Download Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome &
Aortic Stenosis
Alfred Abuhamad, MD.
Eastern Virginia Medical School
Abnormal Left Ventricle
•Bicuspid aortic valve
•Aortic
Aortic stenosis
•Hypoplastic
yp p
left ventricle
Bicuspid
p Aortic valve
• Most common CHD, 0.5-2% population
• Male predominance, 3:1
• Commonly
y results from fusion of 2
commissures (RT and LT)
• Nonvalvular findings in 50%
–
–
–
–
–
Dilation of thoracic aorta
Coarctation of aorta
Shone’s syndrome
Williams syndrome
Turner syndrome
J Am Coll Cardiol 2010;55:2789
Aortic valve
Normal
Aortic valve
Bicuspid
Bicuspid
p Aortic Valve
Normal
Stenosis
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Bicuspid
Bicuspid Aortic valve
Risk Factors
•Age > 30
•Moderate or severe aortic insufficiency
•Moderate
M d
t or severe aortic
ti stenosis
t
i
J Am Coll Cardiol 2010;55:2789
Aortic Valve
Hypoplastic left heart
Critical aortic stenosis
Aortic stenosis
Bicuspid valve
Spectrum
Aortic Stenosis
Narrowing of the aortic valve
• Valvular (most common)
• Sub-valvular
• Supra-valvular
Dysplastic
y p
Aortic Valves
Aortic Stenosis
•Occurs in 3-6 % of heart defects
•More common in male fetuses (3:1)
•Associated cardiac malformations
occur in 20 % (VSD-CoA)
•Extracardiac malformations - uncommon
•Rare association with chromosomal
malformations
•Consider Williams-Beuren Syndrome
((del 7q11.23)
q
)
•Shone’s Complex
Aortic Stenosis
Aortic Stenosis
Critical Aortic Stenosis
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Aortic Stenosis
Ultrasound Findings
•Thickened valve leaflets
•Doming of the cusps
•Lack of complete valve opening
d i systole
during
t l
•Post-stenotic dilation of
ascending aorta
pp
•Turbulent flow on color Doppler
•High PSV (200 cm/sec) on pulsed Doppler
Aortic Stenosis
Aortic Stenosis
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Aortic Stenosis
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Critical Aortic Stenosis
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Aortic Stenosis
•Prenatal follow-up Q 3-4 weeks
•Poor prognosis:
¾Reverse flow across foramen
¾Monophasic LV diastolic filling
¾Reduced aortic PSV
Circulation2004;110:2125 – Circulation2006;113:1401
Hypoplastic Left Heart Syndrome
Hypoplastic Left Heart Syndrome
Spectrum of Malformations
Severe hypoplasia of left ventricle
and left ventricular outflow tract
Obstruction to Systemic Cardiac Output
Hypoplastic Left Heart Syndrome
Classic Types:
• Mitral
Mit al & aortic
ao tic atresia
at esia
• Aortic atresia (Patent MV)
Ultrasound Obstet Gynecol 2000;4:271
Hypoplastic Left Heart Syndrome
DA
AO
PA
RA
RV
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Hypoplastic Left Heart Syndrome
Variants:
• Critical
C iti l AS with
ith hypoplastic
h
l ti LV
• Severe coarctation of Aorta
• Severely unbalanced A-V canal
Ultrasound Obstet Gynecol 2000;4:271
Hypoplastic Left Heart Syndrome
• Incidence:
I id
0
0.1
1 – 0.25
0 25 / 1000 live
li
births
• Karyotypic abnormalities in 5 %
• Extracardiac anomalies in up to 28%
