Download Congenital Absence of the Left Pericardium and Complete Heart Block

Congenital
Absence
Complete
of the Left Pericardium
Heart
Block*
Report
PHILIP
VARRIALE,
New
T
a Case
Rossi,
PI.INIo
M,D.,**
of
York,
and
M.D.f
W. J.
AND
New
GRACE,
M.D.,
F.C.C.P4
York
palpable.
The rhythm
was
regular.
A grade
II/VI
ejection
systolic
murmur
was audible
along
the left sternal
border
with maximal
intensity
in
the second
and third intercostal
space.
Splitting
of the
second
sound
occurred
on
inspiration.
There
was slight accentuation
of the
pulmonic
was
HE
RECOGNITION
AND
DEFINITIVE
nosis of absent
pericardium
impetus
within
recent
years.
described
for
many
years
anomaly
found
tion,
it was
reported
not
the
at
post-mortem
1959
case
of the pericardium
was
successfully
of
with
concomitant
picted
with
proper
15.7
mg
pericardium
white
tial
count
in
terms
of
diagnosis,
and
the
and
The
ventricular
cardiogram
with
was
an
showed
cardiogram
also
block
done
normal
cardiac
to
1).
the
left
of
The
atrioventricular
heart
was
showed
abnormal
prominence
(Fig.
urea
all within
displaced
apparent
contour
complete
were
film
an
and
of
and
Differen-
cent;
per
blood
sugar
x-ray
fingers
hemoglobin
9,500/mm3.
blood
heart
the
strong.
Urinalysis,
Chest
with
a
of 46
of
vascularity
hemithorax
treat-
count
fasting
limits.
contour.
left
showed
normal.
on abdominal
of
were
hematocrit
cell
and
normal
and
paris stressed
prognosis
pulses
was
pulmonary
underscored.
The
differentiation
of complete
tial deficiency
of the pericardium
Peripheral
cent;
blood
nitrogen
deand
is presented
techniques
clubbing
studies
per
palpable
was
Moderate
Laboratory
block
radiologic,
studies
diagnostic
present.
was
the diagnosis
by diagnostic
heart
organ
examination.
absence
left
angiographic,
No
component.
et al’
life.
deficient
complete
hemodynamic
Ellis
of congenital
during
case
examinathat
in which
established
pneumothorax
This
has gained
Although
well
as a curious
until
first
DIAG-
the
electro-
dissociation
(Fig.
(Fig.
2).
A
vector-
3).
ment.
Right
CASE
This
St.
29-year-old
Vincent’s
ical
white
Hospital
evaluation
and
heart
hemodynamics
REPORT:
man
on
of
an
29,
abnormal
electrocardiogram
admitted
was
April
1964
for
chest
noted
x-ray
during
a
catheterization
(Table
showed
1).
Selective
normal
angiography
to
med-
#{149}
film
routine
‘!
Li
examination.
He
was
the
age
the
Armed
sumably
16
and,
years
Forces
that
this
was
The
symptoms.
failed
at
to
examination,
cardiac
in
good
past
murmur
later,
physical
of
he
a heart
of
two
because
stated
no
first informed
of
pre-
murmur.
health
medical
pass
He
and
history
offered
was
nega-
Hg,
pulse
tive.
The blood
was 60 per
ture
cyanosis
were
or
flat
clear.
the
in
lary
sixth
line
eFrom
diology,
ter
of
intercostal
produced
the
Departments
St.
New
distress.
Vincent’s
Cervical
position.
apical
and
and temperadeveloped,
with
well
was
recumbent
cardiac
mm
130/88
regular,
respiratory
the
in
The
was
and
He
normal.
was
no
pressure
minute
impulse
space
was
and
a moderate
of
Hospital
Lungs
were
palpated
anterior
axil-
lift. No
Medicine
veins
thrill
and
and Medical
RaSignificant
features
include
leftward
of the
heart
without
tracheal
deviaaddition,
three
distinct
convexities
are
noted
along
the left border
of the heart.
These
are the aortic knob, pulmonary
artery
segment
and
the left ventricular
contour.
Cen-
FIGURE
York.
**Assistant
Attending,
Department
of Medicine.
of Special Procedures,
Department
of
diology.
Director,
Department
of Medicine.
fChief
1:
displacement
tion.
