embrylogy and congenital heart disease Flashcards
genetic: complete AV canal
trisomy 21
genetics: Holt-oram, ASD
TBX5
genetics: conotruncal and arch anomalies
Del 22q11
double outlet right ventricle is during or after morphogenesis
during
pulm. atresia with small RV during or after morphogenesis
after
left ventricle not sufficient to support the systemic circulation leading to mitral/ aortic stenosis or atresia.
—> poor chamber growth due to inadequate flow
hypoplastic left heart syndrome
blood vessels arise from
blood islands
mesodermal derivatives induced to form
endothelial cells- VEGF
normal heart tube loping
rightward loop- D-looped ventricles
neural crest cells are important for the formation of
normal AV valves, outflow tract and arch development
endocardial cushions develop into
atrioventricular and conotruncal regions to assist with septation
atrioventricular cushions form the
crux or center of heart; atrioventricular valves and adjacent portions of atrial and ventricular septum
what forms the valve leaflets
mesenchymal tissues
muscular cords degenerate into
dense chordae tendineae
thick muscular trabeculae persist as
papillary muscles
conotruncal endocardial cushions form the
semilunar valves and ouflow tract septum
aorticopulmonary septum seperates the
aorta and PA above valves
AV canal septum is formed from the
endocardial cushions
paired series of aortic arches 1-6 with the pharyngeal arches, lead to paired
dorsal aortae
A 9 month year old girl is evaluated in the cardiology clinic for noisy breathing and difficulty swallowing solids. Echocardiography reveals a vascular ring composed of a double-aortic arch.
Which of the following is true concerning her aortic arch development?
A She had only a single dorsal aortic arch in early arch development
B Abnormal neural crest cell migration played an important role
C Incomplete aorticopulmonary septum formation is likely
D One arch did not regress
D One arch did not regress
Assuming that valvar pulmonary atresia is compatible with fetal life, which fetal shunt is most critical to that fetus’ survival to term?
A The patent foramen ovale
B The patent ductus arteriosus
C The ductus venosus
A The patent foramen ovale
fetal circulation is
parallel
failure of delamination
ebstein’s anomaly of the tricuspid vale can lead to non-compaction cardiomyopathy
A 5 year old boy with tetralogy of Fallot is having episodic hypercyanotic episodes with walking. He performs one physical maneuver that helps him. What is it?
A Valsalva maneuver
B Lies down
C Squats down
C Squats down
failure of normal septation of truncus arteriosus
transposition of the Great Arteries; D-loop TGA
