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...

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-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...

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