Download Hypoplastic Left Heart - sha

6/2/2013
University of Florida
The Gator Nation
Hypoplastic Left Heart
“Syndrome”
F. Jay Fricker
University of Florida
Medical Director of the Congenital heart Center
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Hypoplastic Left Heart “Syndrome”
Introduction
 Definition, anatomy and physiology
 Diagnosis and stabilization
 Neonatal surgical management
 Postoperative management
 Surveillance
 Fontan circulation
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Introduction
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HLHS is relatively common CHD
Accounts for 7 – 9 % of neonates diagnosed with
heart disease in first year of life
 Accounts for 2-3% of all CHD
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First description in 1850 (Canton, London)
Underdevelopment of multiple left-sided
structures linked (Lev, 1952)
Noonan and Nadas coin term “hypoplastic left
heart syndrome” (1958)
Outcome uniformly fatal until Norwood
approach (first reported in 1981)
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Hypoplastic Left Heart
Anatomic Features
 Usual
atrial arrangement
 Concordant atrioventricular connections
 Concordant ventriculo-arterial
connections
 Hypoplasia of the left ventricle IS A
NECESSITY
 Inflow and outflow obstruction ARE
VARIABLE
Hypoplastic Left Heart
Anatomic Features
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Atretic MV with absent AV connection
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Hypoplastic Left Heart
Anatomic Features
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Ventriculo-arterial connection
Aortic Stenosis
Aortic Atresia
Hypoplastic Left Heart
Anatomic Features
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MV Stenosis – ventricle with globular appearance
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Hypoplastic Left Heart
Anatomic Features
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Aortic anatomy in HLH
 Often most narrow at the sinotubular
junction
 Functions as a conduit that feeds the
coronaries
 Distally, aortic coarctation is common
 Occurs in 4/5 of patients
 Typically preductal
Hypoplastic Left Heart
Anatomic Features
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Aortic Anatomy – marked size variation
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Hypoplastic Left Heart
Anatomic Features
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Aortic Coarctation
*Can be para-ductal
*Can cause stenosis of the left
subclavian artery
*Coarctation presence affects
outcome
PDA dependent retrograde
ascending aorta and coronary
blood flow
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Hypoplastic Left Heart
Anatomic Features

Typical features of the right ventricle
 Dominant in HLH
 Tricuspid valve can be dysplastic*
 Trabeculations are prominent
 Pulmonary artery provides major
pathway for circulation
Hypoplastic Left Heart
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RV Anatomy
RV forms the apex
Prominent trabeculations
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Hypoplastic left Heart
Anatomic Features
Left Atrium and atrial septum
 Small LA with posterior deviation of the
superior rim of atrial septum
 Atrial septum can be intact and decompressed
by the levocardinal vein
 Associated with pulmonary lymphangectasia and
muscularization of pulmonary veins
 High risk balloon or blade atrial septostomy
Hypoplastic Left Heart
Anatomic Features
Can be seen with…
Ventricular septal defect
Atrioventricular septal defect
Critical aortic stenosis
Aortic coarctation
DORV
*Problem – When is the left ventricle hypoplastic?
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Hypoplastic Left Heart Syndrome
Associated Noncardiac Anomalies
Chromosomal abnormalities
 Genetic defects
 Major extracardiac structural
malformations
 CNS abnormalities
 Present in 25 – 40 % of patients
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Hyploplastic Left Heart Syndrome
Clinical Presentation
 Cyanosis
within first hours of life
 Progressive cardiorespiratory
collapse
 Shock with end-organ dysfunction
 Can
avoid crisis with prenatal
diagnosis
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Hypoplastic Left Heart
Factors affecting Clinical Presentation
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Cyanosis minimal and may not be recognized
because of PDA patency(Infant Discharged
from Newborn Nursery.)
Severe Hypoxemia is related to restrictive interatrial septum.
“Best Clinical Finding” Decrease pulses and
Active precordial impulse
Hypoplastic Left Heart
Echocardiography
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Hypoplastic Left Heart
Assymmetrical AVS
Hypoplastic Left Heart
Echocardiography
Ductal Arch
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Hypoplastic Left heart
Aortic Arch
Hypoplastic Left Heart
AscendingAorta
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Physiology of HLHS
Fetal Flow
atrial
SVC
LA
RA
septum
IVC
pv
LV
SINGLE
VENT
BODY/
Placenta
(low SVR)
PDA-Ao
Qs
LUNGS
(high PVR)
PA
Sat=65%
Qp
Physiology of HLHS
Postnatal Flow
atrial
SVC
LA
RA
septum
IVC
pv
LV
SINGLE
VENT
BODY
( high SVR)
PA
PDA-Ao
Qs
Sat=80%
LUNGS
(falling PVR)
Qp
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Physiology of HLHS
Closing Ductus
atrial
SVC
LA
RA
septum
IVC
pv
LV
SINGLE
VENT
PA
Shock
Closing PDA
Qs
Sat=92%
LUNGS
(falling PVR)
Qp
Hyploplastic Left Heart Syndrome
Initial Medical Management
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Prostaglandin E1
Ventilator with:
 Hypoventilation
 RA oxygen
 pH 7.30– 7.40
 Inspired CO2/N2
Inotropes and vasodilators
Sedation/paralysis
Balance Qp:Qs (increase systemic perfusion
and control pulmonary overcirculation)
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Hyploplastic Left Heart
Options
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No treatment? (Different from European perspective)
