Congenital Heart Disease Flashcards
Genetically what are the most common mutations with congenital heart defects?
3
Environmental factors? 3
Which are more common in boys? 1
Which are more common in girls? 4
Commonly recognized is a
- microdeletion in the long arm of chromosome 22. Also seen in
- Down’s syndrome and
- Turner’s syndrome
- Maternal diabetes,
- ETOH,
- teratogens
- Left sided lesions more common in boys
- ASD,
- VSD,
- PDA &
- Pulmonic stenosis more common in girls.
Describe the following classifications:
1. Acyanotic CHD
- Cyanotic CHD
- Obstructive CHD
- “Pink babies”: Left to Right shunts
- “Blue babies”: Right to Left shunts
- Narrowing structures
Acyonotic Congenital Heart Disease are what?
What are the defects in this category?
3
Left to Right cardiac shunts: condition where blood from the systemic arterial circulation mixes with systemic venous blood.
- Atrial Septal Defect (ASD)
- Ventricular Septal Defect (VSD)
- Patent Ductus Arteriosus (PDA)
Cyanotic Congenital Heart Disease are what?
What are the defects in this category? 2
Right to Left cardiac shunts: Condition where the shunt allows blood to flow from the right to the left side of the heart.
- Tetralogy of Fallot
- Transposition of the Great Arteries
Obstructive congenital heart disease: What can be obstructed? 2
What defects are in this categories? 1 and 2
Obstruction to Right side of heart.
1. Pulmonic Valve Stenosis. (PVS)
Obstruction to Left side of heart.
- Coarctation of the Aorta
- Congenital Aortic Stenosis (AS)
Fetal circulation involves shunting blood between the right and left 1.____, through the 2._______ _____, and between the 3._______ _____ and 4.____ through the 5.______ _______.
The 6._______ supplies oxygen to the fetus through the 7.______ ____ to the heart.
On its way to the heart it enters the 8.____ and 9._____ ____, then toward the right atrium.
From the right atrium the blood is pushed through the 10._____ ____ into the 11.____ ______.
12.
HOw many arteries are in the umbilical cord?
Veins?
- atria
- foramen ovale
- pulmonary artery
- aorta
- ductus arteriosus
- placenta
- umbilical vein
- IVC
- portal vein
- foramen ovale
- left atrium
- Two arteries and one vein
In fetal circulation where is the blood in the left atrium from? Where does this blood move?
The blood in the left atrium is from the pulmonary veins and blood from the right atrium. This blood then enters the left ventricle.
The left ventricle pumps the blood where?
The majority of this output goes to the ____?
through the aortic valve into the aorta.
head
The rest of the output from the 1.____ _______ and the 2._____ _______ via the 3._____ _______ supplies the lower body of the fetus.
- left ventricle
- right ventricle
- ductus arteriosus
After the lower body has been perfused, the 1.________blood is returned to the fetus through the 2. _______ arteries.
- deoxygenated
2. umbilical
When does the ductus arteriosus start to close?
What about the foramen ovale?
15 hours after birth. Might take a couple weeks to close completely.
can take up to two years to close
What causes breathing to begin?
What does this reaction cause?
Umbilical cord is clamped.
This reaction causes sudden increase to the resistance of the systemic circulation.
The lungs begin to oxygenate the blood increasing the 1.___, causing a 2.______ in the pulmonary arterioles constriction and 3._______ pulmonary vascular resistance.
Pulmonary resistance 4._____ ______ systemic circulation which changes the blood flow across the ductus arteriosus to a 5.____ to ____shunt. This results in a closure of the 6._______ _____ and 7._____ _________.
- PO2
- decrease
- decreasing
- falls below
- left to right
- foramen ovale
- ductus arteriosus
What is an Atrial Septal Defect?
THree major types?
Opening in the atrial septum permitting blood between the two atrias.
- Ostium Secundum (most common)
Seen in about 10% of all CHD. - Sinus Venus (least common)
- Ostium Primum
Put the order of acynotic congenital heart disease in order of highest to lowest:
Ostium Secundum
Sinus Venus
Ostium Primum
- Sinus Venous
- Ostium Secundum
- Ostium Primum
ASD Clinical Presentation
Symptoms? 2
What will we hear on auscultation? 3
- -Most often have no cardiovascular symptoms.
- -Rarely present with congestive heart failure
- Heart is usually hyperactive with a right ventricular heave felt best at lower left sternal border.
- S2 is widely split and fixed at pulmonary area
- Crescendo-decrescendo systolic ejection murmur
How do we diagnose ASD?
3
What confirms the diagnosis?
Initial testing is usually
- chest x-ray and
- electrocardiogram.
- Echocardiography is the test of choice.
ASD treatment?
2
- Surgical
2. Percutaneous transcatheter closure
When is a Percutaneous transcatheter closure usually done?
Why would we want to do it earlier?
- Usually done between ages 1-3 years.
2. May be done earlier in children with congestive heart failure/pulmonary vascular problems
What is a ventricular septal defect?
An abnormal opening in the ventricular septum which allows blood to flow across the right and left ventricles.
When does a VSD close usually?
half usually close by age two
90% closed by age 6
What are the four types of VSD?
- Membranous defect (most common)
- Subpulmonic or Outlet defect
- AV or Inlet defect
- Muscular defect
VSD clinical presentation 2
- Small to moderate shunts usually have no cardiovascular symptoms.
- Small shunts have a loud, harsh holosystolic murmur.
VSD pts with large shunts present how?
6
- Frequent respiratory infections.
- Poor weight gain.
- Dyspnea and fatigue are common.
- Congestive heart failure develops between 1-6 months.
- Pansystolic murmur.