• Preponderance in ♂ fetuses (7/10)
• Represents 5 % of all CHD
1
Ultrasound
Obstet Gynecol 2000;4:271
Pediatrics 1988;82:698
Hypoplastic
yp p
Left Heart Syndrome
y
Karyotypic Abnormalities
• Classic HLHS (4-5%)
(4 5%)
• Critical AS (very Low)
• A-V canal defects (High)
( g )
• Coarctation of Aorta (High)
g
Hypoplastic
yp p
Left Heart Syndrome
y
Genetic Syndromes:
• Turner
• Noonan
• Smith-Lemli-Opitz
• Holt-Oram
Orphanet J Rare Dis 2007;100(9):1246
Hypoplastic
yp p
Left Heart Syndrome
y
P
Prenatal
t l US ffollow-up
ll
• IUGR due to 20 % reduction in
combined cardiac output
Am J Epidemiol 1996;143:505
Hypoplastic Left Heart Syndrome
Recurrence Risk:
• 2-3
2 3 % with
ith a prior
i child
hild
• 13
13-18
18 % if mother has AS
• 3 % if father has AS
Ultrasound Obstet Gynecol 2000;4:271
Normal
HLHS
From Little Hearts Matter (www.lhm.org.uk) with permission
Hypoplastic Left Heart Syndrome
Four-Chamber View
• Diminutive, hypertrophic,
hypokinetic
yp
LV
• RV apex forming
• Mitral valve dysplastic,
echogenic
h
i and
d stenotic
i
• Foramen ovale leaflet from
left to right
• Color Doppler fills RV only
LV
RV
RA
LA
Hypoplastic Left Heart Syndrome
RV
LV
LV
RA
LA
RV
RA
LA
N
l
Normal
Hypoplastic left heart syndrome
RV
RV
LV
LV
RA
LA
RA
Aortic & mitral atresia
LA
Critical aortic stenosis
Hypoplastic left heart syndrome
Four-Chamber View
Hypoplastic left heart syndrome
Four-Chamber View
Hypoplastic left heart syndrome
Mitral Atresia - VSD
Hypoplastic left heart syndrome
Left Ventricular Outflow Tract
Hypoplastic left heart syndrome
Right Ventricular Outflow Tract
Hypoplastic Left Heart Syndrome
4-Chamber Color Doppler
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Hypoplastic left heart syndrome
4-Chamber Color Doppler
Hypoplastic left heart syndrome
4-Chamber Color Doppler
Hypoplastic Left Heart Syndrome
Color Doppler – Foramen Ovale
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Hypoplastic Left Heart Syndrome
Left-to-right shunt
At
Foramen Ovale
LA
Hypoplastic Left Heart Syndrome
Three-Vessel Trachea View
• Two vessels are seen
• Dilated PA, next to SVC
• Nonvisible or diminutive aorta
• Color Doppler shows reverse flow
in aortic isthmus
Hypoplastic Left Heart Syndrome
3-Vessel Trachea View
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Hypoplastic left heart syndrome
3 Vessel-Trachea View
Hypoplastic left heart syndrome
3 Vessel-Trachea View
Hypoplastic Left Heart Syndrome
Reconstructive Strategy
• Restoring systemic circulation
• Restoring pulmonary circulation
• Maintaining coronary circulation
SVC
PA
IVC
RV
HLHS
From Little Hearts Matter (www.lhm.org.uk) with permission
Hypoplastic Left Heart Syndrome
Reconstructive Strategy – Stage 1:
•
•
•
•
Atrial septectomy
Proximal PA to Aorta anastomosis
R
Reconstruction
t ti off A
Aortic
ti A
Arch
h
Aorto-PA
Aorto
PA shunt
Semin Fetal Neonatal Med 2005;10(6):553
HLHS
Stage 1 - Norwood
From Little Hearts Matter (www.lhm.org.uk) with permission
HLHS
(Sano Modification)
Preserves coronary
y circulation
Reduces diastolic run-off
