Complex AVSD: what could be done better
Jan Marek
Great Ormond Street Hospital
&
Institute of Cardiovascular
Sciences, London, UK
No Disclosures
Complex AVSD: what could be done better
Case: unbalanced AVSD, T+21, 8 weeks
3 Months of age
LV:RV = 0.9
LAVV 10mm
RAVV 16mm
Complex AVSD: what could be done better
Case #1: unbalanced AVSD
LV:RV = 0.9
LAVV 10mm
RAVV 16mm
LAVVI = .35
LV:RV = 0.9
LAVV 10mm
RAVV 16mm
LAVVI = .35
Parachute valve +/-
At 8 weeks:
AVSD repair
(double patch)
`cleft` not sutured
…. 6 weeks post repair
…. 6 weeks post repair
…. 2 months post redo:
`cleft` sutured, papillary muscles splitted
…. 2 months pos redo:
`cleft` sutured, papillary muscles splitted
Unbalanced AVSD
• One ventricle & corresponding AVV hypoplastic, other
ventricle receives larger portion of CAVV
•Dominant RV, Hypoplastic LV more common (85%)
SMALL LEFT VENTRICLE
Primarily or secondarily small?
Small left ventricle in overloaded
(pressure, volume) right ventricle
is primarily not only due to
compression; rather is due to
underfilling
•TAPVC
•Isolated COA
•AVSD
•PPH/PFC
LV can stretch more than
anticipated (elastic stocking vs.
plastic bag)
Even “hypoplastic” LV cavity < 15-20 ml/m2 can
expand to adequate volume
Phoon CK,Am Coll Cardiol, 1997
At 8 weeks:
AVSD repair
(double patch)
`cleft` not sutured
Predictors of successful 2V repair
in unbalanced AVSD
• RV Dominance / LV hypoplastic
Cohn et al. JACC 1996
– In 26 unbalanced AVSD: No echo parameters
distinguishing survivors from non–survivors of BVR
Van Son et al. ATS 1997
– Preoperative “potential” LV volume > 15 ml/m2 present
in all 5/5 patients who survived BVR
• LV Dominance /RV hypoplastic
De Oliveira et al. JTCS 2005
– Small RV + AVSD and BVR – 4/32 hospital deaths, all
with RAVV area/LAVV area <0.50
Unbalanced AVSD
AVVI = RAVV : LAVV ratio
Cohen M, JACC 1996
Unbalanced AVSD
AVVI = RAVV : LAVV ratio
Cohen M, JACC 1996
Unbalanced AVSD
Cluster analysis of ECHO
variables was used to group
patients with similar features
RV:LV Inflow Angle
(partial R2=0.86)
…may be important defining
echocardiographic measures
of right dominant unbalanced
AVSD
Cohen M, Circ Imaging 2013