Mitral Insufficiency (M/A)
Mitral Insufficiency (MA46)
ISOLATED CONGENITAL MITRAL INSUFFICIENCY
-usually assoc w other CHD, CT dz, metab d/o
-2y/acquired MR seen p ischemia, trauma, CM, CHF, inflmy xx (myocarditis, rh fev, endocarditis, Kawsk)
Physiology:
-In ventric systole, leaflet coaptation--> seal the inlet, prevent reflux
-Mild MR --> only small part of LV SV reflux into LA, so no signif LAE or htn, good antegrade CO
-Mod-Sev MR--> larger reflux to LA--> incr LAP and LA vol and decr fwd LV CO
-Acute MR--> rapid incr LAP and pulm edema
-Ch/gradual MR--> very compliant LA so gets bigger, and venous htn, & resistance to LV ejection goes DOWN (bc easily flows backward to LA)--> early increased LV Ejection
-Big LA--> atrial thrombi & arrhythmias
-Vol return fr pulm vn and regurg vol --> incr LV EDV & ventric and annular dilation --> worsen MR
-Initially LVH to compensate, so syst fx still good
-LV becomes hyperdynamic and tachycardic to maint enough fwd CO
-THEN, latent ventric syst dysfx is masked by the reduced systemic ventric AL and low resistance MR Q, but POSTOP you will see this dysfx once you fix the MR...
-Ch MR eventually--> decr CO and LV syst dysfx, atrial/ventric arrhythmias worsening things, sometimes to the pt that LV won't recover.
Sx/Si:
-Severity d/o age & other CHD
-Adults can handle signif MR for long pd, but infant/kids get CHF Sx sooner- w/in 3yrs of Dx
--> FTT, diaphoresis, pallor, tachypnea, pna, wheeze ("cardiac asthma") bc of bronchial vns congestion --> compress small airways and big LA--> compress LMSB
-Rales bc of inspy re-expansion of collapsed alveoli is rare & ominous!
-PE:
-inc precordial activity, diffuse apical impulse
-quiet S1
-loud P2, narrowly split - if phtn
-Murmur varies
-high freq, plateau blowing or a harsh holosys murmur at apex w rad to axilla/back
-Sometimes, maximal at LSB if ant leaflet is cleft
-Low freq, apical diast murmur and S3 if worse/sev MR
-tachycardia common w Nl SBP
-if severe w low CO, then incr RR w chest hyperexpansion, wheeze bc pulm vn congestion....., & atalectasis if LMSB compression
-poor cap refill, cool limbs if incr sympathetic tone
Mitral Valve Prolapse
-Unsure of cause if isolated MVP
-?abNl myxomatous matrix of the leaflet or collagenous structure of the cords, or ? dvp abNly similar dvpl xx of Ebstein's for TV bc MV has redundant tissue and long cords
-Isolated MVP- rare cause of MR @ <1yo;
-Seen w some Marfan pt's w myxomatous leaflet changes
Dilated or Distorted Mitral Annulus
-Uncommon to have annulus dilation/distortion w/o a CT dz (Marfan/EDS)
--> thick, redundant leaflets w long cords
-Can be assoc w ch ventric enlargement- CM, big L-->R shunt; or it can be distorted bc of RV vol OD/dilation
Cleft MV Leaflet
-Rare; defect of ant leaflet
-AV septum is intact, and no LVOT elongation
-Cleft divides ant leaflet into 2 parts- partial or goes to the base of the valve; edges are thick/rolled
-cleft is directed ant'ly twd outflow septum or Ao root, unlike an AVSD
-Cleft is more post'ly directed twd the inlet septum, and located in a small common leaflet that bridges the septum
-If isolated Cleft MV, paps are usually Nl, but in some complete cleft pts, there are no accessory cords, and ant leaflet usually flails and is grossly insufficient
-Assoc w LVOTO bc of the accessory cords
-Assoc VSD is common, also ASD and TGA
-Annulus often dilated
-Less common: post leaflet xx (aka partial leaflet agenesis)- w clefts fr edge to annulus
Anomalous Mitral Arcade or Hammock Valve
-Free margins of MV are thick and rolled, and may insert directly into pap muscle
-AbNl band of fibrous tissue extends along the free margin of the leaflets--> constrains pap muscles
--> restricted leflet motion--> MR
-If it has cords, they're often fused/short and attach directly to post ventric wall
-leaflet edge can connect directly w pap muscles
-pap muscles often AbNl, small, multiple
-Leaflets are often tethered w poor coaptation
-can also have MS
Secondary MR Causes
