Download Sub- and supravalvular aortic stenoses.

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Congenital Aortic Valve
Disease and Aortopathy:
Recent Advances
Sub- and Supravalvular
Aortic Stenosis
Westfälische
Wilhelms-Universität
Münster
Helmut Baumgartner
Adult Congenital and Valvular Heart Disease Center
University of Muenster
Germany
Subaortic Stenosis
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Operated or unoperated up to 6% of pts. seen in ACHD clinics
Frequently (up to 60%) associated with
other lesions:
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–
–
–
VSD
AVSD
Coarctation of the aorta
Shone syndrome (coarctation, parachute
MV, supravalv. mitral ring)
Subaortic Stenosis Pediatric Considerations
• „Acquired heart disease“
usually not present at birth but appears after
first year of life (anatomic precursor + genetic?)
•
Observation of rapid progression and increasing
presence and severity of AR
 Early surgery even in mild disease?
•
Brauner R at al J Am Coll Cardiol 1997;30:1835
However, progression varies widely and in
particular mild disease frequently progresses
slowly; less effect of surgery on AV disease than
originally thought Coleman DM et al J Am Coll Cardiol 1994;24:1558
 More conservative in mild subaortic stenosis
(gradient < 30mmHg)
Gersony WM J Am Coll Cardiol 2001
Subaortic Stenosis Recurrence After Surgery
•
Overall recurrence rate 15 - 27%
More frequent in fibromuscular than in
discrete (membranous) SAS
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Re-operation rate 12 - 20%
Recurrence rate depends on
– Surgical technique
– Extent of obstruction relief at operation
– Residual gradient ≥ 30mmHg
-> high risk of recurrence
Subaortic Stenosis Recurrence After Surgery
Risk factors for reoperation after repair
for discrete subaortic stenosis
Geva A et al
J Am Coll Cardiol 2007;50:1498-504
• < 6mm distance between AoV and obstruction
HR 5.1
• Peak gradient by Doppler ≥ 60mmHg
HR 4.2
• Peeling of membrane from Ao or MV
Adult Patients with
Subaortic Stenosis
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•
Not diagnosed during childhood
•
Residual obstruction after surgery during
childhood
•
Recurrent obstruction after surgery
during childhood
•
Aortic valve disease (regurgitation)
Recognized but not operated on during
childhood
Echo Diagnosis
Subaortic Stenosis When to (re)-intervene?
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•
Symptoms related to (re)stenosis
-> mean gradient expected > 50mmHg
reduced exercise capacity
shortness of breath
angina
diziness, syncope
Severe aortic valve disease (AR)
Surgery in symptomatic pts. and
asymptomatic pts. + LV enlargement
and/or LVEF < 50%
Subaortic Stenosis When to (re)-intervene?
Asymptomatic pt. (without severe AR) PROGNOSTIC CONSIDERATIONS
• Consequences for aortic valve (AR)
• Consequences of LVOT obstruction with pressure
load for LV
– LVH
– LV myocardial fibrosis
– Arrhythmias
– LV dysfunction
– Sudden death
Subaortic Stenosis When to (re)-intervene?
• Consequences of LVOT obstruction with pressure
load for LV
– LVH
– LV myocardial fibrosis
– Arrhythmias
– LV-dysfunction
– Sudden death
Pts. with unusually profound
LVH (unproportional to stenosis
cardiomyopathy?)
LVH regression after surgery
HEART VALVE PROSTHESES
Subaortic Stenosis When to (re)-intervene?
(Re)-intervention in asymptomatic
patients: AV considerations (AR)
JET DAMAGE
Subaortic Stenosis When to (re)-intervene?
(Re)-intervention in asymptomatic
patients: AV considerations (AR)
Discrete Subaortic Stenosis
in Adults
Oliver JM et al
J Am Coll Cardiol 2001;38:835-42
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134 pts. (31 ± 17yrs, 64 females)
6.5% of pts. with CHD at study period
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Age
A: 56±15 yrs B: 27±13 yrs C: 21±4 yrs
Associated lesions (VSD, AVSD, CoA a.o.)
