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62 Cards in this Set

  • Front
  • Back
What does the right atrium have that the left atrium does not?
-Crista terminalis
-Pectinate muscles
What does the left atrium have that the right atrium does not?
Ostium secundum
Where do the chordae for the Tricuspid valve insert?
Into the septum
Where do the chordae for the mitral valve insert?
NOT into the septum
How many papillary muscles are there for the
-tricuspid valve?
-mitral valve?
Tricuspid = several

Mitral = two
What are the trabeculae like in the
-Right ventricle
-Left ventricle
RV = coarse trabeculations

LV = fine trabeculations
Which ventricle has an infundibulum?
RV only
Whats the difference between the AV-semilunar valves in the right vs left ventricle?
RV = valve discontinuity

LV = valve continuity
Where are the SA and AV nodes found?
In the RIGHT atrium
How is the SA node situated in the right atrium?
How is the AV node situated in it?
SA node = subepicardially

AV node = subendocardially
What is the only normal pathway for conduction from the atria to the ventricles?
Via the AV bundle
What does the bundle of His pass through?
The inferior margin of the membranous septum.
What does the right bundle branch pass through on its way to the RV free wall?
The septal and moderator bands
When do most Congenital heart abnormalities form?
During embryogenesis weeks 2-10
What is the most common congenital heart defect?
VSD
What are the top 5 congenital cardiac abnormalities?
1. VSD
2. ASD
3. PDA
4. Coarctition of Aorta
5. TOF
What 2 cardiac malformations are more common in females?
-ASD
-PDA
What 3 cardiac malformations are more common in males?
-Coarctition of the aorta
-Transposition of great vessels
-Hypoplastic left ventricle
What % of CHD has well defined genetic or environmental influences?
10%
What % have chromosomal abnormalities?
5-8%
In which chromosomal syndrome is there 90% involvement of cardiac defects? 50%? 40%?
90 = Trisomy 18

50 = Trisomy 21

40 = Turner's 45XO
What percent of cardiac defects are related to teratogenes?
2-4%
What are 3 common teratogens?
-Cocaine
-Alcohol
-Metabolic syndromes
What is the nature of inheritance for most CHD?
Sporadic
What are 5 risk factors for development of a congenital heart defect that can be detected in utero? (lowest specificity to highest)
Family history 1%
Maternal diabetes 5%
Fetal arrhythmia 10%
Major organ defect, karyotype 25%
CHD on echocardiogram duh!! 40%
How can babies survive to birth with congenital heart defects?
The defects are tolerated well in utero bc of the parallel doubly connected fetal circulation.
What CHD's don't survive in utero?
Regurgitant lesions
Why don't Regurgitant lesions survive in utero?
Because there is volume overload of the right circulation - develops into fetal hydrops.
What is fetal hydrops?
Abnormal fetal fluid balance
-GENERALIZED EDEMA
What is a way to detect fetal hydrops?
Skin thickening on fetal sonogram
Where do effusions collect in fetal hydrops?
In at least one body cavity:
-Peritoneal
-Pleural
-Pericardial
What are 5 maternal risk factors for fetal hydrops?
-Diabetes
-Hemoglobinopathy
-Rh isoimmunization
-Prior pregnancy with it
-CHD w/ failure
What are 4 lesions that are NOT considered to be congenital heart defects?
1. Bicuspid aortic valves
2. Peripheral pulmonary artery stenosis
3. Persistant PDA of prematurity
4. Mitral valve prolapse
What are the 5 chromosomal defects associated with CHDs?
Trisomy 13, 15, 18, 21
Turner syndrome (45XO)
List the 5 main steps in embryogenesis regarding the heart:
1. Specification of cardiac cell fate
2. Morphogenesis and looping of the heart tube
3. Segmentation and cardiac chamber growth
4. Cardiac valve formation
5. Connection of the great vessels to the heart
What are 2 very important transcription factors in cardiac organogenesis?
TBX5

NKX2.5
In what syndrome is TBX5 transcription factor mutated?
Holt-Oram syndrome
What are the defects found in Holt-Oram syndrome?
heart, arm, and hand
-ASD
-VSD
What does a mutaiton in only one copy of NKX2.5 result in?
Nonsyndromic ASD
What is NKX2.5 the human counterpart to?
The drosophila Tinman gene
What happens in embryonic flies if both copies of tinman gene are absent?
The heart is then absent
What are 4 CHDs that result from mesenchymal migration errors in outflow tract septation?
1. Isolate interruption of the aortic arch
2. PDA
3. Truncus arteriosus
4. TOF
What chromosomal defect causes hypoplasia of the 4th branchial arch, and 3rd/4th pharyngeal pouches?
Chromosome 22q11.2
What are the 2 main defects (in addition to CHDs) seen in Ch 22q11.2 defects?
-Immunodeficiency (thymic hypoplasia)
-Hypocalcemia (Parathyroids)
What do extracellular matrix abnormalities with cell-cell and cell-ECM junction defects cause?
-2 CHDs seen in what syndrome?
-Endocardial cushion defects
-AV septal defects
Seen in Down syndrome (trisomy21)
In what process are Situs and Looping defects important factors?
Determining Laterality
What are the 3 subsets of CHD lesions based on physiological and clinical features?
1. Left to right shunts
2. Right to left shunts
3. Obstructive malformations
What is another name for
-L to R shunts
-R to L shunts
L-R = Acyanotic

R-L = Cyanotic
What is the main pathological effect of L-R shunts?
An increase in pulmonary blood flow to 2-4X the systemic circulation
What are the 4 main L-R shunt defects?
-VSD
-ASD
-AVSD
-PDA
How do you remember the L-R shunts?
They all have a D
What is the main physiologic difference between ASDs and the other 3 L-R shunts?
ASDS = pulmonary volume increases only

VSDs/AVSDs/PDA = volume AND PRESSURE increase in pulmonary circuitry
How does the pulmonary vasculature respond to a L-R shunt, regardless of the type?
CONTRACTURE - increased resistance of the small terminal muscular arteries in the lung.
What is the hallmark of R-L shunts?
Cyanosis of skin and mucous membranes
Why is there cyanosis in R-L shunts?
Because of DIMINISHED pulmonary bloodflow, resulting in deoxygenated systemic blood.
What are the 5 types of R-L shunts?
The 5 T's
What are the 5 T's?
-TOF
-T of GV
-Truncus arteriosus
-Tricuspid atresia
-Total anomalous pulmonary veinous connection
What complication are R-L shunts prone to develop?
Thromboemboli
What can happen to thromboemboli in R-L shunts?
They can pass into the systemic circulation, causing paradoxical embolization with systemic infarcts.
What are 2 clinical signs of long standing R-L shunts?
-Polycythemia
-Hypertrophic osteoarthropathy
-Clubbing
What are Obstructive malformations?
Hypoplasia or atresia of any of the major valves or narrow areas under and above the valves.
What are the 4 obstructive malformations?
1. Coarctition of the aorta
2. Pulmonary stenosis/atresia
3. Aortic stenosis/atresia
4. Mitral stenosis w/ restrictive foramen ovale