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Review the anatomy and physiology of the heart.
What is preload? What factor affect preload?
Preload is the pressure that is stretching the ventricle of the heart.
(Factors affecting preload)
Rate of Return
What is after load?
After-load is the pressure that the chamber has to generate to eject blood. Also called Stroke Volume Resistance.
Which ventricle produces almost all of the systemic pressure? Which ventricle produces pulmonary pressure? Which is the higher pressure chamber? Which chamber has oxygenated blood?
Systemic = LV (Higher Press & Oxygenated Blood)
Pulmonary = RV (LWR Pressure & Deoxygenated Blood)
Summarize the valves of the heart. What principals about valves are important to remember. WHat is important to remember about blood flow?
Valves should not stick
Blood to Lungs = Blood to Body
Oxygenated & Non Oxygenated Blood should NOT mix
Summarize the Pediatric Vital Signs
What are the differences between FETAL Circulation and an adults?
(Fetal circulation Differences)
PARALLEL not SERIES
Pulmonary & Systemic Circulation NOT SEPARATE
Organs get blood from BOTH VENTRICLES
CVO = Combined Ventricular Output
PVR = High
SVR = LOW
Gas Exchange is at PLACENTA not Lungs
Review the anatomy and circulation of the fetal heart (Normal)
What is a shunt? What are the 2 shunts that exist in the fetal circulation? When does the Foramen Ovale close?
Shunt blood AWAY from the LUNGs and towards the BODY
(2 Fetal Shunts)
Foramen Ovale (RA --> LA)
Ductus Arteriosis (PA --> AORTA)
(Foramen Ovale Closes)
Around 3 months of age
What is the Ductus Venosus?
The ductus venosus in the fetus shunts about half of the blood away from the liver, via the UMBILICAL VEIN, and directly into the INFERIOR Vena Cava.
What is the purpose of Fetal Hemoglobin? What is another mechanism for holding onto oxygen in the fetus?
Fetal Hemoglobin (Hgb F) has a much greater affinity for O2 and therefore allow the fetal RBC's to hold onto much more oxygen. This is beneficial bc there is a lot of mixing of oxygenated and deoxygenated blood.
(Another O2 Holding Method in Fetus)
Lots more RBC's (Polycythemia)
What functions do the first breaths of the baby have during those first 4 to 6 weeks of life? What do 3 week old babies look like?
The early breaths DECREASE pulmonary vascular resistance.
(3 Week Old Babies)
Look a little cyanotic bc their pulmonary vascular resistance is still in the process of being lowered. This is NORMAL.
What happens to Systemic Vascular Resistance during the first 4 to 6 weeks of life?
What 2 things are involved in the closing of the FORAMEN OVALE & the DUCTUS ARTERIOSIS?
Oxygen in the environment
High Left ATRIAL Pressure (HELPS close Foramen Ovale)
If you hear a murmur in a 2 month old, what does this mean? WHat about in a 3 month old?
(2 month old murmur)
You should hear a murmur bc the Foramen Ovale and the Ductus Arteriosis have not completely closed yet.
(3 Month old with a murmur)
This is a PROBLEM bc it means the Foramen Ovale and the Ductus Arteriosis have not yet closed.
What causes problems in the fetal heart?
Open Doors where there shouldn't be (VSD/FO/DA)
Closed or narrow doors where there shouldn't be
Missing Parts (Like a Ventricle)
Parts located where they don't belong (Transposition of Great Vessels)(When the arteries cross-See PIC)
What is the incidence of congenital heart defects? Who is at added risk for defects?
(Incidence of defects)
8/1000 live births
(Added Risk For Defects)
Siblings and Offspring of patients with Congenital Defects are at risk
What are the SIGNS that something is amiss and there may be a congenital heart problem?
(These signs could mean problem)
Abnormal Prenatal Ultrasound
Abnormal HEART SOUNDS
ABnormal BREATHING PATTERNS
Poor FEEDING & WEIGHT GAIN
Define Stenosis, Atresia, Regurgitation, Hypertrophic, Dilated with respect to the newborn heart.
Stenosis = Narrowing
Atresia = Absent
Regurgitation = Leaky Valve (Insufficiency)
Hypertrophic = Thickened Muscle Walls
Dilated = INCREASED CAVITY size
What are the types of lesions (4) that may occur in the newborn heart?