In
Ra-
405
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406
VARRIALE,
:
11
ROSSI
AND
11.1. II#{149}..I.l
2:
the
Conventional
The
ventricular
leads
occurs
block.
precordial
was
performed
displaced
to
right
anterior
ventricle
half
flow
tract
artery
of
were
posterior
the
AP
4).
left
of
appeared
the
the
of
the
(Fig.
the
normal.
right
lead
rate
between
The
the
I
(junctional
V, and
tricuspid
shows
atnioventricular
ir 48
per
and
was
tion
cardium
and
occupied
shadow.
The
out-
left
ventricle
was
valve
the
The
transitional
film.
Hence,
technique
silhouette
in
posi-
air
and
He
well.
complete
complex
of
was
was
pen-
on
chest
studied,
with 500
left pleural
noted
absent
findings
patient
the
using
between
and
the
pericardium
(Fig.
and
of
ml
cavity
asymptomatic
parietal
discharged
was
of
the
pneumothorax,
into
of
visceral
by
the
of
collection
possibility
The
suggested
air introduced
occupied
in
origin.
was
x-ray
the
pulmonary
normal
with
QRS
dissociation
minute.
V,.
The
and
aorta
rhythm)
spine.
of the cardiac
The
i
electrocardiogram
ventricle
The
L.Ii:’I1:I1i
vertebral
large
cardiac
portion
projection.
12
of
the Chest
I1r
I
-I
FIGURE
heart
Diseases
GRACE
a
right
5).
has
been
F
DISCUSSION
H
Congenital
pericardial
dominantly
complete
left-sided.
deficiency
or as a partial
left
atrium.’
The
ascribed
LS
left
to
defect,
pulmonary
defects
They
of the
usually
artery
left-sided
premature
predilection
the
or
left
has
been
development
of the
membrane,
atrophy
of
pre-
overlying
segment,
to incomplete
pleuropericardial
are
present
as either
left pericardium,
the
secondary
left
duct
of
Cuvier.1
Strict
3:
The
horizontal
plane
shows
a CCW
with
posterior
displacement
of the maximum
vector.
A prominent
S loop is present. The
loop
in the frontal
plane
is CCW.
The initial portion
is to the left and
slightly
inferior
and
terminal
activation
occurs
toward
the right.
The
left saggital
planar
projection
depicts
.a CCW loop
with
predominant
posterior
displacement.
(The direction
of inscription
is denoted
by the
sharp
end
of the tear-drop).
FIGURE
loop
QRS
QRS
tween
complete
differentiation
must
partial
pericardial
variety.
In the
be
made
be-
defect
and
the
latter,
the pericar-
dial and pleural
space
form a common
cavity and the heart,
displaced
toward
the left,
assumes
an abnormal
anatomic
position
within
the
chest.
In
ciency,
the
heart
maintains
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017
partial
pericardial
defi-
its normal
posi-
Volume
52,
September.
is also
ventricle
tion
CONGENITAL
The
tricuspid
displaced
to the
is also noted.
in the
cardial
ings
of
3
4A:
FIGURE
ity
No.
1967
a
chest
cavity.
may
be
left
hilar
left
valve
and
and
resides
Here
the
either
ABSENCE
is displaced
extends
to
within
chest
normal,
mass
or
the
x-ray
or
apparent
OF
to the left
the outermost
LEFT
of
PERICARDIUM
the vertebral
contour
of
pen-
ment
of
find-
there
is herniation
suggestive
enlarge-
pendage
In
the
the
407
spine.
heart.
pulmonary
case,
artery
of
through
this
The
right
Enlargement
the
the
left
ventricular
of the
cavright
segment
if
atrial
ap-
of the
left
defect.3’4
complete
absence
-t-.
FIGURE
4C
4BFIGURE
FIGURE
4 B and
C:
The
interventricular
septum
assumes
a parallel
body.