 Cardiac transplantation
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Staged approach to Fontan
Cardiac Transplantation
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Good therapy but …
Children die waiting
Stage I Norwood results better
UF CHC Results since 2005
38 Infants(4 Deaths)Norwood.
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Hypoplastic Left Heart
Heart transplantation
Features that Favor primary Heart Transplantation
 Ascending Aorta size
 RV size and Function
 Severe Tricuspid Valve regurgitation
 Congenital Heart center Experience and
outcome with Norwood
 Family Preference
Route to the Fontan …
Stage I - Neonatal palliation
 Stage II - Superior cavopulmonary
connection (BDG or HemiFontan)
 3-6 months
 Stage III – Completion Fontan (ECC or
lateral tunnel)
 18 months – 3 years
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HLHS Treatment
Controversies and Issues
Modified BT shunt vs. RV-PA conduit
 Late effects of RV-PA conduit?
 Regional low-flow vs. Circulatory arrest
 Routine VAD support
 Neurodevelopment
 Gastrointestinal morbidity
 The intact atrial septum . . .
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Hyploplastic Left Heart Syndrome
Stage I
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Hyploplastic Left Heart Syndrome
Stage I
Hyploplastic Left Heart Syndrome
Stage I with RV – PA conduit (Sano)
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Sano Modification
Improved postoperative
hemodynamics
 Higher diastolic pressure
 Lower pulse pressure
 Lower Qp:Qs
 Lower interstage M+M
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Pediatric Heart Network Single Ventricle
Reconstruction trial
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Randomized clinical trial 555 subjects from 15
North American Centers
Norwood Stage 1 repair Using Blalock-Taussig
shunt vs RV to PA conduit(SANO)
Sano subjects had better primary outcome
Transplantation-free survival 74% versus 64%
Sano group had more unintended interventions
and complications
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Hypoplastic Left Heart
Hybrid procedure
Systemic Blood Flow
Secured with PDA Stent
Pulmonary Blood Flow
Secured by Banding of
Branch PA bands
Atrial Balloon Septostomy
Delay
Hybrid Approach for HLH
Results after the Learning Curve
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Mortality stage 1
2%
Mortality Interstage
5%
Reintervention
36%
Mod RV Dysfunction 3%
Mod TV Regurgitation 3%
Mortality Stage 2
8%
ECMO
0%
Open Sternum
0%
Overall Survival 82.5%
NCH-Hybrid
N 40
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Postoperative Management
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Vasodilators and inotropes (milrinone, epi,
NTP)
Ventilation and cardiopulmonary interactions
Sedation
Monitoring (CAP, cerebral and peripheral
oximetry, venous oximetry, UOP, toe temp)
Open chest
Early institution of aggressive diuretic therapy
Others: closing chest, routine ECMO support
Monitoring …
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Postoperative Management
Priorities
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Cardiac and Respiratory stability
Maximize cerebral flow
Then … maneuvers to allow chest closure
Then … weaning ventilator
Then … weaning cardiac medications
Then … maximize nutrition
Then … optimize normal feeding
Then … discharge by Joni and Kim
Hyploplastic Left Heart Syndrome
Results from major centers improved
 First stage survival over 80%
 New modification may improve
outcome
 RV to PA connection instead of
central shunt
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Surveillance
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Close follow-up – low threshold for admission
Physical Exam and ECHO for Clinical change
 Recoarctation
 Increase
AV valve regurgitation
 Decrease Ventricular function
 Increase in Cyanosis
Saturation monitors and scales (Wisconsin
group)
Establish a Multispecialty Followup clinic for
infants who have had single ventrical
Palliation
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Conclusions …
HLHS is most commonly treated with
Norwood procedure
 The Sano modification may improve early
results
 Improved care for other lesions
 Quality of life? … we can learn from our
families
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29 week premature , 1.4 kg
HLHS, aortic arch 1 – 1.5 mm, intact atrial septum
PVs draining into innominate via levoatrio cardinal vein
Transferred for definitive management
Severe preoperative overcirculation
Required resuscitation including hypoxic gas mixture
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Only those who will risk going
too far can possibly find out
how far one can go …
T. S. Eliot
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