Lesions that INCREASE Pulmonary Blood Flow
Lesions that OBSTRUCT Pulmonary Blood Flow
Lesions that OBSTRUCT Systemic Blood Flow
MIXING LESIONS (Truncus Arteriosis/Tricuspid Atresia)
Discuss Increased Pulmonary Blood Flow! What conditions occur as a result of a left to right shunt?
Caused by a Left to Right Shunt resulting in TOO much BLOOD going to the lungs and CONGESTING the PULMONARY VESSELS (Pulmonary Hypertension). A VENTRICULAR SEPTAL DEFECT or an ATRIAL SEPTAL DEFECT may cause a L to R Shunt! This leads to increased Preload in LEFT ATRIUM!
(Conditions that result from increased PBF)
Overloaded Chambers get (DILATED & HYPERTROPHIC)
Heart has to work harder
What are the signs and symptoms of increased pulmonary blood flow in infants?
Sweaty with Feeds
Poor Weight Gain
Tachycardia (Compensatory Mechanism)
Increased Work of Breathing
Hepatomegaly (Caused By Blood Backing Up)
Pulmonary EDEMA (Crackles)
What are the signs and symptoms of increased pulmonary blood flow in older children? What do the O2 sats look like in these older kids? What is the exception? Explain?
Lots of Colds
Pulmonary EDEMA (Crackles)
*Normal O2 Sats
(O2 Sat Exception)
Stiff lungs (Eisenmengers) which are caused by CHRONIC LUNG CONGESTION leading to blood flow changing directions. (SEE PIC)
Discuss the Patent Ductus Arteriosis. What sometimes causes it to stay open? When would we want to keep it open and what would we use to do so?
This occurs when the (tunnel) between the Pulmonary Artery and the Aorta remains open long after birth. This is normal UNLESS IT PERSISTS!
(Remains open when...)
Prostaglandin E1 will keep the DA patent so prostaglandin inhibitors like (NSAIDS) may be given to close it.
(We want this open when...)
If a patient has transposition of the great vessels the DA remaining open is what keeps them alive so we give these patients prostaglandin ANALOGS until we can get them to the OR to fix the great vessels.
What is the sequence that we follow to manage increased Pulmonary Blood Flow?
Manage medically first & allow time to grow
May Resolve on its own
Surgical Closure or Cath Lab
How do we manage increased pulmonary blood flow as a nurse?
Same as CHF
Monitor for S/S of Pulmonary Congestion
What causes obstructions to pulmonary blood flow?
(Causes of PBF Obstructions)
Cyanotic Lesions that either cause NARROWING or LEAKING
What are the common sites of lesions that obstruct pulmonary blood flow?
Main Pulmonary Artery Lesion
Right or Left Pulmonary Artery Lesions
RV Outflow Tract
What happens to the heart as a result of lesions in the pulmonary circulation obstruction pulmonary blood flow?
RV has to work Harder (Right Ventricular HYPERTROPHY - RVH)
If there is a HOLE there may be a Right to Left Shunt because of increased pressure in the RV relative to the LV due to obstruction. (MIXING of Oxy and DeOXY Blood)
What are the signs and symptoms of obstruction to pulmonary blood flow? What may sometimes happen?
Hypoxemia With Cyanosis (Sometimes)
Hypoxemia WITHOUT Cyanosis (Sometimes)
Obstruction + VSD = HYPOXEMIA (Due to R to L Shunt)
What are some important points to remember about murmurs?
Turbulent Blood Flow
May Be Blood Flow Over An OPENING
May Be Blood Flow Over A VALVE (Too Big/Too Small)
Not Uncommon at 1 Month (BC DA not closed yet)
Some are Innocent/functional
Systolic Murmurs Often Innocent
Diastolic Murmurs are BAD
Fever increased CO which may cause Murmurs
Explain Pulmonary Stenosis
Narrowing of the valve area, such that when systole occurs, the blood leaving the right ventricle has difficulty flowing through the pulmonary valve (Should Be Open but is NARROWED) resulting in a MURMUR and reduced pulmonary blood flow.
Explain Pulmonary Atresia.
Pulmonary Atresia occurs when the Pulmonary valve is not present OR is permanently closed.
