unbalanced avc: when is it time to bail?az9194.vo.msecnd.net/pdfs/120401/05.47.pdf · pccc: all...

Unbalanced AVC: When is it

Time to Bail?

David M. Overman

Division of Pediatric Cardiac Surgery

The Children’s Heart Clinic

Chief, Division of Cardiovascular Surgery

Children’s Hospitals and Clinics of Minnesota

A Disclaimer

When is it time to bail?

A Disclaimer


I DON’T REALLY KNOW.

A Disclaimer


I DON’T REALLY KNOW.

(and neither do you)

Unbalanced AVSD

Precise diagnosis

– When does AVSD become unbalanced?

The Challenges of UAVSD

Proper selection of surgical strategy

– When must biventricular repair be abandoned?

uAVSD: BVR or UVR?

uAVSD: BVR or UVR?

“Discordant pursuit of BVR�is more frequent

than discordant pursuit of UVR, likely driven

by an intuitive notion that ‘two ventricles are by an intuitive notion that ‘two ventricles are

better than one’. Discordant BVR is more costly

in terms of survival than discordant UVR.”

Hickey EJ, et al. JTCVS 134(6), Dec 2007.

BVR or UVR : Is uAVSD the same as

“borderline left heart”??“borderline left heart”??

Background

Uncommon

Paucity of outcomes data

Nuanced surgical strategies

Unbalanced AVSD


Variable and complex anatomy

Multiple important comorbidities

uAVSD Literature

n = 19

BVR only

Long axis ratio (LAR): LV/RV

3 early failures (death, OHT)

3 late reoperations (event free survival 56% @ 10 years)

uAVSD Literature

n = 38n = 38

32 BVR, 6 SVR

AVVI (RAV/LAV) and ratio RV length/LV length

Four early deaths (3 BVR)

Six early reoperations (All BVR)

No late follow-up data

Background

Uncommon



Unbalanced AVSD




Anatomic Considerations

Aortic arch obstruction

Systemic and pulmonary venous anomalies

VSD morphology

Complex valve morphology

Atrial septal malalignment

Leaflet Dysplasia & Deficiency

Courtesy of Rachid Idriss

Farouk Idriss Archive

Children’s Memorial Hospital

Anatomic Considerations

Aortic arch obstruction

Systemic and pulmonary venous anomalies

VSD morphology

Complex valve morphology

Atrial septal malalignment

Atrial Septal Malalignment

Courtesy of Rachid Idriss

Farouk Idriss Archive

Children’s Memorial Hospital

Background

Uncommon



Unbalanced AVSD




Balanced AVSD

Balanced AVSD Outcomes: Biventricular Repair

balanced complete AVSD

(STS National Congenital Database)

Discharge mortality 2.3%

Permanent pacer 1.5%

Reop for bleeding 1.3%

Neuro deficit 0.7%

Hospital LOS 14.6 days

-STS National Congenital Database Fall 2011 Harvest

Severely Unbalanced

AVSD

Severely Unbalanced

AVSD Outcomes: Norwood Palliation

not specific to uAVSD

(STS National Congenital Database)

MORTALITY

Norwood 17.1%Norwood 17.1%

BCPS 1.4%

Modified Fontan 1.3%

-STS National Congenital Database

Fall 2011 Harvest

UVR and Trisomy 21

PCCC: All Fontans (n=2853)

Fontan with Trisomy 21 (n=17)

Overall Fontan Mortality: 10%

Fontan/Trisomy 21 Mortality: 35% (p=0.001)

UVR and Trisomy 21

BVR

Trisomy 21: BVR or UVR?

Norwood @ 8d

Glenn @ 4m

Hospital stay 6m

Fontan @ 44m

CMV viral sepsis

RVEF 13%RVEF 13%

Hospice

Died @ 5 yrs

Trisomy 21: BVR or UVR?

Norwood @ 10d

Revision MBTS @ 10w

Sildenafil

BiV Conversion @ 6m

Systemic RVP, MS =15 torrSystemic RVP, MS =15 torr

Reop MS/LVOTO @ 13 m

RVP = 80+, MS = 14 torr

Diminished RV fct @ 17m

Current resp illness on vent

LVEDD

10 (z = -5.6) 21 (z = -0.65)

uAVSD: BVR or UVR?

