Unbalanced AVC: When is it Time to Bail?

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 When is it time to bail? I DON T REALLY KNOW.

A Disclaimer When is it time to bail? I DON T REALLY KNOW. (and neither do you)

Unbalanced AVSD The Challenges of UAVSD Precise diagnosis When does AVSD become unbalanced? 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 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??

Background Unbalanced AVSD Uncommon Paucity of outcomes data Nuanced surgical strategies 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 = 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 Unbalanced AVSD Uncommon Paucity of outcomes data Nuanced surgical strategies Variable and complex anatomy Multiple important comorbidities

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 Unbalanced AVSD Uncommon Paucity of outcomes data Nuanced surgical strategies Variable and complex anatomy Multiple important comorbidities

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

Outcomes: Norwood Palliation not specific to uavsd (STS National Congenital Database) Severely Unbalanced AVSD MORTALITY 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

Norwood @ 8d Glenn @ 4m Hospital stay 6m Fontan @ 44m CMV viral sepsis RVEF 13% Hospice Died @ 5 yrs Trisomy 21: BVR or UVR?

Trisomy 21: BVR or UVR? Norwood @ 10d Revision MBTS @ 10w Sildenafil BiV Conversion @ 6m Systemic 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 Junction

Predicting BVR: Ventricular Hypoplasia Ventricular competence Ability to sustain full cardiac output

Predicting BVR: Ventricular Hypoplasia Ventricular competence Imprecise measurement Apex forming 2D echo geometry (LV length/width) Volume formulae Echo: unreliable MRI: impractical

Predicting BVR: Ventricular Hypoplasia Right Dominant Left Dominant

uavsd: BVR or UVR? Ventricular hypoplasia Malalignment 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 Right Dominant 0.5 Left Dominant 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 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 <0.5 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 required to adequately power such an investigation

Conclusions Unbalanced Atrioventricular Septal Defect: A CHSS Inception Cohort Study First patient enrolled December 2011