TRICUSPID REGURGITATION
-Ebstein's is #1 cause for congen TR
-other causes are usually bc of a VSD patch misplacement or bc of phtn, espec in pts w an RV vol overload or RV diln
Ebstein's Anomaly
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-5 relavent anatomic characteristics
-septal and post leaflets are displaced apically
-ant leaflet is attached ath correct level, at annulus, but it is larger than Nl, w multiple cord attchmts to ventric wall
-segment of RV fr the annulus to level of attchmts of septal and post leaflets is thinned, dysplastic = atrialized, w very dilated RA
-RV cavity is small, lacking inlet, and has a small trabecular part
-infundibulum is often obstructed by redundant tissue of ant leaflet, and by cordal attchmts of the ant leaflet to the infundibulum
-Carpentier classification (ref 38)
-Type A = true RV volume is adequate
-Type B = large atrialzied part of RV, but ant leaflet moves freely
-Type C = ant leaflet very restricted in movement, +/- --> obstruction to RVOT
-Type D = almost complete atrialization of the RV, except for a small infundibular part; only communication bn the atrialized RV and the infundibulum is thru the anteroseptal commissure of the TV
-Assoc anomalies
-ASD in 42-60% pts (ref 39)
-PDA in neonates w sev Sx
-WPW in 10%
-VSD, TGA, TOF, malformed MV also seen
-cc TGA
-noncardiac - low set ears, micrognathia, cleft lip/palate, absent L kidney, megacolon, undesc testes, bilat ing hernias (ref 40)
Path
-ref41- very high rate of in utero death
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Dx
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Medical Tx
-PGE if needed
Surgical Indications
-older kids/adults- surg for NYHA3-4, progressive cyanosis, or arrhythmias
-few data to guide need for/timing of surgery in infant/neonate
-indication if pt fails off PGE, needs ventilation, is acidotic etc
NHx- ref 40- 70% rate of survival to 2 yrs and 50% survival to 13 yrs old
-15% survival to 2 yrs if assoc anomalies present
-but if pt survives beyond early childhood, they do well
-ref 39- n=505, 73% bn 1-15yrs old, 69% bn 16-25yo, and 59% of pts >25yo had minimal sx
--> early dx alone isnt an indication, ASx pt may do very well
Surgical Mgt
Neonates
-their experience w sick neonates is that palliation w ASD closure or BT shunt nearly always fails
Infants, kids, adults
-...
-plicate atrialized part of RV fr apex twd base, so displaced leaflets lie at a more approriate level, close ASD, and plicate redundant RA wall
-Valve replacement- may cause complete HB, so place it above cor sinus...
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Postop mgt
-minimize PVR
Surgical Results
-ref 48- 189 pts bn 172-1991 w surgery- 58% repair, 36% replace, 5% Fontan
-6% hosp mortality
-93% were NYHA 1-2 at 1yr f/u
-10 late deaths
-signif improved exercise late postop
-ref 56- 2003study in Paris of 191 pts fr 1980-90s, repair in 187 pts
-60 had ancillary bidirectional cavopulm shunt too
-4 vlv replacement
-9% hosp mortality- RV failure in 9 of the 18 pts who died
-82% 20yr survival
-80% had 1 to 2+ TR
-reop in 16 pts- 10 w a repair, 6 w replace
-ref 57- 2002 study fr OK- n=87 Sx neonates
-TV repair, reduction atrioplasty, RVOTO relief
-1 hosp death --> repair is possible...
TRICUSPID VALVE STENOSIS
Very rare to have isolated TS, nearly always hypoplastic annulus, w small R heart, usually w pulm atresia intact septum...
-TS can be seen w TGA some times, w RVOTO and arch hypoplasia...