(If Valve if Closed)
A ventricular Septal Defect may be present which allows the blood to get to the lungs to some degree.
If VSD is NOT PRESENT, a patent Ductus Arteriosis must be present for blood to get to the lungs.
If VSD and Ductus Arteriosis are NOT PRESENT with pulmonary atresia, the patient dies.
Explain Tetralogy of Fallot. Who had this disease? How do we fix the heart?
(Four THings Wrong With Heart)
1. Pulmonary Stenosis
2. Thickness of Right Ventricle
3. Displacement of Aorta over VSD
4. Ventricular Septal Defect (VSD)
*Shawn White Disease
(Fixing the Heart)
PATCH the VSD
WIDEN the PULMONARY ARTERY
What prevents patients with Tetralogy of Fallot from passing out? Why?
Squatting bc it increases systemic resistance causing MORE SHUNTING from L to R Ventricle
What is a MAJOR CONCERN for patient with Tetralogy of Fallot? How do we MANAGE these patients?
CLOTTING bc of POLYCYTHEMIA (Hydration is key)
(Management of Patients)
LOTS of ASSESSMENT
Will REPAIR in STAGES
Certain LESIONS are Ductal Dependent
FAMILY SUPPORT (IMPORTANT)
FAMILY EDUCATION (Important)
What are the common sites for obstruction of systemic blood flow.
LV Outflow Tract
What do obstructions to systemic blood flow do to the heart?
Blockage causes high pressure in the Left Ventricle causes it to work harder leading to LOWER Ejection Fractions and HYPERTROPHY!
*Severe blockages cause failure
What are the signs and symptoms of obstructions to systemic blood flow? What are late signs and symptoms?
(S/S may vary depending on obstruction severity)
Tachycardia (Compensatory Mechanism)
Left Ventricular Failure
Poor Perfusion (Feel those pulses)
Organ Damage (Hypo perfusion)
Explain coarctation of the aorta.
Narrowing of the aorta that causes obstruction to systemic blood flow!
Explain Hypoplastic Left Heart Syndrome
Occurs when parts of the left side of the heart do not develop properly such as the aorta, mitral valve, underdeveloped left ventricle. In most cases the aorta and the left ventricle are much smaller than normal.
How are obstructions to systemic blood flow managed? What procedures are performed for a Coarctation of the Aorta.
(Coarctation of the Aorta)
How is hypoplastic left heart syndrome corrected?
Norwood Procedure (See Pic) - A series of surgeries that bypasses the left ventricle
What are Mixing Lesions? How much hypoxemia is there?
Lesions where oxygenated and deoxygenated blood mix at the level of the ATRIA, VENTRICLES, or GREAT ARTERIES.
(Amount of Hypoxemia)
Depends on how much (Pulmonary Blood Flow) blood actually getting to the lungs!
What are examples of Mixing Lesions?
Tricuspid Atresia (Pic)
Hypoplastic Left Heart Syndrome
Double INLET of LEFT VENTRICLE
How is blood flow to the lungs accomplished when patients have mixing lesions?
(Blood Flow to the Lungs)
Discuss Truncus Arteriosis
A condition in which only one blood vessel leaves the heart instead of 2 and its called the truncus arteriosis
What are the signs and symptoms of Congestive Heart Failure in Kids! What causes these signs and symptoms?
Poor weight gain
Slow and sweaty Feeds
Gallop or diastolic rumble
Cardiac enlargement (xray)
(Causes of S/S)
Dilated heart chambers and vascular congestion
What causes excessive fluid retention in CHF?
Decrease in Renal Blood Flow due to low Cardiac Output leads to the activation of the Renin Angiotension Aldosterone System which causes salt to be absorbed at the kidneys thus holding onto water!
*Point is to return more blood to the heart (Venous)
Why does the sympathetic nervous system become stimulated during CHF?
Fight or Flight is a compensatory response to poor cardiac output. (The point is to redistribute to poor output so important organs get blood)
What are the cardiac conditions that predispose one to CHF?
Left to Right Shunts
Heart Muscle Problems
What is the Pharmacological Management of CHF?
Diuretic (Lasix, Aldactone, Diuril)
ACE Inhibitors (Captopril, Enalapril)
What are the non pharmacological management techniques for CHF? When would you NOT want to give O2?
