Atrioventricular Canal Defect

Ernerio T. Alboliras, MD, FASE, FAAP, FACC
Director of Fetal Cardiology
Phoenix Children’s Medical Group -Phoenix
Children’s Hospital
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 Atrioventricular Canal Defects
Atrioventricular Septal Defects
Endocardial Cushion Defects
Embryology of the Atrioventricular Canal
The Normal Cardiac Crux
Eidem, B 2010
Atrioventricular Canal Defects
Characterized by varying degrees of
incomplete development of the septal
tissue surrounding the atrioventricular
(AV) valves along with varying degrees of
abnormalities of the AV valves
themselves.
Types of Common Atrioventricular
Canal Defects
Complete Common Atrioventricular Canal
 Has both defects in the atrial septum just above the AV valves (ostium primum
ASD) and defects in the ventricular septum just below the AV valves.
Partial Common Atrioventricular Canal
 (Also known as incomplete AVC or partial AVSD) has an ostium primum atrial
septal defect (ASD) and may have varying degrees of malformation of the
LAVV leading to varying degrees of LAVV regurgitation.
Intermediate or Transitional Type of Common
Atrioventricular Canal
 Transitional AVC (also known as intermediate AVC) is in the middle of this spectrum and
has two distinct LAVV and RAVV orifices, but also has both an ASD just above and a
VSD just below the AV valves
Atrioventricular Canal-Type of Ventricular Septal
Defect
 Defect in ventricular septum underneath the AV valves and with no muscular
separation between the defect and the valves.
Atrioventricular Canal Defect
Courtesy of Edwards, W.
Atrioventricular Canal Defect
Oh, J 2007
Atrioventricular Canal Defects
Eidem, B 2010
Atrioventricular Canal Defect
Cohen, M 2010
Goose-Neck Deformity in AV Canal
Accuracy of Ulltrasonography for AVC
Defect
Total AVC
Positive Prenatal
Detection
Percentage
Garne 2001
18
10
56%
Heide (Leeds) 2004
92
27
29%
Khoo (S Aust) 2008
21
5
23.8%
Marek 2010 (19861999)
398
102
25.6%
Marek 2010 (20002006)
169
141
83.4%
Friedberg 2009
14
6
43%
Accuracy of Ulltrasonography for AVC
Defect
Meyer-Wittkopf, et al Royal Alexandra Children’s Hosp, Sydney 2001
Fetal Diagnosis of
AVC
OB Ultrasound
(OB, MFM, Radiol)
Fetal Echo
(Peds Cardiol)
False Positive
7
0
False Negative
5
0
True Positive
25
30
No Postnatal
Confirmation
5
7
Segmental Technique for Diagnosing CHD
 Cardiac and visceral positions; cardiac size
 Ductus venosus
 Visceroatrial situs
 Systemic and pulmonary venous connections and flow
 Atrial morphology
 Patent foramen ovale flow
 Atrioventricular valve morphology and function
 Ventricular looping, size, configuration and function
 Defects in atrial and ventricular septation
 Abnormalities of morphologies and relationships of Ao and PA
 Patent ductus arteriosus size and flow
 Cardiac rate and rhythm
 Middle cerebral artery flow
 Umbilical artery and umbilical venous flow
Goals of Fetal Echocardiography in
Atrioventricular Canal Defect
Determination of ASD size.
Determination of VSD size.
Anatomy of the atrioventricular annulus, valve, and
chordal attachments.
Assessment for atrial or ventricular level unbalance.
Assessment for LV outflow tract obstruction if present.
Hemodynamic assessment – valvar regurgitation.
Ventricular function.
Associated lesions – TOF, CoA, additional VSD,
dextrocardia, venous anomaly, Ebstein.
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Imaging AV Canal Defect
Associated Cardiac malformation in
AVC
 RV dominant AVC
 Spongiform myocardium
 LV dominant AVC
 TAPVC or PAPVC
 RVOT Obstruction
 Single RV
 TOF/Pulmonary atresia
 Truncus Arteriosus
 LVOT obstruction
 Complete heart block
 DORV
 D-TGA
 CoA/Hypoplastic Ao
 L-TGA
 Dextrocardia
 Ebstein
 Heterotaxy
 Severe AV valvar regurgitation
Partial AVC
AVC-type VSD
AV Canal with LVOT Obstruction
AV Canal with TAPVC to SVC
AV Canal with Infracardiac TAPVC
AV Canal with TOF
AV Canal with TOF
AV Canal with Common Atrium
AV Canal with Small LV
AV C w/ Common Atr, CHB, Spongy
Myocardium
LV Dominant CCAVC
RV Dominant CCAVC
Importance of Correctly Identifying the
Presence of AVC Defect
Berg, et al 2009
CHD in 487 Fetuses with Down Syndrome
Mogra, Zidere, Allan 2011
Associated conditions and outcome in 246 prenatally
diagnosed atrioventricular septal defects
Berg et al (Bonn, Germany) 2009
Associated conditions in Partial AVC
Paladini, et al 2009
Prognosis of Fetal AV Canal
Rasiah, et al (Birmingham UK) 2008
Counseling the Family
Associated intracardiac lesion
Associated extracardiac structural
fetal lesion
Associated genetic anomaly and its
concomitant anomalies
Natural History of the Abnormal
Fetal Heart
CONCLUSIONS
 A comprehensive diagnosis is important for a
confident discussion with the parents on the
baby’s cardiac condition and for surgical planning.
 A simple diagnosis of “AV Canal” of “AV septal
defect” is not enough.
 Ruling in or ruling out associated anomalies
provides a broad discussion point with the parents
and with the surgeon.
 Accurate diagnosis allows precise prognostication
and appropriate counseling.
THANK YOU!
THANK YOU!
 Atrioventricular Canal Defects
Atrioventricular Septal Defects
Endocardial Cushion Defects