-assoc w AVSD, CoAo, VSD, aSD, PDA, LV divertic, anomalous LCA origin fr PA, Kawasaki, Congestive/hypertrophic CM, cardiac tumor, rh and viral myocarditis, collagen dz, metabolc dz (Hurler dz), homocystinuria, CT dz (Marf/EDS)
-Usually via progle in AV contraction, mitral annulus dilation, leaflet/cord distortion, inflmn, infilitration, fibrosis, and pap muscle/LV dysfx
-eventually --> incomplete systolic coaptation/sealing
-medial pap muscle more vulnerable to ischemia bc of supply fr RCA only
ECG:
-LAE, LV dilation if signif MR
-check for atrial tachyarrhythmias w Holter
CXR:
-LAE, big LV, cephalization/engorgement of pulm veins
Echo:
-PSLA best shows enlarged annulus, valve thickening, redundancy/prolapse, insuffic coaptation
-Ap4C and SC4C show leaflet morphology, chordal attachments, mural displacement
-Check pap muscles for displaced attachments (e.g. mitral leaflets directly inserting into pap)
-PSSA and SCSA - check for cleft ant leaflet
-hard to quantify degree of MR bc of varied physiology/anatomy
-decide based onDoppler, check regurg jet length and the narrowest area of the jet or vena contracta
-Check jet location, direction, LA and LV size, pulm flow patterns
-PW can grade severity of systolic reurg Q into LA
-Regurg fraction
-May have altered MV flow pattern- E and A wave reversal = vold OD and abNl relax'n
-Pulm vn flow reversal- prolonged and w increased Amp
-LV pk syst P estimate from syst regurg jet via modified Bernoulli
Cath:
-can tell about PVR
-elevated LA, PCWP, LVEDP
-LA/PCWP a-wave amp isincreased, w loss of the x descent and rapid rise of v wave
-Grade the regurg (I-IV) w angiography at valve
Treatment:
-Moderate MR in young kids- mainly medical- diuretics, AL reducers (ACEI) +/- digoxin
-note that ACEI may mask LV dysfx
-ch LAE or a-fib should check for atrial thrombi and Tx w platelet antags/anticoagulation
-If severe congenital, postop, acquired MR and hrt failure unresponsive to Rx--> surgery
Surgery
-Infants/Kids get sx early w abNl growth/dvp, but no definitive indications set
-LV dysfx might not be apparent until postop bc it can unload into LA
-Postop LV dysfx usually improves but arrhythmias may remain
-One way to Classify:
-Nl leaflet motion
-Prolapsed Leaflets
-Restricted leaflets
Surgery for MR w Annular Dilation/Deformation
-usually assoc w CT d/o or other CHD w L or R vol OD (VSD, ASD, LVOTO, CM)
-annuloplasty +/- valvuloplasty
-Prosthetic ring to reconstruct annulus to Nl size, but it cant grow so do a rectangular resection and annulus plication w subseuqnet repair of leaflets
-Modified annuloplasty- restores valve competence and accommodates for growth
-Can use pericardium to do a limited posterior plication of the annulus, and can accommodate growth
Surgery for MR w Deficient MV Leaflet
-Cleft MV w MR
-Suture the cleft edge, +/- pericardial patch to augment the leaflet
-may also need annuloplasty
-can sometimes create a 3 leaflet MV bc of cleft, assoc w AVS
Surgery for MR w AbNl Chordal/Papillary Muscle
-May need commissurotomy if they are fused or stenotic
-Restrictive Leaflet motion: if short cords, looks like rh hrt dz, w limited mitral leaflet motion, but unliike w rh hrt dz, the cords are well delineated. Pap muscle hypertrophy adds to narrowed interchordal spaces, w subvalvar AS. Surgeon can fenestrate they paps to increase the spaces, and reimplant the cords or replace them w synthetic cords to improve leaflet mobility
-Excessive Leaflet motion: prolapse, mising cords, long cords/paps
-if absent cords--> do a rectangular leaflet resection if redundant, or replace/reconstruct the cords w teflon sutures
-fix elongated cords by spliting the pap muscle and bring the long cord into the pap muscle w sutures
-Parachute MV & Arcade MV- restrictive or excessive leaflet motion, assoc w much R
-hardest to fix...
Mitral Valve Replacement
-only small studies, w high M&M if <5yo
-No accepted indication...
...
-Replace w bioprosthetic valve (homograft or heterograft); better than mechanical vlv
-Ross II = allograft replacement w Pulm Vlv
-Bioprosthesis xx = if placed on systemic side, can degenerate quickly in ids, and is limited by kid's growth...
...