Transcript Feigenbaum`s Echo

Azin Alizadehasl, MD
ECHOCARDIOGRAPHY
IN SARCOIDOSIS
Sarcoidosis is a systemic
inflammatory disease of unknown
etiology, characterized by non-caseating
granulomas. It mainly affects people in
the 3th and 4th decades of life, but may
also be found in children or elderly
subjects.
Braunwald heart disease:
Sarcoidosis:
RWMA
Thining : basal of posterior and lateral walls
Posterobasal aneurysm
Restrictive morphology
PAH and right side failure
Feigenbaum’s Echo:
 The 2D echo features of myocardial
involvement occur in less than 20% of
sarcoid patients.
 Heart is involved in as many as 50%
of advanced cases.
 LVE+RWMA(base and mid levels).
Otto:
 Cardiac involvement include:
pericardium, conducting system
myocardiom ( microscopic focal
infiltration or larger nodules).
 Basal posterior and lateral walls
and septum.
 MR is not uncommom.
 RWMA is often in a location
inconsistent with unusual coronary
anatomy.
 Disseminated sarcoidosis: DCM
 There are no specific echo findings
in cardiac sarcoidosis.
Heart involvement has been
reported in as many as 58%
of pts and may be responsible
for up to 85% of deaths due
to sarcoidosis .

 Autopsy studies and various types
of examination reveal a high
prevalence of heart involvement,
 Clinical manifestations and
symptoms are rare (about 5% of
cases).
They include advanced AVB,
malignant ventricular
arrhythmia, MR due to
papillary muscle dysfunction,
pericarditis, CHF and
SCD.
 No
single test or investigation
can detect specific abnormalities
enabling diagnosis.
 Various methods including
CMR, thallium-201 and gallium
have been suggested as valuable
diagnostic tools.
 Various forms of echocardiography have
been tried and features such as septal
thinning, LV regional systolic dysfunction,
pericardial effusion and commonly LV
diastolic dysfunction determined, but these
abnormalities only seem to be present in
advanced heart involvement.
Pts and controls had
similar LV diameters,
wall thicknesses, LA
size, eFS and EF.
Doppler echo evidence
of LV diastolic
dysfunction was
detected in 33 (55%)
pts.
 mFS(mid wall fractional
shortening) was determined
using a prolate ellipsoidal model
of LV geometry;
 LV wall is divided into inner
and outer shells with equal
thickness at diastole.
Sarcoid pts had lower
mFS
than controls
(15.8 ± 2.4% vs. 18.8 ±
2.5%, p=0.001.
Pts with diastolic
dysfunction had
lower mFS.
Pts with a longer history of the
illness had lower mFS
(16.6 ± 2.8% vs. 15.1 ± 1.9%, p
= 0.024).
Follow-up of our pts
will show whether a
lower mFS may have
prognostic value in
these pts.
This index may be
predictive of
initial heart
involvement.
Other Studies’ results:
LV systolic dysfunction and RWMA
in pts with severe heart involvement.
when the disease only involved the
conduction system echocardiography
was substantially normal.
Pts with diastolic dysfunction
may have had granulomas in the
myocardial interstitium, which
could stiffen the myocardium
and damage ventricular
Relaxation.
 TDI demonstrated impaired
longitudinal strain and strain
rate of several lateral LV
segments.
 2D echo showed loss of wall
thickness and hypokinesia of
these segments.
PAH
o The frequency of PH is ≈20%.
o The presence of PH
contributed to poor outcomes
in pts with sarcoidosis.
Feigenbaum’s Case
Case report(JACC 2008):
A 59-year-old woman with sarcoidosis
was admitted to hospital for assessment
of AVB3. Echo showed RV dilation and
dyskinesis of the apical free wall,
whereas LV study showed normokinesis,
mimicking RV dysplasia.
Granulomas are most frequently
located in the LV free wall (96%),
followed by the interventricular
septum (73%); whereas, the atrial
wall is rarely affected (right,
11%; left, 7%).
Case report(AJC 2009):
 40% of CS pts had
increased echoes(local scaring)
and hypokinesis of the
proximal part of interventricle
septum.
Case report,Circ-research2007