Ventricular hypoplasia

Malalignment of Atrioventricular JunctionMalalignment of Atrioventricular Junction

Predicting BVR:

Ventricular Hypoplasia

Ventricular “competence”– Ability to sustain full cardiac output

Predicting BVR:


Ventricular “competence”

Imprecise measurementImprecise measurement– “Apex forming”

– 2D echo geometry (LV length/width)

– Volume formulae

• Echo: unreliable

• MRI: impractical

Predicting BVR:


Right Dominant Left Dominant

uAVSD: BVR or UVR?

Ventricular hypoplasia

Malalignment of Atrioventricular JunctionMalalignment of Atrioventricular Junction

AV Malalignment

Apportionment of AV valve over the underlying

ventricles

Anatomy of “inflow physiology”

AVVI

Atrioventricular Valve Index (AVVI)

– Subcostal LAO view

– Measure area of common AV valve apportioned over each ventricle

– LAVV:RAVV or RAVV:LAVV

AVVI Measurement

RAVV

LAVV

CHSS Lookback

Modified AVVI

– LAVV:Total AVV

Left DominantRight Dominant

0.5

Overman DM, et al. WJSPCHS 1(1), Sept 2008

mAVVI: Strategy & Outcome

N=305

Median =0.47

*Jegatheeswaran et al. Circ 2010;122;S209-S215

Predicting BVR: Beyond AVVI

Left Ventricular Inflow Index (LVII)

RV/LV Inflow AngleRV/LV Inflow Angle

Predicting BVR: LVII

Narrowest width into ventricle at level of AV valve

– Indexed to width of common AV valve annulus

22 pts w/ right dominant uAVSD undergoing BVR

– 4/22 died

No survivors with LVII <0.5No survivors with LVII <0.5

Predictive of survival after BVR in right dominant uAVSD

*Swast et al. Usefulness of Left Ventricular Inflow Index to Predict

Successful Biventricular Repair in Right Dominant Atrioventricular Canal.

Am J Cardiol 2011 Jan; 107(1): 103-9.

RV/LV Inflow Angle - Balanced

154°

RV/LV Inflow - Unbalanced

82°

Predicting BVR: RV/LV Inflow Angle

116 pts with right dominant uAVSD or bAVSD

Cluster analysis: 3 homogenous subgroups in strategy

and outcome

RV/LV inflow angle most sensitive discriminator of

subgroups

*Cohen et al. Echocardiographic Features Defining Right Ventricle

Dominant Unbalanced Atrioventricular Septal Defect: A Multi-Institutional

Congenital Heart Surgeons Society Study. Poster presentation. AHA Scientific Sessions,

November 2011. Manuscript in preparation

Beyond Prediction:

Growth Induction Strategies

1995 – 2005, n = 24

Staged Palliation (10), Valve “repartitioning” (9), Repair with residual (5)

All achieved BVR

Mid term survival 88%

Z Scores (n=7)

AV valves: (-1.1 to -6.5) (-2.1 to +1.8)

Ventricles: (-3.6 to -7.5) (-1.0 to +2.0)

Conclusions

Ability to predict ventricular “competence” after

BVR is limited

Malalignment of the AV junction is an important Malalignment of the AV junction is an important

factor in the viability and sustainability of BVR

Conclusions

LVII and RV/LV Inflow Angle may augment the

utility of AVVI in predicting successful BVR

Surgical strategies aimed at inducing growth Surgical strategies aimed at inducing growth

warrant further investigation but current data

supporting this approach are less than robust

Conclusions

Clarification of the interplay of these many factors

is needed to optimize outcomes in uAVSD

A prospective, multi-institutional study will be A prospective, multi-institutional study will be

required to adequately power such an

investigation

Conclusions

Unbalanced Atrioventricular Septal Defect:

A CHSS Inception Cohort Study

First patient enrolled December 2011

Unbalanced AVC: When is it

Time to Bail?

David M. Overman

Division of Pediatric Cardiac Surgery

The Children’s Heart Clinic

Chief, Division of Cardiovascular Surgery

Children’s Hospitals and Clinics of Minnesota

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