Congenital Heart Surgeon Society Atrioventricular Septal Defect prospective inception cohort Webinar Series.

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Congenital Heart Surgeon Society Atrioventricular Septal Defect prospective inception cohort Webinar Series

uAVSD Echo Core Lab Members Michael Quartermain mquarter@wakehealth.edu Luc Mertens luc.mertens@sickkids.ca Meryl Cohen cohenm@email.chop.edu David Gremmels DGremmels@chc-pa.org Gina Baffa gbaffa@NEMOURS.ORG CHSS Data Center Staff Bill Williams bill.williams@sickkids.ca Bill DeCampli William.Decampli@orlandohealth.com Veena Sivarajan veena.sivarajan@sickkids.ca Principal Investigator: David Overman DOverman@chc-pa.org‎

Study protocol Acquire images on enrolled subjects at set time intervals Submit to virtual core lab Measurements will be performed by core lab

Timing of Echo Studies 3 Echocardiograms per patient 1. Pre-operative study (most complete diagnostic study, discretion of site) 2. Pre-discharge post-op study (or 30 days post-op, whichever first) 3. 1 year post-operative study

Inclusion Criteria Diagnosis of complete AVSD Admitted to a CHSS institution for surgery after January 1, 2012 Age < 365 days at admission for surgery Atrioventricular and Ventriculoarterial concordance (includes TOF and DORV). Informed written consent.

Exclusion Criteria Partial or Transitional AVSD. Separate AV valve orifices Non-existent ventricular septal defect Aortic Atresia Total or Partial Anomalous Pulmonary Venous Drainage (TAPVC or PAPVC) Heterotaxy First Intervention at a non-CHSS institution

ASD views

ASD subcostal

ASD views

VSD

Image additional VSDs

AVVI: SC en face view of AVV

AVVI Atrioventricular Valve Index (AVVI) Subcostal LAO view Morphometric Analysis of Unbalanced Common Atrioventricular Canal Using Two-Dimensional Echocardiography MERYL S. COHEN, MD, MARSHALL L. JACOBS, MD, PAUL M. WEINBURG, MD, FACC, JACK RYCHIK, MD, FACC Philadelphia, Pennsylvania (J Am Coll Cardiol 1996;28: 1017-23) Atrioventricular Valve Index (AVVI) Subcostal LAO view Measure area of common AV valve apportioned over each ventricle LAVV:RAVV or RAVV:LAVV

AVVI UAVSD

AVVI

CHSS Lookback Modified AVVI LAVV:Total AVV 0.5 Right dominant Left Dominant Overman DM, et al. WJSPCHS 1(1), Sept 2008

Apica 4 Ch view

APICAL 4-Chamber

LV 2-chamber

LV 3-Chamber

Sweep through LAVV +RAVV

LAVVR + RAVVR

RAVVR

RV inflow

LV inflow

Left AV Valve Index (LVII) Szwast AL, et al. Am J Cardiol 2011;107:103–109

RV/LV Inflow Angle - Balanced 154°

RV/LV Inflow - Unbalanced 154° 82°

Other measurements

Papillary muscles Parachute-like with one dominant papillary muscle group

LVOT views

LVOT

LVOT measurements

LVOTO- describe mechanism

Doppler gradient

RVOT

PA branches

Ductal cut

Aortic arch

Pulmonary veins

Systemic Venous anomalies

3-D if available (subcostal)

3-D if available (apical 4)

Further information Two additional webinars in March Online information via the CHSS website: http://www.chssdc.org/studies Ongoing open forum with Echo core and Data Center

Summary There are no unique or novels views Focus on high quality, complete sweeps with particular attention to: Subcostal (Left anterior oblique) Apical 4 chamber on inlet region and secondary inflow LV outflow tracts from multiple views 3D when available

Questions ? Thank you for your participation