Devo + Clinical Flashcards
what is associated with ectopia cordis? and what is?
anterior thoracic wall fails to close properly. assoc w pentalogy of cantrell: EC, VSD, sternal cleft, diaphragmatic hernia, omphalocele
secondary heart field allows formation of?
primitive RV, outflow region, primordial atrium, AV canal, sinus venosus
what is the precursor tissue of the heart?
splanchinic mesoderm
what is precursor additions is looping driven by?
secondary heart field
what are the tissues used for heart partitioning?
cardiac muscle, cardiac mesenchyme, extracardiac mesenchyme, neural crest mesenchyme
structure: coronary sinus
derived from?
left horn of sinus venosus
structure: crista terminalis
derived from?
right valve of sinus venosus
structure: valve of IVC
derived from?
right valve of sinus venosus
structure: valve of CS
derived from?
right valve of sinus venosus
structure: smooth area of RA
derived from?
absorption of sinus venosus, primarily the right horn
structure: IVC (terminal portion)
derived from?
right vitelline vein
structure: valve of SVC
derived from?
right common cardinal & proximal anterior cardinal vein
structure: valve of appendage (auricle) (L and R)
derived from?
primordial atrium
structure: definitive LA
derived from?
absorption of pulmonary veins and primitive left atrium
structure: smooth part of LA
derived from?
absorption of the pulmonary veins
how is common AV canal expanded?
myocardialization: cushion tissue into cardiac muscle
what does division of common AV canal depend on?
formation of cardiac mesenchyme (cushion tissue). epithelium to mesenchyme transformation (endocardial cells transform into cardiac mesenchyme cells which move into the cardiac jelly).
what structures are formed from cardiac mesenchyme?
valve leaflets, chordae tendinae and fibrous skeleton of the heart
embryonic primordia: proximal outflow region
adult derivative: ?
ventricular outlets (R & L)
embryonic primordia: distal outflow region
adult derivative: ?
pulmonary and Ao valves, and pulmonary and Ao root
embryonic primordia: aortic sac
adult derivative: ?
pulmonary and aortic root
aortic sac partitioning occurs due to what tissue type?
neural crest-derived mesenchyme that forms the aorticopulmonary septum
what components form the membranous IVS?
conotruncal septum + endocardial cushion, fuse with muscular IVS
structure: inlet of RV
derived from?
AV canal
structure: apical trabeculated component of RV
derived from?
primitive RV
structure: outlet of RV
derived from?
proximal outflow region
structure: inlet of LV
derived from?
AV canal
structure: apical trabeculated component of LV
derived from?
primitive LV
structure: outlet of LV
derived from?
proximal outflow region
what cell types come from cardiogenic mesoderm (3)
endocardial endothelial cell, atrial myocyte, ventricular myocyte
what other cell type is derived from endocardial endothelial cells?
cushion cell
what other cell type is derived from ventricular myocytes?
purkinje fibers
what cell types are derived from cardiac neural crest cells?
aortic smooth muscle (and poss pulmonary vessels), and neurons
what cell types are derived from proepicardium?
coronary smooth muscle, endothelial cells, fibroblasts
pharyngeal arch artery 3 developmental fate?
proximal: common carotid arteries
distal: internal carotid arteries
pharyngeal arch artery 4 developmental fate?
left: part of aortic arch
right: proximal right subclavian artery
pharyngeal arch artery 6 developmental fate?
left, proximal: proximal left pulmonary artery
left, distal: ductus arteriosus
right, proximal: proximal right pulmonary artery
right, distal: degenerates
what are the 3 fetal shunts? where to where?
ductus venosus: O2 rich blood from placenta around liver
foramen ovale: blood in IVC from R atrium to L atrium
ductus arteriosus: blood from RV into aorta (bypassing left heart)
what types of gene products are affected with CHD?
transcription factors, signaling proteins & receptors, helicase-binding protein
what are the L to R shunts? are they cyanotic?
ASD, VSD, AVSD, PDA. late cyanotic
what are the obstructions? are they cyanotic?
pulmonary stenosis, aortic stenosis, coarctation. acyanotic.
what are the R to L shunts? are they cyanotic?
Tetralogy of Fallot, Transposition of the Great Arteries, Truncus arteriosus, TV atresia, TAPVR
what is the valvular regurgitation associated with CHD?
ebstein
clinical consequence of ASD?
potential pulmonary hypertension and right-sided failure
clinical consequence of PFO?
paradoxical emboli, decompression sickness, migraines possibly
long term effects of ASD?
RVH and dilation, RA and LA dilation
murmor with PDA?
harsh, continuous, machinery like murmur
what other anomalies are AVSDs associated with?
mitral and tricuspid valve anomalies
what is a partial AVSD?
primum ASD and cleft MV with MR
what is a complete AVSD?
AVSD and common AV valve
what genetic syndrome is associated with complete AVS?
down syndrome (40%)
clinical symptoms of R to L shunts
early cyanosis. paradoxical emboli and decompression sickness (clots or gas bubbles not filtered by lungs). digital clubbing. polycythemia.
findings of tetralogy?
VSD, subpulmonary stenosis, overriding aorta, RVH
CXR of tetralogy?
boot shaped heart d/t RVH
gross findings of tetralogy?
small PA
definition of transposition of the great arteries?
aorta arises from RV. pulmonary artery arises from LV.
sequelae of TGA?
aorta anterior and to R of PA. separate pulmonary and systemic circulations. RVH. pulmonary hypertension develops unless pulmonary stenosis is present.
2 types of TGA?
stable: with VSD
unstable: with intact VS (need surgery)
truncus arteriosus definition?
origin of aorta and pulmonary artery from truncal artery. + most have large VSD
etiology of truncus?
developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery
sequelae of truncus?
mixing of blood, increased pulmonary blood flow, pulmonary HTN, cyanosis
what other syndrome is truncus associated with?
DiGeorge
what does tricuspid atresia result from?
unequal division of the AV canal (MV enlarged)
what defects must be present also?
ASD/PFO and VSD
what does tricuspid atresia cause?
RVH
what is TAPVR (total anomalous pulmonary venous return). why does it occur?
pulmonary veins do not directly drain into LA; left atrial hypoplasia. d/t common pulmonary vein failure of development
how does ASD/PFO help?
allows oxygenated blood to enter systemic circulation
what are 2 types of aortic coarctation?
preductal/infantile (tubular hypoplasia with PDA); postductal/adult (ridgelike infolding at ligament without PDA)
what is the definition of pulmonary stenosis?
PV obstruction d/t hypoplasia, dysplasia, or abnormal number of cusps
results of isolated PV stenosis?
RV dilation and hypertrophy. post-stenotic injury to PA.
what occurs with PV atresia with intact VS?
hypoplastic RV and TV. PDA needed to get blood to lungs.
long term changes d/t PS?
pulm artery dilation, RVH & RA dilation
isolated aortic valve stenosis results in?
LVH and LA dilation
AS murmur type?
systolic murmur
what is hypoplastic left heart syndrome due to?
aortic valve atresia with intact VS. need PDA for survival.
what is an ebstein anomaly?
inferiorly displaced and adherent septal and posterior leaflets of tricuspid valve. redundant anterior leaflet. dilated annulus with tricuspid regurgitation.
secondary effects of ebstein anomaly?
RV and RA dilation
other features of ebstein anomaly?
arrhythmias (including WPW). may be asymptomatic until adulthood.
