Abnormalities of ventricular inflow

Question 1

What is Cor triatriatum?

Answer 1

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.

Question 2

During embryonic development what event causes Cor Triatriatum?

Answer 2

Incomplete absorption of the common pulmonary vein

Question 3

In Cor triatriatum the Pulmonary veins will usuall drain into which LA chamber

Dorsal or ventral

Answer 3

Dorsal/superior chamber

Question 4

In Cor triatriatum which LA chamber communicates with the MV

Doral or ventral

Answer 4

ventral/inferior chamber

usually PFO and atrial appendage communicate with this chamber aswell

Question 5

What two common shunts are usually associated with Cor Triatriatum?

Answer 5

• ASD- almost always 70-80%
• VSD- most cases (eval direction of shunt)

Question 6

Congenital anomalies associated with Cor Triatriatum may include?

Answer 6

• CoA
• AV Canal
• Pulmonary Stenosis
• Depending on membrane location it can also cause pulmonary vein stenosis
• PAPVR

Question 7

Cor triatriatum symptoms typically does not present till____of life

Answer 7

second or third week of life

Question 8

Surgical repair for Cor Triatriatum involves

Answer 8

excising the membrane and closing ASD and VSD (if present)

Question 9

Cor triatriatum post op followup should look for?

Answer 9

• residual membrane in LA
• residial IAS shunt
• estimate RV pressures by TR jet/PHTN
• eval pulmonary vein dilation

Question 10

Don’t confuse Cor Triatriatum with

Answer 10

Mitral stenosis, MS is rarely seen in neonates

Question 11

What are congenital forms of MS?

5 variations

Answer 11

• 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

Question 12

MVP is commonly associated with what syndromes?

Answer 12

Marfan syndrome
Ehlers-Danlos syndrome

Question 13

Ebstein’s anomaly
- TV leaflets characteristics
- Effects on the RV and O2

Answer 13

-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

Question 14

What are characteristics found on echo in the presence of Ebstein’s?

Answer 14

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

Question 15

Celermajer index

Ebsteins

Answer 15

Ratio of
RA + atrialized RV/ RV, LV, LA

Normal celermajerindex <0.50
Abnormal index >1.50 (increase early morbidity)

Question 16

Ebstein apperance on Chest X-ray

Answer 16

Cardiomegaly with decreased pulmonary vasculature markings

Question 17

What is the optimal view to see Ebsteins?

Answer 17

Apical 4 chamber

can be seen in multiple views but AP4 is best

Question 18

Tricuspid valve displacement is greater than ____ mm?

Answer 18

15mm

Question 19

Congenital anomalies and shunts associated with Ebstein’s

Answer 19

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

Question 20

Ebstein’s surgical repair involves

Answer 20

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

Question 21

Post op Ebstein corrective surgery one should look for?

Answer 21

residual TR or TS
residual Atrial shunt
Rv function

Question 22

Definition of tricuspid atresia

Answer 22

Dense fibrous membrane covering the exspected orfice of the TV,
no antegrade flow through the TV

Question 23

What must be present for survival in the case of tricuspid atresia

Answer 23

ASD

Question 24

Other congenital defects often associated with tricuspid atresia are?

Answer 24

CoA
transposition of the great arteries
HRHS