposed to lungs)
-The High PVR forces blood to travel through the ductus & FO to deliver mom's oxygen rich blood to the brain and other vital organs
fetal circulation transition to newborn circulation
-Baby takes first breath which opens up the lungs and along with many other physiologic changes causes the PVR to decrease (less pressure in lungs)
-FO closes because of increased pressure &
PDA are stimulated to close b/c of local mediators (bypasses of lungs are closed)
-The umbilical cord is clamped and the SVR is increased so now blood is likely to take the normal path through the right side of the heart and lungs.
major concepts of blood movement through the heart
- There are major circulatory changes that are suppose to happen at Birth
- Blood will flow from High Pressure to Low pressure and will most often take the path of LEAST RESISTANCE
- After birth the SVR is HIGH and the PVR is low
general signs and symptoms of congenital heart defect
*-Respiratory Distress
-Fatigue (often seen during feedings)
-Failure to Thrive (FTT)*
-Murmur
-Cyanosis that worsens with crying
-Delayed Capillary Refill
-Diminished peripheral pulses
What determines congenital heart disease classification?
altered hemodynamic
altered hemodynamic categories used to determine congenital heart disease
-Increased Pulmonary Blood Flow
-Obstructive
-Decreased Pulmonary Blood Flow
-Mixed Blood Flow
What sort of shunt constitutes a cyanotic lesion?
usually a right to left shunt (unoxygenated blood gets to circulatory system)
What sort of shunt constitutes an acyanotic lesions?
usually a left to right shunt (oxygenated blood re-enters the right side of the heart to go through pulmonary system again)
What forms of altered hemodynamics fall into the category of cyanotic lesions?
1. decreased pulmonary blood flow
2. mixed lesions
What forms of altered hemodynamics fall into the category of acyanotic lesions?
1. increased pulmonary blood flow
2. obstructive lesions
left to right shunt
a portion of oxygenated blood is shifted from the left side of the heart back to the right side to reenter pulmonary circulation which increase the right side of the heart's workload
right to left shunt
unoxygenated blood is shunted from the right side of the heart to the left side of the heart via septal opening or vessel connection and then enters systemic circulation
defects with increased pulmonary blood flow (left to right)
1. Atrial Septal Defect (ASD)
2. Ventricular Septal Defect (VSD)
3. Patent Ductus Arteriosus (PDA)
4. Atrioventricular Canal (AVC)(CAVC)
-considered acyanotic defects
clinical manifestations of congenital heart defects with an increased pulmonary blood flow
-Congestive heart failure
+Tachycardia & Tachypnea
+Decreased UOP
+Sweating, Fatigue, weakness
+Difficulty w/ feeding
+Wt loss or poor wt gain
+Pale, cool extremities
+Cardiomegaly
+Retractions, nasal flaring, grunting, Resp Distress
+Hepatomegaly
+Ascites
+Peripheral Edema
-Murmur
-Atrial dysrhythmias
-Risk for Development of Pulmonary Vascular Obstructive Disease
atrial septal defect
-defect with increased pulmonary blood flow
-left to right shunt
-failure of the entire atrial septum to close resulting in a communication between right & left atrium
s/s for atrial septal defect
-may be symptomatic
-murmur
-s/s of CHF
-risk for atrial dysrhythmias
Under what circumstances would the medical team choose to observe atrial septal defect?
-If the patient is asymptomatic, medical team may elect to observe and the hole may close on its own.
-Depends on type of lesion, age, clinical picture
What sort of surgical treatment can one undergo with an atrial septal defect?
-Closure with a patch
-Requires full open Heart Surgery with Cardiopulmonary Bypass
What sort of non-surgical treatment can one undergo with an atrial septal defect?
