John Brown MDJohn Brown MD

Left Ventricular Outflow Tract Left Ventricular Outflow Tract Obstructions in Patients with AVSDObstructions in Patients with AVSD

John Brown MDJohn Brown MD

Harris B Harris B SchumackerSchumacker Professor EmeritusProfessor EmeritusSection Section of of Cardiothoracic Surgery Cardiothoracic Surgery

Indiana Indiana University School of Medicine, Indianapolis, University School of Medicine, Indianapolis, ININ

DISCLOSURESDISCLOSURES

�� NNo conflicts of interest to discloseo conflicts of interest to disclose

Associated Cardiac Anomalies with Associated Cardiac Anomalies with AVSD AVSD

�� Tetralogy 6Tetralogy 6--16%16%

�� DORV 5DORV 5--6%6%

�� Double Orifice MV 0Double Orifice MV 0--3%3%

Ventricular Dominance 3Ventricular Dominance 3--20%20%�� Ventricular Dominance 3Ventricular Dominance 3--20%20%

�� LVOTO 0LVOTO 0--3%3%

Surgeons View of AVSDSurgeons View of AVSD

Incidence of Narrow LVOT in AVSDIncidence of Narrow LVOT in AVSD

Up to 75% of AVSD patients have a narrow LVOT on Up to 75% of AVSD patients have a narrow LVOT on ECHO and/or ECHO and/or CathCath

When When LVOT diameter : LVOT diameter : AscAsc.. AAoo. Diameter < 0.7 . Diameter < 0.7 (Incidence=24%) the technique of repair of AVSD may (Incidence=24%) the technique of repair of AVSD may require alteration to minimize risk of acquired LVOTOrequire alteration to minimize risk of acquired LVOTO

Up to 8% of AVSD patients will develop clinically significant Up to 8% of AVSD patients will develop clinically significant LVOTO after AVSD repairLVOTO after AVSD repair

Relative incidence of Post Op LVOTO Relative incidence of Post Op LVOTO in AVSDin AVSD

Partial > Transitional > Complete Type A > Partial > Transitional > Complete Type A > Complete Type CComplete Type CComplete Type CComplete Type C

Causes of LVOTO in AVSDCauses of LVOTO in AVSD

�� Shortened Inlet SeptumShortened Inlet Septum

�� Elongated Outlet SeptumElongated Outlet Septum

�� SeptalSeptal hypertrophyhypertrophy

�� Anterior displacement of the AortaAnterior displacement of the Aorta

�� Narrow Angle of Outlet Axis to Inlet AxisNarrow Angle of Outlet Axis to Inlet Axis�� Narrow Angle of Outlet Axis to Inlet AxisNarrow Angle of Outlet Axis to Inlet Axis

�� Adherence of Superior Bridging leaflet to deficient Adherence of Superior Bridging leaflet to deficient SeptalSeptal CrestCrest

�� Accessory Accessory ChordalChordal or Fibroor Fibro--muscular tissue in the muscular tissue in the LVOT under SBLLVOT under SBL

Goose Neck Deformity in AVSDGoose Neck Deformity in AVSD

Common ReCommon Re--operations Required after AVSD Repairoperations Required after AVSD Repair

Incidence of ReIncidence of Re--operation=20operation=20--30%30%

�� Repair or Replacement of Left AV Valve 60Repair or Replacement of Left AV Valve 60--70%70%

�� Pacemaker Procedures 10Pacemaker Procedures 10--20%20%

�� Acquired LVOTO 10Acquired LVOTO 10--20%20%�� Acquired LVOTO 10Acquired LVOTO 10--20%20%

–– May be delayed in onsetMay be delayed in onset

–– Usually progressiveUsually progressive

–– Can be recurrentCan be recurrent

FollowFollow--up of repaired up of repaired atrioatrio--ventricular ventricular septalseptal defectdefectWhat does cause reWhat does cause re--operation in AVSDoperation in AVSD

59 pts needed re59 pts needed re--op [op [ppAVSDAVSD and and cAVSDcAVSD]](re(re--op median 10 yrs. After primary surgery)op median 10 yrs. After primary surgery)

53/59 pts. Due to mitral valve regurgitation53/59 pts. Due to mitral valve regurgitation(10pts. Additional TKR)(10pts. Additional TKR)