A: 7%
B: 64%
C: 44%
•
Group A (N=29) surgery during adult life
Group B (N=64) unoperated
Group C (N=41) surgery < 15 years of age
Discrete Subaortic Stenosis
in Adults
Oliver JM et al
25 pts. with 2 exams, average interval 4.8±1.8 yrs
Gradient increase 7.6±14mmHg; 2.3±4.7mmHg/yr
Age ≥ 50 yrs
LVOTO (mmHg)
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•
J Am Coll Cardiol 2001;38:835-42
Age < 50 yrs
120 -
P = NS
100 80 60 40 20 -
P = 0.01
0-
Initial Follow-up
Initial Follow-up
Discrete Subaortic Stenosis
in Adults
Oliver JM et al
•
J Am Coll Cardiol 2001;38:835-42
Presence and degree of AR in with (C) and without
surgery during childhood (A+B)
80 60 40 20 0
P = 0.03
3-
AR Degree
Mean ± SD
AR (Percent)
100 -
210-
Group C
N=41
trace to mild
Group A+B
Group C
N=93
N=41
mild to moderate
Group A+B
N=93
moderate to severe
Discrete Subaortic Stenosis
in Adults
Oliver JM et al
AR Degree
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•
J Am Coll Cardiol 2001;38:835-42
25 pts. with 2 exams, average interval 4.8±1.8 yrs
Change in AR degree over time 1.3±.8 1.5±.9
3-
1 = trace to mild
2 = mild to moderate
3 = moderate to severe
P = 0.096
210
1.3±
0.8
Baseline
1.5±
0.9
Follow-up
AR degree sign. related
to LVOTO (p <.001)
but not age (p =.055)
4 pts. with endocarditis!
Discrete Subaortic Stenosis
in Adults
Oliver JM et al
•
J Am Coll Cardiol 2001;38:835-42
Prevalence is increasing (greater number of
repaired CHD)
• LVOTO increases but very slowly, particularly at
age < 50 years
Average age for surgical repair > 50 years
•
• AR is very common but rarely hemodynamically
significant
AR shows usually little progression over time
•
• AR is more prominent in pts. after surgery
Subaortic Stenosis and AR
• Surgical relief of LVOTO improves AR
Serraf A et al
J Thrac Cardiovasc Surg 1999;117:669
• Progression in severtiy of AR not significantly different in
surgical and nonsurgical groups
Giuffre RM et al
Adv Ther 2004;21:322-8
• No substantial change in AR during follow-up after surgery
Stassano P et al
Thorac Cardiov Surg 2005
Karamlou T et al
Ann Thorac Surg 2007:84
• Late worsening of AR related to initial gradient (>30mmHg)
• Predictors: small distance to AV, higher gradient, peeling
of the membrane from the AV
Geva A et al
J Am Coll Cardiol 2007:50
• Surgery did not have impact on the incidence and severity
of AR
Drolet Ch et al Can J Cardiol 2011:27
Baumgartner H et al
Eur Heart J 2011
Patel B et al J Am Coll Cardiol 2010;56
Salahuddin S et al Heart 2010;96:1808
Supravalvular AS
• By far rarest obstructive lesion of the LVOT
• Histology: diseased media with an increased
collagen content and reduced elastic tissue in
the form of broken and disorganized elastin
fibers (elastin arteriopathy)
normal Aorta
thickening
irregular arrangement
of elastic lamellae
Stamm C et al
Eur J Cardio-thoracic Surg
Supravalvular AS (SVAS)
• SVAS associated with Williams syndrome
• SVAS as inherited, autosomal dominant
familial form without the nonvascular
features of Williams syndrome
-> elastin gene deleted or disrupted ±
neighboring genes
• Sporadic cases of isolated SVAS
 hemizygous microdeletion on chromosome
7q11.23 identified in all three
Supravalvular AS (SVAS)
• Abnormalities of
the AV in up to 50%
• In appr. 30% entire
ascendig aorta,
sometimes arch
• Obstruction of the
pulmonary vasculature
in up to 83% (all three
forms of SVAS)
• Coronary arteries:
- adhesion of the cusp leaflet
edge to the narrowed STJ
- obstr. by thickened aortic wall
- high pressure -> premature
arteriosclerosis
Stamm C et al
Eur J Cardio-thoracic Surg
Supravalvular AS (SVAS)
Other Associated Lesions
• Aortic coarctation
• Patent ductus arteriosus
• Atrial septal defect
• Ventricular septal defect
• Tetralogy of Fallot
• Mitral valve abnormalities (elastin defect??)
