Abnormalities of ventricular inflow Flashcards
MS, Shone Complex, MR, MVP, Cor Triatriatum, tricupid Atresia, Ebsteins, (28 cards)
What is Cor triatriatum?
Partitioning of the LA by a perforated membrane creating a dorsal and ventral
- two LA chambers
membrane may have more than one perforation
Cor triatriatum is a very rare finding
Membrane can be in the RA but is even more extremely rare.
During embryonic development what event causes Cor Triatriatum?
Incomplete absorption of the common pulmonary vein
In Cor triatriatum the Pulmonary veins will usually drain into which LA chamber
Dorsal or ventral
Dorsal/superior chamber
In Cor triatriatum which LA chamber communicates with the MV
Doral or ventral
ventral/inferior chamber
usually PFO and atrial appendage communicate with this chamber aswell
What two common shunts are usually associated with Cor Triatriatum?
- ASD- almost always 70-80%
- VSD- most cases (eval direction of shunt)
Congenital anomalies associated with Cor Triatriatum may include?
- CoA
- AV Canal
- Pulmonary Stenosis
- Depending on membrane location it can also cause pulmonary vein stenosis
- PAPVR
Cor triatriatum symptoms typically does not present till____of life
second or third week of life
Surgical repair for Cor Triatriatum involves
excising the membrane and closing ASD and VSD (if present)
Cor triatriatum post op follow-up should look for?
- Residual membrane in LA
- Residual IAS shunt
- Estimate RV pressures by TR jet/PHTN
- Eval pulmonary vein dilation
Don’t confuse Cor Triatriatum with
Mitral stenosis, MS is rarely seen in neonates
What are congenital forms of MS?
5 variations
- Parachute MV: all chordae is attached to a single papillary muscle, best seen in PSAX
- Arcade MV: papillary muscles are connected by a fibrous band or arcade.
- Double orifice MV: MV has 2 small openings
- Hypoplastic MV: MV is displasia, highly associated with multiple levels of leftsided obstructions
-HLHS
-Shones Complex (CoA, bicuspid AV, MS)
- critical AS - Mitral Atresia: absent MV, no flow across, common with HLHS
MVP is commonly associated with what syndromes?
Marfan syndrome
Ehlers-Danlos syndrome
Ebstein’s anomaly
- TV leaflets characteristics
- Effects on the RV and O2
-Downward displacement of the TV posterior and septal leaflets into the RV “arterializing the RV”.
-Anterior leaflet is usually much larger than normal.
RV is smaller than normal resulting in less blood getting pumped to the lungs. In severe cases a PDA can be crucial and become the main source of flow to lungs
What are characteristics found on echo in the presence of Ebstein’s?
Attachment of the leaflets to the RV free wall (how many)
TR
ASD;
- 80% of the time
- R-L flow / Septum bows leftward
RA dilation
paradoxial septal motion of IVS due to ^volume in RV
cyanosis due to RV failure
Assess RVOT
Jugular venous distension
Celermajer index
Ebsteins
Ratio of
RA + atrialized RV/ RV, LV, LA
Normal celermajerindex <0.50
Abnormal index >1.50 (increase early morbidity)
Ebstein appearance on Chest X-ray
Cardiomegaly with decreased pulmonary vasculature markings
What is the optimal view to see Ebsteins?
Apical 4 chamber
can be seen in multiple views but AP4 is best
Tricuspid valve displacement is greater than ____ mm?
15mm
Congenital anomalies and shunts associated with Ebstein’s
VSD
pulmonary atresia with intact septum
pulmonary stenosis (due to septal leaflet location)
Tetralogy of Fallot
CoA
PDA
Sever cases prostaglandin may be used to keep PDA open
Ebstein’s surgical repair involves
Typically closing the ASD and repairing the tricuspid valve
In severe cyanotic cases a palliative procedure may be performed with a Glenn or BT shunt, atrial septostomy, and TV repair
Post op Ebstein corrective surgery one should look for?
residual TR or TS
residual Atrial shunt
RV function
Definition of tricuspid atresia
Dense fibrous membrane covering the expected orifice of the TV,
no antegrade flow through the TV
What must be present for survival in the case of tricuspid atresia
ASD
Other congenital defects often associated with tricuspid atresia are?
CoA
TGA
HRHS
