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Congenital Heart Defects

Terms in this set (32)

When do congenital heart defects usually arise?
During embryogenesis (usually weeks 3-8)
How common are congenital heart defects?
Seen in ~1% of live births
Defect in the septum that divides the R & L ventricles, most common congenital defect.
VSD

(Ventricular Septal Defect)

*If VSD is small, can be asymptomatic. If VSD is large, large amounts of blood can cross over to RV (L --> R shunt), which increases the blood volume in the pulmonary circuit --> eventually results in pulmonary HTN. Eventually, the pulmonary HTN will be severe enough that blood entering RV from RA will prefer to pass directly into the LV, instead of through the high pressure pulmonary circuit ( = REVERSAL OF THE SHUNT (R --> L)). When you have reversal of the shunt, you get deoxygenated blood entering systemic circulation --> CYANOSIS!
VSD is associated with ...
Fetal Alcohol Syndrome
Large VSDs can lead to _______________.
Eisenmenger Syndrome

*With this, pt will have RVH (because RV is now pumping against a much higher pressure)

*You can also get polycythemia, because when shunt reverses (R --> L) you get deoxygenated blood entering the systemic circuit --> hypoxemia --> release of EPO to stimulate production of RBCs

*You can also get clubbing of fingernails as a result of the cyanosis
The Tx for small VSDs is ...
Small defects may close spontaneously.
The Tx for large VSDs is ...
Surgical Closure
Defect in the septum that divides the L & R atria, most common type us ostium secundum, ostium primum type is associated with Down syndrome
ASD

(Atrial Septal Defect)
ASD results in a _____________ shunt, with a ______________ on auscultation.
L --> R shunt, Split S2 on auscultation

*L --> R shunt means more blood volume on R side of heart --> delayed closure of the pulmonic valve --> results in splitting it S2 (sound when aortic and pulmonic valve close)
A patient with a DVT in the context of an ASD is at increased risk for ____________.

This is specifically called a ____________.
Stroke, Paradoxical Embolus

Normally, if an embolus broke off of a DVT it would travel through the right heart and get lodged in the lungs, however, is the pt has an ASD, it can cross from the RA --> LA then travel to LV --> Aorta --> Carotid --> Brain = STROKE
Failure of the ductus arteriosus to close is called _________.
PDA

(Patent Ductus Arteriosis)

The Ductus Arteriosis is fetal connection between the Left Pulmonary Artery and the Arch of the Aorta. Usually closes at birth.

*Initially, the PDA will result in blood from Aorta shunting back to Pulmonary Artery (high --> low pressure). Over time, increased amounts of blood in pulmonary circuit will lead to pulmonary HTN, which will cause the SHUNT TO REVERSE, so now deoxygenated blood will shunt from pulmonary artery --> aorta ==> CYANOSIS of lower extremities

*Reversal of this shunt is called: EISENMENGER SYNDROME (it results in lower extremity cyanosis)
PDA is associated with ...
Congenital Rubella
PDA is asymptomatic at birth, but is associated with a _________________ murmur.
Holosystolic 'machine-like'
Do PDAs arise before or after the major branches off of the aortic arch?
After
What is the Tx for PDA?
Indomethicin

Indomethicin decreases Prostaglandin E (PGE). PGE keeps the PDA open, therefore decreased PGE should result in PDA closure.
What are the 4 characteristic problems in Tetralogy of Fallot?
1. Stenosis of RV outflow tract
2. RVH
3. VSD
4. Aorta that overrides the VSD
Babies born with Tetralogy of Fallot will be born ________.
Cyanotic

*Because blood passes directly from RV to Aorta via a VSD

*Degree of R --> L shunting depends on the degree of stenosis of the Pulmonary Artery (if really tight = more shunting, if loose = less shunting)
Patients with Tetrallogy of Fallot can become very cyanotic, especially with exercise. How can they reverse this cyanosis?
Squatting down

*When they squat down, they are increasing the pressure on the aortic/left heart side (increased arterial vascular resistance), which will decrease the R --> L shunt, therefore sending more blood to through the pulmonary circuit --> better oxygenation --> less cyanosis
Describe the classic CXR finding in Tetralogy of Fallot
'Boot-shaped' heart on CXR
Pulmonary artery arises from LV and Aorta arises from RV is known as ...
Transposition of the Great Vessels

*This results in 2 independent (pulmonary and systemic) circuits that do not mix --> presents with early cyanosis

*The only way for a baby to survive after birth, is for use to create an opening between these circuits
Transposition of the Great Vessels is associated with ...
Maternal Diabetes

HIGH YIELD!
What is a treatment approach to deal with Transposition of the Great Vessels?
In order for baby to survive, you must create an opening between the two separate circuits:

1. Can give PGE to keep PDA patent until definitive surgical repair is performed

*Creation of shunt after birth is required for survival
Single large vessel arising from both ventricles, because Truncus failed to divide
Truncus Arteriosus
Truncus Arteriosus results in _________.
Early Cyanosis

Blood leaving both RV and LV go to same main trunk so some deoxygenated blood goes to Aorta

(Deoxygenated blood from RV mixes with oxygenated blood from LV before the pulmonary and aortic circulations separate)
Tricuspid valve orifice fails to develop, right ventricle is hypoplastic, associated with ASD resulting in R --> L shunt, presents with early cyanosis
Tricupid Atresia

*If tricuspid valve fails to form, you can't send blood into RV. In response to that, RV becomes hypoplastic

*Ticuspid Atresia is classically associated with an ASD, so there is an opening, which allows blood to cross over.
Narrowing of the aorta.

Divided into infantile and adult forms.
Coarctation of the Aorta
INFANTILE Coarctation of the Aorta is associated with a ________.
PDA

*Coarctation lies distal to the aortic arch, but proximal to the PDA.

Presents as lower extremity cyanosis (because coarctation makes aorta distal to the narrowing a low pressure environment, therefore blood passes from pulmonary artery to the aorta via PDA. Deoxygenated blood entering the systemic circuit past the major vessels of the aorta = CYANOSIS OF LOWER EXTREMITIES!
INFANTILE Coarctation of the Aorta is commonly found in patient's with ________.
Turner Syndrome
Not associated with a PDA, Coarctation lies distal to the aortic arch, Presents as HTN in UE and HoTN with weak pulses in LE, classically discovered in adulthood
ADULT Coarctation of the Aorta
ADULT Coarctation of the Aorta is associated with a ...
Bicuspid aortic valve
In ADULT Coarctation of the Aorta, collateral circulation will develop across the intercostal arteries in order to try to bypass the block. This will result in engorged arteries causing what finding on CXR?
"Notching of the ribs"
"Notching of the ribs" on CXR secondary to ADULT Coarctation of the Aorta
THIS SET IS OFTEN IN FOLDERS WITH...