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66 Cards in this Set

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Incidence of CHD:
8-10/1000 live births in US

-Leading cause of mortality due to congenital defect
% of all deaths in 1st year of life due to CHD:
10%
3 classes of CHD:
1. L->R shunt
2. Cyanotic (r->L shunt)
3. Obstructive
4 types of CHD w/ L-R shunts:
1. VSD Ventricular Septal Defect
2. ASD Atrial Septal Defect
3. ECD Endocardial Cshion Defect
4. Patent Ductus Arteriosus
4 types of CHD w/ R-L Shunts:
1. TOF Tetralogy of Fallot
2. TGV Transpsn of Great Vessels
3. HLH Hypoplastic Left Heart
4. PS/PA Pulm Stenosis/Atresia
What is the main problem that results from a L-R shunt?
The right side gets overloaded with blood, which overloads pulmonary circulation.
What progression occurs when Pulmonary circulation is overloaded?
Pulmonary Hypertension
Right heart hypertrophy/failure
Shunt reversal
What characterizes a R-L shunt?
Cyanosis - the baby turns blue
What causes cyanosis?
Increased unoxygenated Hb greater than 1/3 total Hb conc.
Obstruction of bloodflow out of left heart will result in:
1. Pulm circulation pressure overload
2. Pulm venous hypertension
3. Left heart failure
Right heart obstruction causes
Hypoxia
How are HLH and PS/PA classified?
EITHER Cyanotic OR obstructive
What are 3/4 of CHD related to?
Cardiac mesenchyme formation (cushion tissue) so PDA, VSD, ASD.
What is the majority of CHD caused by?
90% - UNKNOWN/multifactorial
Most common major CHD? What %?
Ventricular Septal Defect VSD
-25% of all CHD
Where is the L-R shunt most commonly located in VSD?
Membranous Interventric Septum (80% of the time)
-Can be muscular though
What usually happens if it is in the Muscular IVS?
50% close spontaneously
Who is VSD more common in?
Males
Most common type of ASD:
Secundum type
What is Secundum type ASD?
Patent foramen ovale - allows blood to go from LA to RA
What are the 3 types of ASD?
-Secundum
-Primum
-Sinus venosus
What is the result of ASD in 20-25% of cases?
A probe patent Foramen ovale, but not clinically significant b/c it is not functional.
What is the ridge around fossa ovalis?
The septum secundum
What is the floor of Fossa ovalis?
Septum Primum
In what sex is ASD more common?
Females
What is the mildest form of ECD?
Bifid mitral valve
What is the most severe form of ECD?
Common AV valve
What percent of all CHD is ECD?
2%
What disease is ECD associated with?
Down syndrome (60% of them have ECD)
Why is ECD so bad?
L-R shunting occurs at both the ATRIAL and VENTRICULAR levels
Where is a Patent ductus arteriosus located?
A shunt between the aorta and pulmonary trunk
What does the ductus arteriosus normally become?
The ligamentum arteriosum
What is the ductus arteriosus formed FROM?
6th Aortic Arch
What is the frequency of PDA? In what sex is it more common?
-1/2000 live births
-Females
What can close the PDA?
cox inhibitors
4 Allos of the Tretalogy of Fallot:
1. Stenosis of Pulm Trunk/valve
2. VSD
3. Aorta overriding L/R Ventricles
4. Hypertrophy of R Ventricle wall
What makes a Pentology of Fallot?
Adding in an ASD
Most common type of cyanotic heart defect:
Tetralogy of Fallot
Where is the infundibular region?
The outflow from the right ventricle to the pulmonary trunk.
What happens to the infundibular region in Tetralogy of fallot?
It may narrow and sometimes not even be open.
What percentage of all CHD is Tetralogy of Fallot?
6%
What is TGV?
Switching of the great vessels:
-Aorta connects to RV
-Pulm Trunk connects to LV
What MUST happen in order for infants with TGV to survive?
A shunt must be created.
What sex is TGV more common in?
Males
What is the problem in Persistent Truncus Arteriosus?
No partitioning of the Truncus and Aortic Sac occurs; a single vessel empties both ventricles.
What disease is PTA associated with?
Digeorge's
What is the cause of hypoplastic left lung?
Absence of filling during development
What is the absence of filling caused by?
Mitral valve stenosis or atresia or foramen ovale closed too soon.
What is necessary for extrauterine survival in HLH?
Creation of a shunt between L/R heart
What is the unique symptom of Coarctition of the Aorta?
-Differential pulses in the extremities:
Upper limb is strong
Lower limb is weak
With what disease is CA associated?
Turner's syndrome in girls
What sex is coarctition of the aorta more common in?
Males
What abnormality is CA commonly associated with?
Bifid aortic valve
What is Aortic Stenosis?
Narrowing of the aortic valve
What is Aortic Atresia?
Absence of the valve
What can result from aortic stenosis?
Left heart failure
What sex is Aortic stenosis more common in?
Males
What are the 2 obstructive CAD associated with the left heart?
-Coarctition of the Aorta
-Aortic stenosis/atresia
What Obstructive CAD is associated with the right heart?
Pulmonary stenosis/atresia
When will cyanosis occur in Pulmonary Stenosis/Atresia?
Only if a R-L shunt is present
What are 3 additional anomalies of the heart?
-Acardia
-Cardiac Malrotation
-Ectopic cardia
What is the distinct feature of Acardia?
Unique to Monozygotic Twinning
What happens in Cardiac malrotation?
Looping to the left so the apex is on the right side of the thorax.
What is Cardiac malrotation also called?
Dextrocardia
What is the term for organreversal in both the thoraxand abdomen?
Situs inversus
What is Ectopic Cardia?
Heart located on outer surface of thoracic cavity.