BirimBirim O et al. Interact O et al. Interact CardiovascCardiovasc Thoracic Thoracic SurgSurg 20092009

96 pts needed re96 pts needed re--opop((reopreop median 10 yrs. {8days median 10 yrs. {8days –– 45 yrs after primary surgery})45 yrs after primary surgery})

�� 64 pts (67%) 64 pts (67%) –– mitral valve regurgitationmitral valve regurgitation

�� 24 pts. (25%) LVOTO24 pts. (25%) LVOTO

�� 21 pts. (22%) 21 pts. (22%) –– tricuspid valve regurgitationtricuspid valve regurgitationStulakStulak JM et al. Ann JM et al. Ann ThoracThorac SurgSurg 2010 2010

Indications for Intervention in Indications for Intervention in SubaorticSubaortic StenosisStenosis

�� Symptomatic patients (spontaneous or on exercise test) with a mean Symptomatic patients (spontaneous or on exercise test) with a mean Doppler gradient >= 50 mmHg* or severe AR should undergo surgeryDoppler gradient >= 50 mmHg* or severe AR should undergo surgery

�� Asymptomatic patients should be considered for surgery when:Asymptomatic patients should be considered for surgery when:

–– EF < 50%**,EF < 50%**,

–– AR is severe and LVESD > 50mm (or25mm/m2 BSA) and or EF < 50%**AR is severe and LVESD > 50mm (or25mm/m2 BSA) and or EF < 50%**

–– Mean Doppler gradient is >= 50mmHg* and LVH marked, Mean Doppler gradient is >= 50mmHg* and LVH marked,

–– Mean Doppler gradient is >= 50mmHg* and blood pressure response is Mean Doppler gradient is >= 50mmHg* and blood pressure response is

Class Level

l C

lla C

lla C–– Mean Doppler gradient is >= 50mmHg* and blood pressure response is Mean Doppler gradient is >= 50mmHg* and blood pressure response is

abnormal on exercise testingabnormal on exercise testing

�� Asymptomatic patients may be considered for surgery when:Asymptomatic patients may be considered for surgery when:

–– Mean Doppler gradient is >= 50mmHg*, exercise testing normal and surgical Mean Doppler gradient is >= 50mmHg*, exercise testing normal and surgical risk low,risk low,

–– Progression of AR is documented and AR becomes more than mildProgression of AR is documented and AR becomes more than mild

lla C

lla C

llb C

llb C

a = class of recommendation. b = level of confidence. *Doppler derived gradients may overestimate the obstruction and may neea = class of recommendation. b = level of confidence. *Doppler derived gradients may overestimate the obstruction and may need cd confirmation by cardiac onfirmation by cardiac catherizationcatherization

**See ESC guidelines on the management of **See ESC guidelines on the management of valvularvalvular heart disease. 35heart disease. 35

AR = aortic regurgitation; BSA = body surface area; EF = ejection fraction; LV left ventricle; LVEF left ventricular ejectioAR = aortic regurgitation; BSA = body surface area; EF = ejection fraction; LV left ventricle; LVEF left ventricular ejection fn fraction; LVESD = left ventricular end systolic diameter; LVH raction; LVESD = left ventricular end systolic diameter; LVH --left ventricular hypertrophyleft ventricular hypertrophy

Surgical Techniques to relieve Congenital or Surgical Techniques to relieve Congenital or Acquired LVOTO in AVSDAcquired LVOTO in AVSD

�� TransaorticTransaortic resection of a resection of a fibromuscularfibromuscular ridge or ridge or accessory accessory chordalchordal tissuetissue

�� SeptalSeptal MyotomyMyotomy & & MyectomyMyectomy�� SeptalSeptal Augmentation (Augmentation (ConvertingtingConvertingting Type A > Type C)Type A > Type C)�� Anterior Mitral leaflet augmentation (tissue Anterior Mitral leaflet augmentation (tissue �� Anterior Mitral leaflet augmentation (tissue Anterior Mitral leaflet augmentation (tissue

patch)patch)LappenLappen, , Kirklin,Deleon,Mace,StarrKirklin,Deleon,Mace,Starr�� Valve Sparring Konno/Valve Sparring Konno/RastanRastan�� Ross/KonnoRoss/Konno�� LVOT Bypass without LVOT Bypass without ApicoApico Aortic ConduitAortic Conduit