Depending on the type/location of the hole, repair can be done through catheterization with Amplatzer Septal Occluder
ventricular septal defect (VSD)
-defect with increased pulmonary blood flow
-left to right shunt
-abnormal opening between Left & Right Ventricles
-frequently Associated with other Defects
s/s for ventricular septal defect
-CHF
-characteristic murmur
VSD management
20-60% will spontaneously close in first year of life
surgical repair of ventricular septal defect (VSD)
-Open heart surgery w/ Cardiopulmonary bypass
-Repaired with sutures or with Patch depending on sized
non-surgical repair of ventricular septal defect (VSD)
repair through cardiac catheterization
atrioventricular canal defect (AVC)
-defect with increased pulmonary blood flow
-left to right shunt
-Incomplete fusion of Endocardial cushions leads to a low ASD, high VSD and incomplete formation of the mitral & tricuspid valves
-Flow is completely dictated by SVR vs PVR
-Common in Trisomy 21
s/s of atrioventricular canal defect (AVC)
-Moderate to Severe CHF
-Murmur
-Cyanosis w/ crying/agitation
complete surgical repair of atrioventricular canal (AVC)
-Patch closure of septal defects & reconstruction of the AV valve
-Postoperative complication can include heart block, dysrhythmias, CHF, pulmonary HTN
complications of complete surgical repair of atrioventricular canal (AVC)
-Mortality w/ surgery is <5%
-Common to have mitral regurgitation later in life
patent ductus arteriosus (PDA)
-defect with increased pulmonary blood flow
-left to right shunt (aorta has higher pressure because it is considered left side of heart than the pulmonary arteries)
-Failure of Ductus Arterious to close in 1st weeks of life
s/s of patent ductus arteriosus (PDA)
-may be asymptomatic
-watch for CHF s/s
-murmur often heard
-excess pulmonary blood flow
medical management of patent ductus arteriosus (PDA)
use of Indomethacin (given IV)
indomethacin's use in PDA management
-Non-steroidal Anti-inflammatory
-used to initiate PDA closure
-Prostaglandin Inhibitor
-Contra-indicated if pt has active bleeding, Increased Creatinine, or Decreased UOP, Decreased Platelet count
surgical treatment of patent ductus arteriosus (PDA)
Ligation of the vessel or placement of a metal clip on the vessel
non-surgical treatment of patent ductus arteriosus (PDA)
Via heart catheterization coils can be placed to occlude the vessel
management of congenital heart defects with increased pulmonary blood flow
-increased Energy expenditure is seen in these patients because of increased workload on the Cardiac & Respiratory systems
-therefore, main goal of therapy is to minimize CHF s/s & reduce their energy expenditure
common medications given for congenital heart defects with increased pulmonary blood flow to treat CHF symptoms
-Digitalis
-Diuretics
-Captopril
obstructive congenital heart defects
1. coarctation of the aorta
2. aortic stenosis
3. pulmonic stenosis
considered acyanotic defects
cause of obstructive congenital heart defects
-an anatomic narrowing leads to obstruction of blood flow to the systemic circulation which results in:
+low cardiac output (CO)
+increased pressure in the ventricles & vessels before the obstruction
coarctation of the aorta
-considered acyanotic
-obstructive defect
-obstruction as the blood leaves the left ventricle through the aorta
-decreased CO and blood getting to the systemic circulation
-BP is increased in the left ventricle
clinical manifestations of coarctation of the aorta
-May have high blood pressure in arms
-Bounding pulses in arms and/or jugular vein distention (JVD)
-Diminished pulses in lower extremities, cool
-4 extremity blood pressure discrepancy
-Infants may experience s/s of CHF
-Older children may experience dizziness, headaches (HA), fainting
surgical repair of coarctation of the aorta
-Treatment of Choice, especially for infants < 6mo
-End to End anastomosis of the aorta or enlargement of the constricted area using a graft
non-surgical management of coarctation of the aorta
-Balloon angioplasty can be done in older infants & children
-Decision based on individual cases and where the narrowing is within the aorta
aortic stenosis
-considered acyanotic
-obstructive defect
-narrowing or stricture of the aortic valve leading to resisitance to blood flow trying to leave the left ventricle
clinical manifestations of aortic stenosis
-decreased CO, pulmonary congestion, & increased work on the left ventricle
-increased work of the left ventricle results in Left Ventricular Hypertrophy which is thickening of the heart wall & muscle
pulmonic valve stenosis
-considered acyanotic
-obstructive defect
-narrowing at the entrance to the pulmonary artery resulting in decreased pulmonary blood flow & increased workload to the right ventricle
clinical manifestations of pulmonic valve stenosis
Increase workload to the right ventricle results in right ventricular hypertrophy and right-sided heart failure
lesions with decreased pulmonary blood flow
cyanotic lesions
1. tetralogy of Fallot
2. tricuspid atresia
What four defects are present leading to tetralogy of fallot?