- Tachycardiac, tachypneic, heptaomegaly
How do we diagnose a VSD?
3
What could cause early onset cor pulmonale?
Initial
- chest x-ray and
- EKG
- Echocardiogram confirms diagnosis, location of the lesion, and size of shunt.
volume overlead in the right atrium makes it thicker
Treatment for VSD?
4
- Small to moderate shunts in asymptomatic patients do not require surgery or other intervention.
- Medical management
- Transcatheter closure
- Moderate to large shunts with left ventricular volume overload and or history of infectious endocarditis need surgical repair.- may or may not want to put on prophylactic antibiotics
What is a Patent Ductus Arteriosus?
What is it commonly associated with? 2
Normal fetal vessel that connects the pulmonary artery to the aorta.
Commonly associated with
- VSD and
- Coarctation of the aorta
What is PDA of seen in? 2
- seen often with premature infants
2. NSAIDS during third trimester
Why will PDA cause right sided heart failure?
higher pressure in aorta going to shoot through the PDA and cause higher volume in the lungs and higher volume in right heart.
VOLUME PROBLEM
PDA Clinical Presentation?
3
- Usually asymptomatic unless large
- A continuous rough “machinery” murmur accentuated late in systole at time of S2, heard at the left sternal border at the first and second interspaces.
- Fast breathing, poor growth
What complications can come from PDA? 2
- LV failure or
2. pulmonary hypertension can develop.
PDA Diagnosis?
3
- Chest x-ray- pulmonary vascular markings
- EKG
- Echocardiogram confirms diagnosis
PDA Treatment?
3
- In pre-term infants Indomethacin (a prostaglandin inhibitor) is used to close the shunt.
- Cardiac catheterization with coils or occluder devices.
- Large shunts require surgical ligation.
How do we treat cyanotic and ancyanotic defects differently?
cyanotic- keep it open
ancyanotic then you gotta close it
Tetralogy of Fallot
What are the 4 components?
4
What complications is it associated with?
3
- VSD
- Pulmonary obstruction
- Overriding aorta- aorta is over a defect thats letting deoxygenated blood into the aorta
- RVH
Associated with
- cleft palate,
- hypospadius,
- skeletal malformations.
TOF Clinical Presentation
4
- Cyanotic.
- Fatigability and dyspnea on exertion.
- Tet spells (turn blue when they cry)
- Harsh systolic ejection murmur
TOF Diagnosis?
4
- Chest x-ray: Boot shaped heart
- EKG
- Echocardiogram
- Cardiac catheterization
TOF Treatment:
Immediate? 1
Surgical? 2
Immediate
1. prostaglandins
Surgical
1. Temporary placement of
2. Blalock-Taussig shunt
Total repair
Describe the Transposition of the Great Arteries?
Also known as?
Division of the truncus arteriosus which aorta comes off from the right ventricle and pulmonary artery comes off the left ventricle
Also known as the “blue-baby syndrome”
TGA Clinical Presentation?
3
- Cyanotic
- Poor feeders
- fatigue
Diagnosis of TGA?
4
1. Chest x-ray “egg on a string” apperance 2. EKG: little help 3. Echocardiogram: usually diagnostic 4. Cardiac Catheterization Rashkind balloon
TGA Treatment?
3
- Prostaglandins to keep ductus open
- Atrial septostomy: Rashkind balloon
- Arterial switch operation usually at 4-7 days after birth
- What is Pulmonic valve stenosis?
- What is the obstruction usually caused by?
- Where are the areas of narrowing? 3
- What happens to the valve?
- Obstruction of blood flow from the right ventricle to the pulmonary artery
- Obstruction usually caused by narrowing
- Areas of narrowing:
- Thickened muscle below pulmonary valve
- Stenosis of valve itself (most common)
- Stenosis of pulmonary artery above the valve - Valve leaflets are thickened and fuse together
PVS Clinical Presentation?
4
- Usually asymptomatic
- Newborn
- Cyanotic - Older child
- Rough ejection systolic murmur and ejection click
PVS diagnosis?
2
Treatment:
MIld?
Moderate/Severe? 2
- Echocardiogram
- Cardiac Catheterization
Mild stenosis
–rarely needs intervention
Moderate-Severe
- -Require treatment with balloon valvuloplasty
- -Surgery required for more complex valves
What is Coarctation of the Aorta?
What is it associated with?
Narrowing of the aorta most commonly at the juxtaductal aorta
Associated with Turner’s syndrome
COA Clinical Presentation:
1. When does it usually present?
- Whats the cardial finding?
- What may you hear on auscaltation?
- Usually presents at age 4-10 days
- Cardinal finding is decrease or absence of femoral pulses
- Can have a blowing systolic murmur heard in the left axilla and left back that is pathognomonic
COA Diagnosis?
3
- Chest x-ray
Notching of ribs - EKG
- Echocardiogram
COA Treatment?
3
- Prostaglandins
- Primary balloon aortoplasty
- Surgery to repair defect
What is Congenital Aortic Stenosis?
What are the three types?
Obstruction of blood outflow between LV and aorta
3 types
- Valvular aortic stenosis (most common)
- Subvalvular aortic stenosis
- Supravalvular aortic stenosis
AVS Clinical Presentation?
4
- Usually asymptomatic until severe
- Usually do well until the 3rd-5th decades of life
- Mild exercise intolerance and fatigue
- Systolic ejection murmur
AVS Diagnosis? 3
Treatment?
2
- Chest x-ray and
- EKG: may show LVH
- Echocardiogram: most diagnostic
- Cardiac catheterization
- Surgical repair or replacement
Ross Procedure
-take diseased aorta, replace it with pulmonary valve. then replace pulmonary valve with a cadaver pulmonary valve
