Congenital Flashcards
What is SINUS OF VALSALVA ANUERYSM?
aneurysm of one of the sinus’, appears as a thin dilated area that projects into adjacent cardiac structures often with a fistula
Draw out what a SINUS OF VALSALVA ANEURYSM would look like & in the view you would see it in
PSSAX AV
NCC - into RA
LCC - into LA
RCC - into RV
What is a CORONARY AV FISTULA?
rare congenital anomaly where there is an abnormal communication from coronary artery to coronary sinus
When does a CORONARY AV FISTULA present?
in young adults as continuous murmur
What is seen on ECHO of a CORONARY AV FISTULA?
- receiving vessel/chamber & proximal coronary artery may be dilated
- may see echolucent entro site
- may see abnormal color flow into vessel/chamber
- may see disturbed color flow in fistula
What is EBSTEIN ANOMALY?
one (usually septal) or more of the TV leaflets are adhered to RV endocardium
Draw out what EBSTEIN ANOMALY would look like
Define LEVOCARDIA
apex towards left
Define DEXTROCARDIA
apex towards right
Define SITUS SOLITUS
normal position of thoracic and abdm organs
Define SITUS INVERSUS
organs of chest and abdm are arranged in a perfect mirror image reversal of the normal position
Define & Draw out NORMAL POSITION SITUS SOLITUS
normal position of thoracic and abdm organs with apex to left
Define & Draw out SITUS INVERSUS WITH DEXTROCARDIA
organs of chest and abdm switched with apex to right
Define & Draw out SITUS SOLITUS WITH DEXTROCARDIA
normal position of thoracic and abdm organs BUT apex to right
Define & Draw out SITUS INVERSUS WITH LEVOCARDIA
organs of chest & abdm switched BUT apex to left
what is the FIRST and SECOND most common congenital defect in adults?
- bicuspid AV
- ASD
What are the types of ASDS?
- secundum
- primum
- sinus venosus
What is a SECUNDUM ASD and DRAW it out
defect at the central section of IAS at fossa ovalis
Name a type of SECUNDUM
patent foramen ovale
What is a PFO and DRAW it out
lesser form of ASD and it’s failure of the foramen ovale to close
What is a PRIMUM ASD and DRAW it out
ASD inferior to secundum, located near atrioventricular connection
What is a SINUS VENOSUS?
defect at the base of LA, superior and posterior near junction of SVC and RA
A Sinus Venosus may be associated with what?
- PAPVR (partial anomalous pulmonary venous return)
- TAPVR
What is the most common form of ASD and what is it associated with?
-secundum
-MVP
PRIMUM ASD is associated with what abnormalities?
abnormalities of the atrioventricular valves like:
1. AV canal defect
2. cleft (divided) AMVL
What are the ECHO FINDINGS for ASDs?
- color jet across atrial septum into RA
- RAE, RVE,PSM due to increased volumes
- L - R shunt bc LA pressure > RA
- may have IAS aneurysm
- “T” artifact in apical
What are DOPPLER FINDINGS for ASDs?
- PW location, CW velocity, Qp/Qs severity
- low velocity flow; slightly faster in diastole due to pressure
What are the TYPES of VSDs and WHERE are they located?
1.membranous/perimembranous - membranous septum
- muscular/trabecular- any location in the muscular portion of the septum
- inlet - below AV between TV and MV annulus
- supracristal/outlet - RVOT portion of septum
What are the ECHO FINDINGS for VSDs?
- LAE, hyperdynamic LV or LVE due to LV volume overload
- color jet LV to RV
- PISA may be seen on left side of IVS if signif.
What can lead to PHTN in regards to VSDs?
large shunts
What ECHO findings would you suspect if there is PHTN in VSDs?
RVH, RVE & subsequent Eisenmenger (R-L)
DRAW OUT a view you see MEMBRANOUS VSD?
DRAW OUT a view you see MUSCULAR/TRABECULAR VSD?
DRAW OUT a view you see INLET VSD?
DRAW OUT a view you see SUPRACRISTAL/OUTLET VSD?
What are you doing in terms of DOPPLER for VSDS?