Anterior Leaflet Augmentation to Anterior Leaflet Augmentation to Relieve MR & LVOTO in AVSDRelieve MR & LVOTO in AVSD

AVB INSERTION TECHNIQUEAVB INSERTION TECHNIQUE

IU experience with LVOTO in AVSD IU experience with LVOTO in AVSD over last 35 yearsover last 35 years

�� 606 AVSDs606 AVSDs

�� 1 patient pre type C1 patient pre type C--AVSD repair AVSD repair RxedRxed with with ApicoaorticApicoaortic conduit 28 yrs. agoconduit 28 yrs. ago

�� 77 patients with acquired LVOTO & all patients with acquired LVOTO & all RxedRxed�� 77 patients with acquired LVOTO & all patients with acquired LVOTO & all RxedRxedwith with FibromuscularFibromuscular resection + M&M with 3 resection + M&M with 3 recurrencesrecurrences

�� 2 patients with AVSD and post2 patients with AVSD and post--op LVOTO op LVOTO RxedRxed with with ApicoaorticApicoaortic conduit conduit

CONCLUSIONSCONCLUSIONS

�� Operative mortality is low and longOperative mortality is low and long--term survival is relatively term survival is relatively good after AVSD repairgood after AVSD repair

�� LVOT is rarely seen by the surgeon during AVSD repairLVOT is rarely seen by the surgeon during AVSD repair

�� LVOTO is uncommon before AVSD repair (0LVOTO is uncommon before AVSD repair (0--3%) & higher 3%) & higher after (3after (3--8%)8%)after (3after (3--8%)8%)

�� LVOTO more common in LVOTO more common in pAVSDpAVSD than than cAVSDcAVSD ~8~8--10%10%

�� LVOTO less common in Downs than nonLVOTO less common in Downs than non--DownsDowns

�� LVOTO is underestimated by preLVOTO is underestimated by pre--op ECHO due to relatively op ECHO due to relatively low systemic output & large L>R shuntlow systemic output & large L>R shunt

ConclusionsConclusions

�� TransaorticTransaortic resection + M&M indicated for 1resection + M&M indicated for 1stst

occurrenceoccurrence

�� Recurrence after Recurrence after subaorticsubaortic resection is high resection is high (40%(40%))(40%(40%))

�� More More aggressive Rx needed for 2aggressive Rx needed for 2ndnd

occurrence, occurrence, ieie. . SeptalSeptal and/ or SB and/ or SB leaflet patch leaflet patch augmentation or augmentation or KonnoKonno

CautionCaution

�� Wilcox/ Nunn technique of AVSD repair Wilcox/ Nunn technique of AVSD repair increases the substrate for post repair LVOTOincreases the substrate for post repair LVOTO

My recommendation for Rx of My recommendation for Rx of LVOTOLVOTO

�� TransTrans--aortic resection of aortic resection of FibromuscularFibromuscular tissue tissue an/or anomalous an/or anomalous chordeachordea

�� For persistent or recurrenceFor persistent or recurrence-- TransatrialTransatrial take take down of SBL to look at LVOT directly, resect down of SBL to look at LVOT directly, resect down of SBL to look at LVOT directly, resect down of SBL to look at LVOT directly, resect abnormal attachments & abnormal attachments & AtollogousAtollogousGluteraldehideGluteraldehide pericardial patch augmentation pericardial patch augmentation of ventricular septum and/or SBLof ventricular septum and/or SBL

Thank youThank youThank youThank you

CONCLUSIONSCONCLUSIONS

�� Reoperation after initial repair is safe and can be performed Reoperation after initial repair is safe and can be performed with a low operative mortality rate.with a low operative mortality rate.

�� Presence malformed left Presence malformed left valvularvalvular apparatus, apparatus, pulmunarypulmunary artery artery hypertension, and moderatehypertension, and moderate--toto--severe left severe left atrioventricularatrioventricularvalve regurgitation are independent predictors of death and valve regurgitation are independent predictors of death and valve regurgitation are independent predictors of death and valve regurgitation are independent predictors of death and defectdefect--related morbidity after surgical repairrelated morbidity after surgical repair..