-VSD
-Pulmonary Stenosis
-Overriding Aorta
-Right Ventricular Hypertrophy
assessments of infants with tetralogy of fallot
-May be acutely cyanotic at birth or mildly cyanotic that worsens as the pulmonary stenosis worsens
-Do better if have a PDA
-Characteristic Murmur
-Hypercyanotic Episodes or "Tet" Spells: acute episodes of hypoxia during crying, feeding, or stooling (blue spells) where severe right to left shunting occurs
role of PDA in tetralogy of fallot
A patent ductus arteriosus (PDA) can play a very important role by providing an alternate pathway for blood to reach the lungs, allowing adequate pulmonary blood flow even in the face of very severe RV outflow obstruction. The flow across the PDA goes from left (the aorta) to right (the pulmonary artery) in this setting. Reason why PDA is necessary because a lot of un-oxygenated blood enters the aorta through the VSD; through the PDA un-oxygenated from the aorta returns to pulmonary arteries to be oxygenated by lungs before re-entering the aorta.
assessments of children with tetralogy of fallot
-Chronic hypoxemia & poor growth
-Utilize squatting position when in distress
treatment of hyper-cyanotic spells (tet spells)
-KNEE TO CHEST POSITION
-Calm the Patient
-Oxygen
-Morphine
complete repair of tetralogy of fallot
-Done in first year of life
-Done when increasing cyanosis/Tet Spells occur
Under what circumstance are palliative shunts put in place for infants with tetralogy of fallot?
done to increase pulmonary blood flow & increase oxygen saturation
What risk do infants with tetralogy of fallot run who have either a complete repair or palliative shunt placed?
post-op risk for CHF
tricuspid atresia
type of heart disease that is present at birth (congenital heart disease), in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle
congenital heart defects that lead to mixed blood flow
1. Tranposition of the Great Arteries
2. Total Anomalous Venous Return
3. Truncus Arteriosus
4. Hypoplastic Left Heart Syndrome
Tranposition of the Great Arteries
-congenital heart defects that lead to mixed blood flow
-Pulmonary artery leaves the LV
Aorta leaves the RV
-Minimal communication between pulmonary & circulatory systems so unoxygenated blood leaving the RV goes to the systemic circulation
danger of transposition of the great arteries
-severely cyanotic at Birth unless have an adequate PDA, ASD, VSD
-minimal communication between pulmonary & circulatory systems so unoxygenated blood leaving the RV goes to the systemic circulation
What is essential for the transposition of the great arteries?
-For survival it is essential to keep communication/ mixing of blood from left & right sides
-Ways to encourage the mixing of blood:
+For newborns a Prostaglandin E-1 as an IV continuous infusion to keep the PDA open
+Can do a balloon atrial septostomy to enlarge an ASD
-Atrial Switch Operation is done in first weeks of life
hypoplastic left heart syndrome (HLHS)
-Underdeveloped left side of the heart
-Must have a PDA to allow blood to go to systemic circulation, past the narrowed aorta (decrease pressure after narrowed or stenotic area; therefore, decreased pressure of aorta causes blood from the pulmonary arteries to travel to the aorta, decreasing the workload and volume that enters the left side of the heart)
clinical manifestations of hypoplastic left heart syndrome
-Mild cyanosis @ birth
-Rapid deterioration seen as PDA begins to close
-Decreased CO which leads to severe cardiovascular collapse
-Fatal in first month of life if not treated
-Prostaglandin E-1 started as soon as suspected to keep PDA patent
stages of repair done for hypoplastic left heart syndrome
-Norwood Procedure
-BT Shunt
-Hemi-Fontan or Bidirectional Glenn
-Modified Fontan
expected outcomes for patients with congenital heart disease
-Child's G & D progresses regularly
-Gas exchange will be maximized
-Workload on the heart is minimized
-Family Develops positive coping strategies
prenatal diagnostics for early diagnosis and intervention
-Prenatal care with ultrasounds
-When known CHD, arrange delivery at tertiary medical center
respiratory assessments that can be performed to identify congenital heart defect
rate
effort
saturations
cardiac assessment that can be performed to identify congenital heart defect
-rate
-rhythm
-murmur
-peripheral perfusion
-edema
-liver
How can growth and development charts be useful in identifying congenital heart defects?