The
right
ventricle
occupies
the anterior
half
of the heart,
and
This
is a consequence
of an absent
pericardium
which
causes
cardiac
(pseudo-levorotation).
course
with
the
the left ventricle,
rotation
to the
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017
frontal
plane
of the
the posterior
half.
left
and
posteriorly
408
VARRIALE,
TABLE
1-DATA
Pressures
OF
in mm
Right
atrium
Right
ventricle
Pulmonary
CARDIAC
31/5
26/9
artery
(12)
left
(17)
Content
Rest
artery
Brachial
artery
Exercise
12.58
12.12
18.47
18.54
saturation
oxyhemoglobin
blood
94.3%
liters/mm
blood
Systemic
Stroke
rate
volume
Hydrogen
Dye
uptake
dilution
2.61
50
56
95 ml.
was
findings
on chest
most
striking
feature
FIGUGE
5A:
Diagnostic
the right
was
right
parietal
ing
by
two
roentgenograms.
related
pneumothorax:
penicardium
to
signifiThe
an
abnor-
Patient
(arrow).
Another
diagnosis
the
lying
in
The
heart
shows
along
however,
pneumothorax.
air into
a distinct
left
visceral
chest
is the
left
lateral
border.
separating
the
pericardium.
to the demaccord-
right
posterior
roentgenograms.
In this
decubitus
displacement
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017
ob-
This
with the patient
lying
a horizontally
directed
supine.
further
the left
pneumo-
cardiac
air
most
favorable
pneumopericardium,
et al,’
the
aortic
artery
segment
contour.
technique
that
permits
is the lateral
roentgenognam
patient
the
the
and
be performed
left side, with
suggested
of
a
three
confirmed,
as
of the
with
of the
of left
discernable
to Ellis
lique
was
along
This
noted
pulmonary
ventricular
500
ml
produced
The
position
onstration
of
85 ml.
studies-normal
cant
outlines
2.17
studies-normal
penicardium
cardium
position.
5.15
flow
L/min/M’
Ventricular
4.27
left
left,
Secondly,
consisting
utilization
of
space
the
were
diagnosis
after
to
position.
border,
pericardium
flow
heart
convexities
Injection
pleural
Arterial
Systemic
the
cardiac
only
in
Per Cent
Volumes
of
tracheal
The
Oxygen
the Chestof
knob,
elongated
and
flattened
(10)*
Pressure
Pulmonary
shift
distinct
29/9
(8)
Pulmonarywedge
*Mean
5
28/5
Diseases
GRACE
normal
Exercise
5
AND
mal
CATHETERIZATION
Rest
Hg
ROSSI
position,
position.
to
the
can
on the
beam.
accurate
with
the
heart
Pneumoperileft in
this
Volume
No.
52,
September,
3
CONGENITAL
1967
is displaced
posteriorly
the left pericardium
mothorax
is
study
if the
of the
an
against
is deficient.
diagnosis
of the
ing
the
heart,
of complete
on
definitive
cardium.
This
diagnosis
added
technique,
with
of
have
cluding
with
of
congenital
congenital
has
Despite
not
Stokes
may
be
the
pre-
disease,
been
presence
patient
There
attack
in-
broncho-
pulmonary
lobe.4
kind
of conduction
previously
was
described
or
these
combined
completely
no
been
described
and
one
history
dyspnea.
asympof
Most
absence
and
defects
been
described.
left
pain
pectoris
is
death
a serious
fall
through
a
mechanical
reported,
as
sequelae
Adams-
gical
patients
the
be
absence
of partial
other
herniation
of
left
pen-
ominous
argue
would
or
to oc-
the
Complete
A
activity
expected
possible
defect
hand,
strangula-
of
of cardiac
not
correction.#{176}
sudden
foramen.’#{176}”
These
exists.
and
of
with
pericardial
if complete
memay
output
cases
a result
would
cardium
to
activity
in cardiac
Two
restriction
of
leading
been
strangulation
in
have
incarceration
heart
left
exists
and
of cardiac
syncope.
com-
the pericarpotential
of
always
appendage
produce
the
more
pericardium
Transient
have
to
indistinguishable
absence
of
the frightful
atnial
of
have
pain,
related
perhaps
of the
restriction
tion
cases
chest
was
sudden
chanical
death
pericardium
position.”3
with
partial
Moreover,
small
pain
chest
mon
the
the
body
the
unexplained
which
angina
syncope
of
Several
with
in
in
dium.3
409
asymptomatic.
from
cur
of
was
been
subsequent
deficiency.
the
our
tomatic.
peri-
anomalies,
heart
pericardial
lesions,
absent
this case was
heart
block,
genic
cysts,
and aberrant
To our knowledge,
this
disorder
in promot-
congenital
origin.