Stamm C et al
Eur J Cardio-thoracic Surg
Kaushal et al Ann Thorac Surg 2010
Aortic Regurgitation
Cardiac Outcomes in Adults With
Supravalvular Aortic Stenosis (SVAS)
Greutmann M, Tobler D, Sharma NC, Mebus S, Schuler P, Beauchesne L,
Salehian O, Hoffmann A, Oechslin EN, Silversides CK. ESC 2011
• 8 ACHD centers N = 113 >18yrs
• Cardiac Events: CV death, SVT/VT >30s, ACS,
Stroke, new onset CHF, endocarditis
Surgery during adulthood
•
• Age at 1st visit: 20 ± 4 yrs
Williams-Beuren Syndrome
SVAS surgry during childhood
Multiple operations
NYHA ≥ II
RBBB
≥16mmHg residual peak ∆P
≥50mmHg residua peak ∆P
> mild AS / > mild AR
> mild MS / > mild MR
55%
67%
34%
8%
11%
45%
6%
10/10%
3/4%
Cardiac Outcomes in Adults With
Supravalvular Aortic Stenosis (SVAS)
Greutmann M, Tobler D, Sharma NC, Mebus S, Schuler P, Beauchesne L,
Salehian O, Hoffmann A, Oechslin EN, Silversides CK. ESC 2011
• 8 ACHD centers N = 113 >18yrs
• Cardiac Events: CV death, SVT/VT >30s, ACS,
Stroke, new onset CHF, endocarditis
Surgery during adulthood
•
• Age at 1st visit: 20 ± 4 yrs
Williams-Beuren Syndrome
SVAS surgry during childhood
Multiple operations
NYHA ≥ II
RBBB
≥16mmHg residual peak ∆P
≥50mmHg residua peak ∆P
> mild AS / > mild AR
> mild MS / > mild MR
55%
67%
34%
8%
11%
45% (more likely in WBS)
6%
10/10% (less likely in WBS)
3/4%
Cardiac Outcomes in Adults With
Supravalvular Aortic Stenosis (SVAS)
Greutmann M, Tobler D, Sharma NC, Mebus S, Schuler P, Beauchesne L,
Salehian O, Hoffmann A, Oechslin EN, Silversides CK. ESC 2011
• Follow-up of 96 pts.
• 20 events:
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median FU 6 yrs (0.1 – 30yrs)
- death
2
- SVT / VT
8 (7/3)
- new onset CHF 7
- endocarditis
2
- stroke
1
Surgery
- AVR
5
- SVAS repair
4
- PVI
1
- LV assist device 1
Predictors of events: multiple surgery, NYHA≥II, sign. MV disease
Predictors of surgery: BWS, RBBB, >50mmHg gradient
Baumgartner H et al
Eur Heart J 2011
Balloon Dilatation in Discrete
Subaortic Stenosis
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Feasibilty shown (gradient reduction, no
increase in AR)
Very small patient numbers
Residual gradient of concern
No sufficient follow-up
Rao PS et al
J Invasive Cardiol 1990;2:65-71
Sharma S et al
J Interv Cardiol 1991;4:105-9
Moskowitz WB et al J Invasive Cardiol 1999;11:116-20