growth charts and developmental assessment tools are useful in identifying if a child is experiencing failure to thrive
diagnostic tools to identify congenital heart defects
-Chest X-ray
-ECG
-Echocardiogram
-Cardiac Catheterization
-Blood counts
nursing diagnoses related to congenital heart defects
-Impaired Gas Exchange
-Altered tissue perfusion
-Risk for decreased Cardiac Output
-Altered nutrition: less then body requirements
-Risk for impaired growth & development
-Risk for Infection
-Anxiety: Ineffective family coping
interventions to manage family anxiety related to congenital heart defect
-Assess coping mechanisms regularly
-Provide resources like support groups, information
-Consider entire family
interventions to manage nutrition related to congenital heart defect
-Small, frequent feedings
-Increase calories of formula or supplement
-Soft nipple with large whole to maximize suck
-Organize care to maximize rest
interventions to promote development related to congenital heart defect
Educate family to treat child as normally as possible
interventions to decrease infection related to congenital heart defect
-Limit exposure
-Handwashing & education
-Prophylactic antibiotic use when undergoing surgery or dental procedures to prevent SBE
interventions to address altered gas exchange, perfusion, and cardiac output seen in congenital heart disease
-Promote good pulmonary hygiene
-Monitor I & O, daily weights
-Change position Q 2hrs
-Oxygen as needed
-Limit activities
-Knee to chest position for hypercyanotic spells
-Anti-failure medications
+Digitalis
+Diuretics (lasix, spironolactone)
+ACE Inhibiotrs (Captopril)
+Milrinone
What sort of health care professionals are involved in caring for children with CHD?
-RN
-Case Manager
-Cardiology
-Cardiothoracic Surgery
-Nutrition/dietician
-Child Life
-OT
-PT
-Pharmacy
-Support groups for parents & siblings
-Home Resources
Post-op management of post-op patients from procedures aimed at addressing CHD
-vital signs
-respiratory assessment
-pain management
-neuro assessment
-maximization of rest periods/ bundling of care
-monitoring of fluids
-cardiac assessments
vital signs monitored post-op from procedures aimed at addressing CHD
-Count respirations & HR for full minute
-Observe for dysrhythmias
-Temperature
respiratory assessments monitored post-op from procedures aimed at addressing CHD
-Immediately postop mechanical ventilation
-Listen to breath sounds (atelectasis, pleural effusion, pneumothorax)
-Caution with suctioning, done only as needed
pain management interventions post-op from procedures aimed at addressing CHD
-Morphine
-Versed
-Tylenol, Tylenol with Codeine
-Toradol
fluid goal for post-op patients following procedures related to congenital heart defects
monitor UOP closely for goal of 1ml/kg/hour
cardiac assessments monitored post-op from procedures aimed at addressing CHD
-Maximize rest for the heart
-Monitor for s/s infection (Bacterial Endocarditis)
What sort of signs will we use to identify good cardiac output in post-op CHD patients?
Signs of good perfusion: cap refill, skin color, warm extremeties, pulses, UOP
What could be the cause of dysrhythmias in the post-op CHD patient
Can be from electrolyte imbalance or from surgery
cardiac tamponade
acute compression of the heart caused by fluid accumulation in the pericardial cavity; restriction of the normal heart movement b/c of blood collecting in the pericardial sac
S/S related to cardiac tamponade
S/S include tachycardia, dyspnea, severe decrease or stopping of chest tube output
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