Supplemental O2 (Occasionally)
Packed Red Blood Cells (Occasional)
(Don't Give O2)
If BIG Left to RIght Shunt Exists (Worsens pulmonary overcirculations)
What are the acyanotic/increase pulmonary blood flow defects of the heart?
ASD Atrial Septal Defect
VSD Ventricular Septal defect
PDA patent ductus Arteriosis
What are ASD Atrial Septal defect manifestations?
asymptomatic in less severe cases.
May develop CHF (least likely), characteristic murmur, poor feeding. Risk for: atrial dysrhythmias, pulmonary vascular obstructive disease. Later in life: emboli formation.
Left to right shunting/Increase pulmonary blood flow
-increased volume on Right
-decrease systemic blood flow
Formed in early development.
Left to right shunting/ increase pulmonary blood flow
Most common defect
What are clinical manifestations of VSD
risk for: bacterial endocarditis and pulmonary vascular obstructive disease
Important to keep ductus open until VSD repaired
Left to Right Shunting/Increase pulmonary blood flow
artery connecting the aorta/pulmonary artery does not close. should close within the first 15 hours of life.
What are some clinical manifestations of PDA?
asymptomatic or show signs of CHF,
machinery-like murmur (sounds like a friction rub), widened pulse pressure, bounding pulses
Risk for: bacterial endocarditis, pulmonary vascular obstructive disease
What are some mixed flow (Cyanotic) defects?
Hypoplastic Left heart
Transposition of the great vessels
total anomalous Pulmonary Venous Connection
Hypoplastic Left Heart
small left ventricle
patent foramen ovale-must stay open to sustain life
What are some manifestations of Hypoplastic left heart?
appear during first week of life
cyanosis, CHF when PDA begins to close-leads to cardiovascular collapse.
Fatal if not fixed...use prostaglandin E1
Transposition of Great Vessels
Mixed blood flow
no communication with circulation
septal defects must be present for mixing of blood
Prostaglandins are given to maintain PFO
What are some clinical manifestations of transposition of great vessels?
cardiomegaly, cyanosis, depressed at birth
Total Anomalous Pulmonary Venous Connection
Mixed blood flow
Pulmonary veins attach somewhere other than the left atrium.
must have PFO to survive
What are the mixed blood flow (cyanotic) defects of the heart?
Hypoplastic left heart (or right)
Transposition of the Great Vessels
Total Anomalous Pulmonary Venous Connection
What are the decreased pulmonary blood flow (Cyanotic) heart defects?
Tetrolagy of Fallot
TOF Tetrology of Fallot
4 defects: VSD, PS, overriding aorta, Right ventricular hypertrophy
Right to left shunting of blood
NOT treated with ventilation
What are some manifestations for TOF?
acutely cyanotic at birth
others may have mild cyanosis that progressively gets worse
blue/tet spells: bend knees to chest (Morphine)
Anoxic spells during crying or feeding
Risk for: emboli, sudden death, seizures
no opening from right atrium to right ventricle
must keep ASD and VSD open
What are some manifestations of tricuspid atresia?
newborn: cyanosis, may be tachycardia/dyspnea
older child: chronic hypoxemia, with clubbing
risk: bacterial endocarditis, brain abscess and stroke
treat with protaglandins
What are the obstructive (cyanotic) heart defects?
coartication of aorta
Coartication of Aorta
increase pressure to the upper extremities
decrease pressure to the lower extremities
What are some manifestations of Coartication of the aorta?
infants: CHF, deteriorate rapidly-PICU, severly acidodic, hypotensive
older child: dizziness, headaches, fainting, epistaxis, HTN
risk for: HTN, ruptured aorta, aortic aneurism or stroke
hypertrophy biggest concern
What are some clinical manifestations of Aortic Stenosis?
infants: decrease cardiac output, faint pulses, hypotension, tachycardia, poor feeding
older child: exercise intolerance, chest pain, dizziness, characteristic murmur
risk for: bacterial endocarditis, cardio insufficiency, ventricular dysfunction
narrowing of pulmonary artery
right ventricular hypertrophy
decrease pulmonary blood flow
What are some clinical manifestations for PS pulmonic stenosis?
May be asymtomatic, mild cyanosis or CHF
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