FollowFollow--up of repaired up of repaired atrioatrio--ventricular ventricular septalseptal defectdefect

Factors for LVOTOFactors for LVOTO

�� AnteroAntero--positioned aortic valvepositioned aortic valve

�� SeptalSeptal hypertrophyhypertrophy

�� Fibrotic tissue (native or Fibrotic tissue (native or postsurgpostsurg.).)

�� AnomalAnomal insertion of the anterior bridging insertion of the anterior bridging leafletleafletleafletleaflet

�� ProtudingProtuding mitral valve ring after valve mitral valve ring after valve replacementreplacement

5 5 –– 10% develop hemodynamic significant LVOTO during follow10% develop hemodynamic significant LVOTO during follow--upupCalderoneCalderone et al. JTCVS 2010et al. JTCVS 2010

FollowFollow--up of repaired up of repaired atrioatrio--ventricular ventricular septalseptal defect defect When to (re) When to (re) –– intervene?intervene?

�� Timing of Timing of reinterventionreintervention is affected by the leading is affected by the leading clinical symptomsclinical symptoms

�� Decision should be in concordance with the current Decision should be in concordance with the current �� Decision should be in concordance with the current Decision should be in concordance with the current (ACC) (ACC) –– guidelinesguidelines

�� The trend for earlier surgical LAVV Repair is evident, The trend for earlier surgical LAVV Repair is evident, but not proven valid yetbut not proven valid yet

Left Ventricular Outflow Obstruction in Left Ventricular Outflow Obstruction in AVSDAVSD

�� IncidenceIncidence

–– 1% if 1% if unoperatedunoperated

–– Higher incidence after operation (8.9% in CAVSD)Higher incidence after operation (8.9% in CAVSD)

�� EtiologyEtiology

–– Elongation & narrowing due to more extensive area of direct fibrous Elongation & narrowing due to more extensive area of direct fibrous –– Elongation & narrowing due to more extensive area of direct fibrous Elongation & narrowing due to more extensive area of direct fibrous continuitycontinuity

–– Short, thick Short, thick chordaechordae that anchor to the crest of ventricular septumthat anchor to the crest of ventricular septum

–– Bulging of Bulging of anterolateralanterolateral muscle bundle (muscle of the muscle bundle (muscle of the MoulartMoulart))

–– Morphologically discrete Morphologically discrete subaorticsubaortic membrane or excrescences below membrane or excrescences below aortic valve orificeaortic valve orifice

–– Abnormally positioned papillary muscleAbnormally positioned papillary muscle

CAVSDCAVSDPrevalence of Associated Cardiac MalformationsPrevalence of Associated Cardiac Malformations

SagitalSagital View of LVOT in AVSDView of LVOT in AVSD

Surgeons view of AVSDSurgeons view of AVSD

AtrioventricularAtrioventricular SeptalSeptal DefectDefectIssues with impact on long term follow upIssues with impact on long term follow up

Type of AVSD

Ventricular balance

Associated cardiac defects and other anomalies

Severity of pulmonary hypertension

ASD and VSD size, additional VSDs, LV outflow tract

AV Valve morphology: DOMV, single papillary muscle, dysplasia

AVV incompetence grade (1-4 scale)

FollowFollow--up of repaired up of repaired atrioatrio--ventricular ventricular septalseptaldefectdefect

Potential longPotential long--term problemsterm problems

�� Left Left atrioventricularatrioventricular valve regurgitationvalve regurgitation

�� Left ventricular outflow tract obstructionLeft ventricular outflow tract obstruction

�� Late onset complete heart blockLate onset complete heart block

�� Pulmonary vascular diseasePulmonary vascular disease

�� AtrialAtrial or ventricular or ventricular dysrhythmiasdysrhythmias�� AtrialAtrial or ventricular or ventricular dysrhythmiasdysrhythmias

�� Left Left atrioventricularatrioventricular valve valve stenosisstenosis

�� Right Right atrioventricularatrioventricular valve valve stenosisstenosis/regurgitation/regurgitation

�� Residual ventricular Residual ventricular septalseptal defectdefect

�� Aortic incompetenceAortic incompetence