Provides pulsatile flow to PA
Stage 1 - Modified
From Little Hearts Matter (www.lhm.org.uk) with permission
Hypoplastic Left Heart Syndrome
Stage 1 – Cardiovascular Implications:
• RV as systemic
y
&p
pulmonic
ventricle
• RV volume
l
overload
l d
• O2 saturation ~ 75 - 85 %
Semin Fetal Neonatal Med 2005;10(6):553
Hypoplastic Left Heart Syndrome
Reconstructive Strategy – Stage 2:
• Elimination of shunt
• Anastomosis of SVC to branch PA
(Glenn or hemi-Fontan)
• Augmentation of branch PA as
needed
(Delayed due to early high PVR)
Semin Fetal Neonatal Med 2005;10(6):553
Stage 1
Stage 2
From Little Hearts Matter (www.lhm.org.uk) with permission
Hypoplastic Left Heart Syndrome
Stage 2 – Cardiovascular Implications:
• RV volume
l
unloading
l di
• O2 saturation ~ 80 - 85 %
Semin Fetal Neonatal Med 2005;10(6):553
Hypoplastic Left Heart Syndrome
Reconstructive Strategy – Stage 3:
• IVC fl
flow channeled
h
l d tto PA
PAs
(Fontan)
Semin Fetal Neonatal Med 2005;10(6):553
Stage - 2
Stage 3 - Fontan
From Little Hearts Matter (www.lhm.org.uk) with permission
Hypoplastic Left Heart Syndrome
Stage 3 – Cardiovascular Implications:
• Increase pulmonary flow
• O2 saturation > 90 %
Semin Fetal Neonatal Med 2005;10(6):553
Hypoplastic
yp p
Left Heart Syndrome
y
Survival of Staged Repair:
• Stage 1: 46 – 76 % (90 % in
some centers)
• Stage 2: 95 %
• Stage
St
3:
3 95 %
• (4 – 15 % die at home before Stage 2)
Ultrasound Obstet Gynecol 2000;4:271
Pediatrics 2007;119(1):109
Hypoplastic
yp p
Left Heart Syndrome
y
Prenatal Follow
Follow-up:
up:
• Monthly
y
• Fetal growth (IUGR)
• Size inter-atrial communication
(PV Doppler)
• Function of A-V valve - RV
Semin Fetal Neonatal Med 2005;10(6):553
Hypoplastic Left Heart Syndrome
Restriction of flow across foramen
From: A Practical Guide to Fetal Echocardiography – Abuhamad, Chaoui
Hypoplastic Left Heart Syndrome
Poor Prognostic Factors:
• Low birth weight
• Prematurity
P
t it
• Non-cardiac anomalies
• Obstruction to PV return (restricted
atrial septum)
• Poor RV function
Ultrasound Obstet Gynecol 2000;4:271
Pediatrics 2007;119(1):109
Hypoplastic Left Heart Syndrome
Morbidities Post Therapy:
•
•
•
•
•
Exercise Intolerance
Atrial Arrhythmias (20 – 50 %)
Thrombo-embolic disease (10%)
Protein-losing
g enteropathy
p
y ((< 5 %))
Neurocognitive disabilities (10 – 70 %)
Semin Fetal Neonatal Med 2005;10(6):553
Hypoplastic
yp p
Left Heart Syndrome
y
Neurocognitive Disabilities:
•P
Possible
ibl associated
i t d CNS anomalies
li
• Hemodynamic instability in preoperative period
• Intra-operative
p
p
perfusion techniques
q
Semin Neonatol 2003;8(6):461
Hypoplastic Left Heart Syndrome
Fetal Interventions
•Balloon Atrial Septostomy (severe restriction)
•Balloon
B ll
aortic
ti valvuloplasty
l l l t (critical aortic stenosis)
Hypoplastic Left Heart Syndrome
Courtesy of Dr. Benson
Hypoplastic Left Heart Syndrome
Courtesy of Dr. Benson
Hypoplastic
yp p
Left Heart Syndrome
y
Team Approach
• Sonographer
g p
• MFM Specialist
p
• Pediatric Cardiologist
• Neonatologist
• Cardiac Surgeon