- PW for location
- CW for velocity - high velocity in systole (5-6m/s)
- brief flow reversal less likely so saline not used
- color doppler is highly sensitive to signif VSDs
- QpQs not needed in adults
What is the expected velocity in VSDs?
high velocity flow (~5m/sec) during systole due to high LV systolic pressures
what is the most COMMON type of VSD?
perimembranous
what ABNORMALITY is common with perimembranous VSD?
AI
what is the SECOND most COMMON VSD?
muscular/trabecular
what is OUTLET/SUPRACRISTAL VSD associated with?
60-70% have RCC prolapse
What is a PATENT DUCTUS ARTERIOSUS?
fetal communication between descending aorta and pulmonary artery that remains open after birth
DRAW out what a PDA would look like
What are the ECHO FINDINGS for PDA?
- LAE and LVE due to chronic overload
- color flow shows red jet towards transducer in diastole in the PA
- flow and murmur are continuous
What are the DOPPLER FINDINGS for PDA?
- CW Doppler looks like PI due to normal antegrade flow through PV
- PW in DA shows holodiastolic flow reversal similar to severe AI
- shunt ratio can determine severity
What is PAPVR? And what may it be associated with?
- when one or more PV drain into RA or SVC or IVC
- sinus venosus ASD
What is AV CANAL DEFECT? DRAW it out
consists of large inlet VSD which may extend to atrial septum creating ASD and one large atrioventricular valve
AV Canal Defect is most seen in?
down syndrome
AV Canal Defect may be associated with what?
- primum
- malformation of atrioventricular valves
What is an AORTIC COARCTATION?
narrowing of the descending thoracic aorta
DRAW out how a DOPPLER of COARCTATION would appear on SSN
DRAW out how a DOPPLER of COARCTATION would appear on ABDM AO
What would show in SSN DOPPLER OF COARCTATION?
high velocity systolic flow (> 1m/sec) with persistent antegrade diastolic flow “sawtooth”
What would show in ABDM AO DOPPLER OF COARCTATION?
dampened systolic velocities (~60cm/s) with gradual upstrokes and diastolic continuation
Aortic Coarctation is often associated with what?
bicuspid AV
What is EISENMENGERS?
a R to L shunt in result of large shunt causing PHT to cause R pressures to exceed L pressures
DRAW out how EISENMENGERS would appear
right to left shunt
What is TETRALOGY OF FALLOT?
VSD, PS, RVH and override aorta
What does CCTGA stand for?
congenitally corrected transposition of the great arteries
What is CCTGA also known as?
L-transposition/ventricular inversion
What is L-TRANSPOSITION/CCTGA?
ventricular inversion where only the ventricles have switched places
With L-transposition
The RV with TV serves as what?
The LV with MV serves as what?
- systemic ventricle
- pulmonary ventricle
What makes L-transposition UNCOMPLICATED?
- pathway of blood is normal
- dx may be made incidentally in adulthood
What makes L-transposition COMPLICATED?
if associated with defects such as VSDs, PS, complete heart block and Ebstein
CCTGA can present with what?
dextrocardia
DRAW out what CCTGA would look like
What is TGA? and DRAW it out
transposition of the great arteries where Aorta originates from RV and PA from LV
TGA is also known as?
D- transposition
What occurs with PERSISTENT LEFT SVC?
- SVC drains into coronary sinus
What ECHO FINDING suggests PERSISTENT SVC?
dilated coronary sinus in PLAX
Where is the QP AND QS site for VSD?
Qp- RVOT
Qs- LVOT
Where is the QP AND QS site for ASD?
Qp- RVOT
Qs- LVOT
Where is the QP AND QS site for PDA?
Qp- LVOT
Qs- RVOT
What is BORDERLINE for QpQs?
1.5 - < 2
What is SIGNIFICANT for QpQs?
> 2
what is ENDOCARDIAL CUSHION DEFECT?
abnormal chordal attachments; consists of large VSD which may extend to atrial septum creating ASD and create one large atrioventricular valve
Endocardial cushion defect is seen in what type of patients?
down syndome