Heart

Question 1

primative ventricle folds to the R and outflow tract ends up on L –> R sided LV

Answer 1

ventricular inversion

Question 2

abnormal L-R development of some or all organs; seen in immobile cilia syndrome and Kartagener syndrome

Answer 2

heterotaxia

defect in dyenin

Question 3

complete reverse symmetry of the heart and GI organs, not fatal but may be symptomatic

Answer 3

situs inversus

situs ambiguous- reversal of some organs

Question 4

heart and GI tract are asymmetrically arranged from one another (R sided heart, left sided GI), causing problems with inflow and outflow; life threatening

Answer 4

visceroatrial heterotaxia

Question 5

chromosome 22q11 deletion, thymic aplasia, hypocalcemia

Answer 5

ToF

Question 6

associated with downs syndrome

Answer 6

persistent AV canal; ASD/VSD

Question 7

failure of contruncal ridge formation and fusion, abnormal migration of NCC

sx: pulmonary congestion, RV hypertrophy, increased R ventricular pressure

Answer 7

persistent truncus arteriosus (blood from RV and LV flow into same vessel b/c no separation occurred)

usually accompanied by VSD

Question 8

conotruncal ridges fail to spiral; abnormal migration of NCC

prognosis: 1/3 die within first year, many within first few months

Answer 8

transposition of great vessels (aorta and pulmonary a connected to wrong ventricles); complete separtion of pulmonary and systemic circulation

not compatible with life unless shunt (PDA, PTA, or VSD) is present

Question 9

not enough AV cushion to form valve; complete agenesis of valve; no communication between RA and RV

accompanied by patent foramen ovale, IV septal defects, hypoplastic RV, hyperplastic LV, PDA

Answer 9

tricuspid atresia

needs ASD/VSD or transplant

Question 10

• conotruncal ridges form off center causing unequal division of pulmonary trunk and aorta; abnormal migration of NCC skews development of AV septum
• pulmonary trunk is small, aorta is large

VSD; pulmonary infundibular stenosis; overriding aorta; RV hypertrophy; cyanosis caused by crying, fever, exercise

Answer 10

tetralogy of fallot

associated with digeorge syndrome; most common cause of early childhood cyanosis

PROVe it

Question 11

failure of AV septum fusion

pulmonary HTN, exercise intolerance, SOB, cardiac congestion, increased risk of endocarditis

Answer 11

persistent AV canal

Question 12

initial L –> R shunting d/t increased blood flow from lungs and decreased pulmonary resistance

pulmonary congestion –> RV hypertrophy –> CHF

Answer 12

ASD

RV hypertrophy causes switch to R –> L shunting, thats when cyanosis starts

2:1 prevalence in females vs males
foramen secundum defect most common

Question 13

excessive absorption of septum I or inadequate development of septum II

vs

failure of AV cushion from AV septum and DMP to fill in ostium

Answer 13

ostium II (high atrial septal defect)

ostium I (low atrial septal defect)

Question 14

faulty fusion of R/L bulbar ridges and AV cushion –> free flow of blood between R and L ventricles

starts out L –> R shunt (acyanotic) and pulmonary HTN
when pulm pressure > systemic pressure switches to R –> L shunt and cyanosis

Answer 14

VSD

most common congenital heart defects

Question 15

SL valves fuse –> RV hypoplasia

univentricular heart

Answer 15

pulmonary valvular atresia

patent foramen ovale forms as only way blood gets from R –> L
ductus arterious remains patent as only way for blood to get to lungs
better if there is VSD so blood can get over there

Question 16

hypertrophy of LV –> cardiac failure and pulmonary HTN

can be congenital, d/t infection (rheumatic fever), or degenerative with age

Answer 16

aortic valve stenosis

Question 17

atrial septum, membranous IV septum, AV and SL valves

Answer 17

endocardial cushion

Question 18

coronary sinus

Answer 18

L horn of sinus venosus

Question 19

IVC

SVC

Answer 19

IVC: posterior, subcardinal and supracardinal veins

SVC: R common and anterior cardinal veins

Question 20

trabeculated parts of atria

trabeculated parts of ventricles

Answer 20

a: primitive atrium
v: primitive ventricle

Question 21

smooth part of LA

smooth part of RA

Answer 21

L: primitive pulmonary vein

R: R horn of sinus venosus

Question 22

ascending aorta and pulmonary trunk

Answer 22

truncus arteriousus

Question 23

• 1/3 to 1/2 of blood traveling to aorta enters pulmonary a causing blood to be circulated through the lungs 2-3 x for every 1x it enters systemic circulation –> LV hypertrophy, increased pulmonary resistance and pulmonary congestion –> CHF
• blowing type of murmur
• maternal rubella

Answer 23

PDA

low O2 content stimulates prostaglandin release, which relaxes smooth muscle and keeps it open

Question 24

narrowing or aortic lumen distal to L subclavian a

sx: increased blood pressure in the upper extremities, lack of pulse in femoral artery, high risk of both cerebral hemorrhage and bacterial endocarditis,

Turner syndrome

Answer 24

coarctation of aorta

postductal: nbd d/t collateral circulation through intercostal and internal thoracic a
preductal: no collaterals, little to no blood gets to lower body or legs

Question 25

vascular ring around trachea and esophagus, may lead to esophageal dysfunction and strangulation of trachea

Answer 25

double aortic arch

Question 26

• L AA IV and L dorsal aorta completely obliterated and replaced by corresponding R sided vessels
  sx: dysphagia and dyspnea (if L subclavian a passes behind the esophagus and ligamentum arteriosum passes in front of trachea on R)

Answer 26

R aortic arch

Question 27

• R subclavian is formed by distal portion of R dorsal aorta and 7th intersegmental a.
• R AA IV and proximal R dorsal aorta obliterated

sx: dysphagia and dyspnea d/t R dorsal arch crossing esophagus

Answer 27

aberrant origin of R subclavian

interrupted aortic arch- similar + disappearance of L AA IV; digeorge syndrome

Question 28

aortic arches:
3
4
6

Answer 28

3: R/L carotids
4: R subclavian, aortic arch
6: R/L pulmonary arteries, ductus arteriosus

R/L coronary arteries from hepatic progenitor stem cells