Download Hypoplastic Left Heart Syndrome What to Expect Down the Road

Hypoplastic Left Heart Syndrome
What to Expect Down the Road
Edward L. Bove, MD
Helen and Marvin Kirsh Professor
Chair, Department of Cardiac Surgery
University of Michigan
No Disclosures
What to Expect Down the Road
Edward L. Bove, MD
Helen and Marvin Kirsh Professor
Chair, Department of Cardiac Surgery
University of Michigan
“Hypoplastic left heart syndrome
is a plumbing problem…a serious
plumbing problem.”
-William Norwood, MD
 Arch reconstruction
Ascending aorta
Coronary artery
 Systemic to PA Shunt
 Modified BTS
 RV to PA conduit
 Superior cavopulmonary
shunt
 BDG
 HFP
 Fontan procedure
 Lateral tunnel
 Extracardiac conduit


 LV hypoplasia, 64%

HLHS, 52%
 RV hypoplasia, 36%
 Lateral tunnel, 92%
 Extra-cardiac, 8%
Hirsch et al, Ann Surg 2008;248(3):1-10
 Hospital survival, 96%
Survival with intact Fontan, 94%
 Takedown, 3% (17/636); mortality, 41% (7/17)
 4/10 survivors underwent later Fontan
 Median LOS, 10 days
 Morbidity
 Arrhythmias, 20%
 Prolonged pleural drainage (< 2 wk), 19%
 Neurologic event, 5%

 Median follow-up, 50 months
 Actuarial survival at 14 yrs, 91%
 Late morbidity

PLE, 6%

Neurologic event, 4%

Arrhythmia requiring treatment, 3%
 Chronic systemic venous hypertension
 PA hypotension
 Reduced ventricular preload/diastolic dysfunction
 Elevated systemic vascular resistance
 Low cardiac output
 PLE/plastic bronchitis
 Liver disease/cirrhosis/coagulopathy
 Pulmonary thromboembolism
 Recurring cyanosis/pulmonary AVM’s
 Exercise intolerance/ventricular dysfunction
 Dysrhythmias

Valve: neoaortic, tricuspid

Arch: obstruction, dilatation

Fontan pathway/pulmonary artery
Stenosis
Hypoplasia
Thrombus

Coronary artery stenosis
 NB male, 37 weeks, 3.4 kg
 Fetal diagnosis of HLHS

Aortic atresia, mitral atresia

Ascending aorta 2 mm

Normal RV function

Mildly restrictive ASD

Mild TR
 Stable on PGE infusion
The Year: 1994
 Successful Norwood operation performed with
3.5 mm MBTS
 Stage 2 and 3 procedures completed at 6 mos and
18 mos of age
 Active life, star pitcher for his HS baseball team
 Age 17 years, c/o angina symptoms while
pitching
 PAP 12 mmHg, no





Fontan pathway
obstruction
PVR, 2 units
Trivial TR
Normal RV function
No arch obstruction
But…aortogram
demonstrated narrowed
connection into the
native ascending aorta
 Successful revision of
coronary artery
connection performed
 Now w/o symptoms
and fully active
 Difficulty measuring the PVR
Non-pulsatile flow
 Low pulmonary blood flow
 AP collaterals
 Thromboembolic events
 Elevated antibody levels
 Transfusions
 Homograft patch material
 Technical difficulties

Carey et al Eur J Cardiovasc Surg 1998;14:7
65% actuarial
survival at 5 years,
p=ns, Fontan vs
non-Fontan
Kanter et al, Ann Thor Surg 2011;91:823
 Small increments in flow and pressure needed to be
effective
 Absence of venous reservoir/vessel collapse
 Avoid obstruction to native flow
 Potential for competing caval flows
 Bridge to transplant vs destination therapy
Giridharan et al JTCVS 2013;145:249
Giridharan et al, JTCVS 2013;145:249
Lacour-Gayet et al, Ann Thor Surg 2009;88:170
 PHN Study

546 children with a Fontan

Mean age, 12 yrs (6 – 18 yrs)
 Physical and psychosocial scores within 80% of normal
for healthy children, but point averages below the
mean for the normal population
Anderson et al, JACC 2008;52:85
Variable
Anderson
d’Udekem
% predicted peak VO2
65% + 16%
57% + 8%
% predicted peak VO2 at
anaerobic threshold
78% + 25%
78% + 16%
↓ percent predicted peak VO2
↓ percent predicted peak VO2 at anaerobic threshold
d’Udekem et al, JTCVS 2009;88:1961
Author
IQ
Uzark1
97.5 + 12.1
Goldberg2
Total Cohort
HLHS
Non-HLHS
101.4 + 5.4
93.8 + 7.3
107 + 7.0
Wernovsky3
95.7 + 17.4
1. Pediatrics 1998;101(4):630-3
2. J Pediatr 2000;137:646-52.
3. Circulation 2000;102:883-9
 Overall, the functional outcomes of Fontan patients are
excellent
 The majority of both parent and self-reported QOL
domains are lower than those score of the reference
population and their siblings, but are still within the
range of normal
 While peak exercise parameters are below normal, the
majority of Fontan patients subjectively report good
QOL in most physical domains
 Levels of physical activity are lower than normal, but
are not related to peak exercise thresholds, suggesting
that these limitations are self-imposed
 Neurocognitive outcomes, as measured by IQ, appear
to be well within the range of normal
 The “modern” Fontan era provides 20-25 year follow-up
Majority of patients are doing well within this time
frame
 Structural issues will be relatively few
 Dysrhythmias will increase over time
 Functional problems will become more prevalent with
age
 The use of transplant will increase
 The need for assist devices will become paramount
