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 • Christopher Caldarone christopher.caldarone@sickkids.ca • Maulik Baxi maulik.baxi@sickkids.ca • Veena Sivarajan veena.sivarajan@sickkids.ca • Susan McIntyre susan.mcintyre@sickkids.ca 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. Late post-op (6-12 months) Additional echoes for identified re-interventions • – AV valve replacement or resection of subAS – Pts with initial PA band or other “non- definitive” palliation – Would require additional 3 studies (pre, post and late) – CHSS to develop triggers for participating centers to upload echo studies when due – Echo reports for late studies (>12 months) will be sent directly from centers to CHSS Data Center (Echo Reporting Form pending) Inclusion Criteria • Diagnosis of or referral with complete AVSD at a CHSS member institution within first year of life. – Includes TOF and Double Outlet Right Ventricle with complete AVSD • Atrioventricular and Ventriculoarterial concordance (with the exception of DORV). • Informed written consent. • Note: we are including heterotaxy and TAPVC/PAPVC Exclusion Criteria • Partial or Transitional AVSD. – Separate AV valve orifices – Non-existent ventricular septal defect • Aortic Atresia • 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 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