Aortic Regurgitation
-very rare to have true congen AR that is isolated of other disease
Anatomy
-Primary Congenital Aortic Valve Regurgitation
-often bc of a bicuspid Ao vlv (ref 2,3)
-R leaflet freq xx- hypoplastic, partly fused w either non or L leaflet--> poor comissural support, w prolapse; over time--> Ao annulus enlarges bc of vol load on the vlv --> even more regurg.... spiral
-regurg jet also damages free edge of vlv leaflet--> thick and rolled vlv leaflet
-quadricupsid Ao vlv can also --> AR (ref 4)
-Ao Regurg post Balloon Dilation
-after initial balloon dilations, 13% had AR post cath, incr to 38% at f/u (ref 5)
-mainly bc detach R cor leaflet at ant commissure
-factors contributing to AR- anterior commissure avulsion, cusp dehiscence, simple cusp tear, central incompetence, Ca cups... RCC most often affected
-Ao Regurg assoc w VSD
-subpulm VSD- just below the belly of the RCC of Ao vlv--> jet can cause prolapse into the VSD fr Venturi effect; less likely w a PM VSD (however, bc PM VSD is more common than subPA/Ao VSD, we see AR more commonly w PM VSDs...)
-Ao Regurg 2y to SubAS
-membrane or tunnel stenosis--> Ao vlv damage--> AR, w thickened and rolled free edge so poor coaptation (ref 8)
-Ao Regurg assoc w Ao Root Dilation
-Marfan's & Ehlers-Danlos - w root dilation --> AR
-the tops of the commissural posts "are distracted" --> central AR
-may see it p TOF repair...
-Other Ao Regurg causes
-Ao LV tnnel, sinus of Valsalva aneurysm & fistul
-p bacterial endocarditis
-truncal valve...
Path & Sx
-...
Dx
-...
Medical & Interventional Tx
-no cath technique for Tx
-Rx Tx = CHF Tx...
Surgical Indications
-+Sx despite Rx
-decr in preload independent contractile indicies, or LV diln >2SD (ref 11)
-Goal in young child is to avoid Ao vlv replacement
-Longstanding AR - assoc w damage to AO vl free edge, and Ao ann diln--> harder to reconstruct, so if it seems like an easy AR repair, then c/s going sooner to prevent the long term xx of AR that complicate late repair
Surgical Mgt
-CP Bypass with moderate systemic hypothermia
-Cool slower than usual to minimize risk of early VF
-harvest a patch and fix in glutaraldehyde for 30min
-Ao Cross Clamp - apply before the hrt is at risk of distention fr bradycardia and reduced contractility
-helpful to infuse first cardioplegia dose w gentle LV massage- prevent distension, before making the aortotomy or placing the vent
-place LV vent thru R sup pulm vn
-Aortotomy transversely, and extend twd noncor sinus of Vals
-then give rest of the 1st dose of cardioplegia to each cor art ostia
-to do a repair, the Ao must have enough annular diameter to not require LV outflow enlargement, mobile cusps or cusps that can be made mobile w resetion/shaving fibrous tissue, and must have ability to achieve coaptation without -->ing stenosis
-Valvulopasty techniques (ref 5, 12-16) vary
Primary Repair
-excess fibrous tissue (often at raphe of fused commissures) - remove aggressively (shaving)--> more mobile cusp
-open fused commissures with a scalpel
-repair simple tears w simple running sutures
-if the cusps are of ok size, then can repair the RCC and NCC of bicuspid valve by resuspending the commissure w sutures that pass thru the Ao wall. Otherwise, resect the commisssure and then reconstruct the vlv.
-Prolapsed but otherwise good (competent, pliable) cusps- shorten them by ressupending the cusp to the commissure w a pledget supported suture
-If it is central AI w dilated sinus of Valsv, then do a sinus reduction plasty to reduce commiss splaying by resecting a wedge of noncor sinus, then primary close the aortotomy
Treated Aortologous Pericardial Patch Repair
-repair perforated cusps w a patch
-defic cusps, usually fr balloon induced tear w retraction of the free cusp edge- augment w a half moon shaped patch to the free edge; make it so it overlaps with the opposite free cusp edge by a few mm. But, if the opposite cusp is too far or also deficient, then do NOT extend the patch. Instead, use a patch on the other defic cusp, making the free edge slightly longer and redundant, ensure that most patches are further anchored to at least one commissure--> resuspends the leaflet
-if the native usp is very stiff/Calcified, then then partly or completely reset the cusp and replace w path (but this incr risk of calcification later)
Surgical Results
-ref 5- n=21 w Ao vlv surg mainly in 90s- no early or late deaths, mean 31mo f/u
-pericard effusion needing CT in 1 pt
-reop fr dehisced pericardial patch w much AR in 1
-1 pt needed repeat balloon dilation for recur AS 3.5 yrs post vlvplasty
-80% 3yr freedom fr reop (re-repair or replace Ao)
-?nothing found to be assoc w re-op in MVA analysis
-all pts ASx at f/u
-Echo f/u
-most had mild AR at f/u, mean LVEDD was much reduced to Nl range
-ref12-16- ok medium term results w Ao repair
-ref 17- Ross alternative has been "disappointing" at f/u