TRICUSPID VALVE ANOMALIES
-Tricuspid Stenosis- hypoplastic/thick valve leaflets w decr orifice
-Ebstein Anomaly- displacement of part of the origin of the vlv leaflets fr the AV jct into RV
-usually w malformed TV w TR, ventric dysfx
-?bc of underlying RV morph xx, so incomplete separation of the septal/posterior leaflets so they adhere to RV wall; the ant leaflet dvps separate fr the wall so isn't adherent, but becomes elongated, redundant, fenestrated --> the vlv is rotated R and ant in addition to displaced fwd
-Tricuspid Dysplasia- malformed leaflets
-again bc of undermining of the RV like Ebstein Anomaly; almost always affects septal leaflet, which forms late
-Double Orifice TV- 2 orifices; aka duplication of the TV
-bc of imperfect vlv formation by endocardial cushions; thus assoc w AVSD
-3 types: extra hole in a commisure, in a leaflet, or bc of a fibrous bridge dividing the orifice
-Double Outlet RA- if one orifice straddles the IVS
Anatomy
-septal, ant, post (inf/mural) positioned leaflets
-medial pap muscle supports the zone of apposition bn the septal and ant leaflets
-anterior pap muscle (larger) supports the zone of apposition bn the ant and inf leaflets
-inferior muscle (smaller) or multiple muscles support the zn of app bn inf and septal leaflets
-TV cords fr septal leaflet DO attach to ventric septum
Ebstein Valve:
DDx as Ebstein bc of:
-Septal & post leaflets displaced fwd
-+/- Restricted ant leaflet motion
-atrialized RV w true tricuspid annulus dilation
-+/- myocardial dysfx
-Nl TV is offset at the septal leaflet fr the ant MV leaflet, usually 0.8cm/m2 bn the vlv hinges
-so if >0.8cm/m2, then +Ebstein valve
-some might have only mild displacement, while others have thickened, dysplastic displaced septal/post leaflets w focal muscularization, and only a few chordal attachment; septal leaf might missing, or all 3 leaflets might be adherent to the ventric wall which can --> triscupid sac w/o an orifice
-Ant leaflet not displaced, but is malformed
-RV may become thinner or aneurysmal at diaph wall or RVOT; LV also abNl bc of L ward bowing of IVS
-Assoc xx- MV: cleft, parachute, double orifice
-TV annulus is incomplete fibrous ring, so muscle fr atrium to ventric --> incr risk for WPW...
Imaging
Goals:
[ ] see the TV
[ ] leaflet morph and movement
[ ] check for dysplastic thickened-rolled leaflets, short cords, adhered leaflets, underdvpd paps
[ ] chordae
[ ] pap muscles
[ ] size of the TV annulus
[ ] z-score
[ ] compare relative to MV annulus
[ ] r/o TV clefts
[ ] check for multiple orifices/sizes
[ ] Check for TS
[ ] thick, rolled TV leaflets that dome in diastole, further shortening cords w abNl attchmts
[ ] stenosing membrane within the funnel of the TV--> restricts leaflet opening
[ ] measure Vmax across TV
-Nl 0.8m/s; if >1.3m/s w/o a L to R shunt, then +TS
[ ] calculate max and mean diastolic transvalvar P gradient
[ ] Check for TR (Doppler)
[ ] TR grade of TR jet
[ ] systolic RV-RA P grad; IVC, SVC, CS flow profiles
[ ] dilated R hrt chambers and veins
[ ] Check for Ebstein's
[ ] displaced septal leaflet >0.8cm/m2
[ ] amt of septal/postal leaflet displacement & dysplasia
[ ] RV check
[ ] fractional area change, Tei index
[ ] aneurysmal dilation of RVOT, = if it's 2x Ao root size
[ ] RV thinning or dyskinesis
[ ] LV check
[ ] M mode for SF, diameters at syst/diast
[ ] Assoc lesions
[ ] DDx by r/o other things that cause TR-
-Ebstein, tricuspid dysplasia, ungaurded orifice, TV prolapse, TV annular dilation, 2y annular dilation fr phtn, CM, infarct; RV dysplasia, rh TV disease, carcinoid herat disease, CT dz, endocarditis, trauma
-espec c/s ruptured pap muscle fr ischemic necrosis in newborns
-AP4C- good to check displacement of TV septal leaflet twd apex. Often it's thick/dysplastic w impaired mvmt bc of short cords, or it is absent w just a cauliflower like remnant on the IVS
-PSLA thru RV inflow - see the ant and post (aka inf aka mural) leaflets; note the hingepoints of each leaflet
-PSSA- good to see TV ant leaflet to check its sail-like movement and bulging into RVOT
-SC4C (SA and LA) good to check TV ant and septal leaflets
-by turning transducer 30-45degrees clockwise fr a SC coronal view, you can see all 3 leaflets
-can see the ant leaflet attachments, and see any fenestrations
-TEE can show subvalvar apparatus well
-3D TEE can show post leaflet and degree of tethering
Prenatal Assessment
-Check TV in 4 chamber view
-rare to be an isolated xx
-Ebstein's more common prenatally than post, likely bc of fetal demise fr hydrops...
-check degree of TR, if + RVOTO, ratio of FO diam to Ln of atrial septum (by 4ch view), if >0.3 then it's beneficial
-Ao vlv pk velocity
-arrhythmias
-Uhl anomaly (big RV without much myocardiam/no trabeculations)
-r/o fx'l TR (fr fetal hyperthyroidism, other cause of fetal HF w 2y TV annular dilation)
-check cardiothoracic ratio
-check if + venous pulsations at umbilical vn/ductus venosus
-check for pericardial effusion
-check for hydrops
Perioperative Assessment
Pre
-success depends mainly on if the leading edge of the ant leaflet is mobile and free, if the pap muscles/cords are short, and if there are fenestrations
-check if post leaflet is big enough to allow a bifoliate repair
-(rare) check if all 3 leaflets are big enough to allow for a trifoliate repair
Intraop
-TEE to check above
-r/o double outlet TV (may impair transatrial repair)
-If +Ebsteins and TV dysplasia, then confirm Dx first, check for ASDs, check if any residual TR, TS, and ASDs, check ventric fx
-ensure RCA territory is still fx well after the RV plication...
-If TV is replaced, check for paravalvar leakes, iatrogenic membranous VSDs
Post
-early f/u to r/o effusion, check fx, check TV gradient, check TR
-80-88% pts are free from reoperation at 10yrs in large centers...
TRICUSPID VALVE ALIGNMENT ANOMALIES