Download Hypoplastic Left Heart Syndrome: Echocardiographic Assessment

Hypoplastic Left Heart Syndrome:
Echocardiographic Assessment
Craig E Fleishman, MD, FACC, FASE
Director, Non-invasive Cardiac Imaging
The Hear Center at Arnold Palmer Hospital for
Children
SCAI Fall Fellows Course 2012
Las Vegas
HLHS: Echo Assessment SCAI 2012
Disclosure Information
HLHS: Echocardiographic Assessment
Craig E Fleishman MD, FACC, FASE
As a faculty member for this program, I disclose the following relationships
with industry:
(GRS): Grant/Research Support (C): Consultant (SB): Speaker’s Bureau
(MSH): Major Stock Holder (AB): Advisory Board (E): Employment
(O):Other Financial or Material Support
W.L. Gore Medical: C, Echo Core Lab
HLHS: Echo Assessment SCAI 2012
HLHS
HLHS: Echo Assessment SCAI 2012
HLHS
•Spectrum of Malformations
– 7-9% CHD diagnosed in infancy
•Underdevelopment of left heart
– Left atrium
– Mitral Valve
– Left Ventricle
– Aortic Valve
– Aortic Arch
HLHS: Echo Assessment SCAI 2012
Left Atrium
– Hypoplastic
– Thick walled
– Variable size of ASD
• Some restriction
helpful
• Intact atrial septum
– Look for levoatrial
cardinal vein
– Malalignment of atrial
septum
• Leftward and posterior
attachment to LA free
wall
• Small, superior,
posterior PFO
HLHS: Echo Assessment SCAI 2012
Mitral Valve
• Stenotic, hypoplastic,
or atretic
– MV annulus
diameter ≤ 6 mm
• May not have well
defined chordae
(arcade)
• Papillary muscles
– Closely spaced
– Parachute
HLHS: Echo Assessment SCAI 2012
LV
• Variable hypoplasia
– Does not extend to cardiac
apex
– LV area < 1.5 cm2 (PLAX)
– Smallest with MA/AA
– Larger with VSD
• Hypertrophy
• Echo bright areas reflect
endocardial changes
– Endocardial fibroelastosis
HLHS: Echo Assessment SCAI 2012
Aortic Valve and Aorta
• Aortic atresia (plate) or
dysplastic
– Larger with VSD
• Hypoplastic ascending aorta
– <5 mm, usually smaller
– Main coronary artery
– Fills retrograde from ductus
arteriosus
• Aorta becomes larger at 1st
arch vessel, but not normal
until distal to PDA
• Aortic arch branching pattern
• Juxtaductal posterior
shelf/coarctation
HLHS: Echo Assessment SCAI 2012
PDA
• PDA usually large, with
aorta inserting onto ductal
arch
• Right to left flow in systole
• Left to right in diastole
– Diastolic flow reversal
in abdominal Ao
– L to R decreased with
Pulm HTN
• PPHTN
• Intact atrial septum
HLHS: Echo Assessment SCAI 2012
Coronary Arteries
•Anomalous origins are rare
•Abnormalities more common with MS/AA
– Single coronary
– Tortuosity
– Sinusoids between coronaries and LV
•? left ventricular dependent coronary
circulation
HLHS: Echo Assessment SCAI 2012
Other areas of interest
•Pulmonary stenosis or regurgitation
•Right ventricular function
•Tricuspid regurgitation
•Left SVC
•Abnormal pulmonary venous connections
(6%)
HLHS: Echo Assessment SCAI 2012
HLHS Intact Atrial Septum
•18/316 infants over 6.5 yr period
•Atrial septal morphology evaluated
•Lung histopathology reviewed
HLHS: Echo Assessment SCAI 2012
Type A atrial morphology
• 12/18 infants
• Relatively large LA
• Thick septum secundum
• Thin septum primum
• 5/12 with leftward and
posterior deviated atrial
septum
• All had LA
decompression
pathway, 3 severely
obstructed
Rychik, J. et al. J Am Coll Cardiol 1999;34:554-560
Copyright ©1999 American College of Cardiology Foundation. Restrictions may apply.
HLHS: Echo Assessment SCAI 2012
Type B atrial morphology
• 4/18 infants
• Small, muscular LA
• Circumferential
thickening of atrial walls
• Thick atrial septum w/o
distinction between
septum primum and
secundum
• 4/4 with absence or
severe obstruction of LA
decompression pathway
Rychik, J. et al. J Am Coll Cardiol 1999;34:554-560
Copyright ©1999 American College of Cardiology Foundation. Restrictions may apply.
HLHS: Echo Assessment SCAI 2012
Fetal echocardiographic image of type C
atrial morphology
• 2/18
• Giant LA
• Thin, rightward
bulging atrial septum
• Severe MR
• No LA decompression
pathway
Rychik, J. et al. J Am Coll Cardiol 1999;34:554-560
Copyright ©1999 American College of Cardiology Foundation. Restrictions may apply.
HLHS: Echo Assessment SCAI 2012
HLHS Intact Atrial Septum
•17/18 stage I Norwood
•6/17 Early survivors
– Type A atrial morphology
– Unobstructed decompression pathway
•3 additional late deaths after CPB
•Lung pathology
– Severely dilated lymphatics
– Arterialization of the pulmonary veins
Rychik, J. et al. J Am Coll Cardiol 1999;34:554-560
HLHS: Echo Assessment SCAI 2012
HLHS Intact Atrial Septum Example
HLHS: Echo Assessment SCAI 2012
HLHS Restrictive Atrial Septum Example
HLHS: Echo Assessment SCAI 2012
Time Passes
•Stage I with atrial septectomy #1
– Aortic arch stent via carotid cutdown
– ASD stent #1 for increased restriction
•Bidirectional Glenn with atrial septectomy #2
– PA stent
– ASD stent #2 for increased restriction
•Presents at 20 months of age with worse
cyanosis
HLHS: Echo Assessment SCAI 2012
HLHS Restrictive Atrial Septum Example
HLHS: Echo Assessment SCAI 2012
HLHS Restrictive Atrial Septum Example
HLHS: Echo Assessment SCAI 2012
Pre-Hybrid Screening
HLHS: Echo Assessment SCAI 2012
PFO/ASD
HLHS: Echo Assessment SCAI 2012
HLHS Pre-Hybrid Screening
• Follow path from PDA
to ascending aorta
– Lack of alignment
can signal twisting
• Retrograde flow
obstruction despite
unobstructed PDA can
be worsened with
PDA stent
• Potential areas for
stenoses
HLHS: Echo Assessment SCAI 2012
HLHS Pre-Hybrid Screening
HLHS: Echo Assessment SCAI 2012
Pre-Hybrid Screening
• Stenosis at isthmus
and at ascending
aorta/transverse arch
transition
• Treated with
traditional Stage I
– Anatomy confirmed
at surgery
HLHS: Echo Assessment SCAI 2012
Pre-Hybrid Screening
• Transverse arch and
ascending aorta
adequate
• Obstruction at distal arch
despite PGE and
unobstructed PDA
– Confirmed by color
flow and CW Doppler
HLHS: Echo Assessment SCAI 2012
Thank You
HLHS: Echo Assessment SCAI 2012
HLHS: Echo Assessment SCAI 2012