Pericardial
been
described
in association
a variety
complete
have
Recurrent
change
however,
PERICARDIUM
with
hand,
deficiency,
particularly
appendage
herniates
border.3’4”3”4
interest
in
of complete
association
sumably
defects
of
LEFT
changes
other
of no value
conclusive
in partial
when
the
left
atrial
beyond
the pericardial
Of
deficiency
positional
was
of
substantiated.
the
abnormal
but
a
method
is to be
Angiocardiography,
delineated
the spine
if
Left pneu-
indispensable
pericardium
OF
ABSENCE
for
its sur-
deficiency,
not
require
on
surgical
intervention.
Cardiac
parent
enlargement
than
the
left
the
leftwai-d
real,
Clinically,
a
may
complete
have
influenced
be
myocardial
the
of
of
heart.
manifested
heart
block
actual
increased
increased
absence
beyond
slow
heart
ap-
consequence
of
impulse
The
with
more
complete
as
this
apical
The
be
displacement
boundary.’3
sent
with
pericardium
forceful
of the
sequent
may
as
its
rate
a
normal
associated
in this
case
enlargement
may
by dint
stroke
volume,
with
subdiastolic
length
of the
fibers.
life
span
of most
pericardium
compromised.
The
pericarditis
as
genital
defect
mately
75
patients
is probably
per
with
not
occurrence
ab-
seriously
of
pleuro-
a complication
of
this
has
in
approxi-
cent
been
found
of
cases.’1
con-
REFERENCES
FIGURE
Posterior
retrosternal
5B:
Translateral
displacement
space.
supine
of the
view
heart
of
and
the chest.
increased
1 ELLIS,
K.,
“Congenital
cardium,”
N. E. AND HIMMELSTEIN,
deficiencies
in the
parietal
Am.
J. Roent.,
82:125,
1959.
LEEDS,
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017
A.:
pen-
VARRIALE,
410
2
3
4
5
6
7
8
ROSSI
PERNA,
G.:
“Sopra
un arresto
di sviluppo
della
sierosa penicardica
dell’uomo.”
Anat.
Anz.,
35:
323,
1909-1910.
TUCKER,
D. H., MILLER,
D. E. AND JACOBY,
W. J.: “Congenital
partial
absence
of the
pencardium
with henniation
of the left atrial
appendage,”
Am.
J. Med.,
35:560,
1963.
BAKER,
W.
P.,
SCHLONG,
H.
A. AND
BELLENGER,
F. P.:
“Congenital
partial
absence
of
the pericardium,”
Am.
J. Cardiol.,
16:133,
1965.
KAVANOGH-GRAY,
D.,
MUSGROVE,
E. AND
STANWOOD,
D.:
“Congenital
penicardial
defects,”
New
Engi.
J. Med.,
265:692,
1961.
RUSBY,
N.
L. AND
SELLORS,
T.
H.:
“Congenital
deficiency
of pericardium
associated
with
bronchogenic
cyst,” Brit.
J. Surg.,
32:357,
1945.
Osooor,
R. AND SPECTOR,
B.:
“Defective
pencardial
sac
and inter-atnial septum
and atresia
of
pulmonic
orifice,”
Am.
J. Di:.
Children,
61:1028,
1941.
JONES,
P. H.:
“Developmental
defects
in
lungs,”
Thorax,
10:205,
1955.
C0,-O,
The original
phy
has
been
contrast
subfaclal
of the
after
planes
of
mediastlnum
demonstration
of
of
the
CO,
medlastinum.
are
then
the
to
0,
as
dissect
the
Polytomograms
for
detailed
made
Instances
been
of
resulted
per-
and
respiratory
refiux.
symptomatic
tract
in
of
patients
proved
87.5
per
13
14
surgical
therapy
for
surgery
resistant
in
include
to
in
patients
medical
Cardiovasc.
“Incomplete
Surg.,
pericardial
into left
28:209,
pleural
1887.
S. AND
“Congenital
penicardial
6:167,
1944.
SOUTHWORTH,
H.
“Congenital
defects
sac:
cavity,”
Tr.
AND
Brit.
STEVENSON,
of pericardium,”
J.:
R.
Heart
WRIGHT-SMITH,
defects,”
es-
Obst.
J.,
C.
Arch.
S.:
mt.
Med.,
61:223,
1938.
FOWLER,
N. 0.: “Congenital
defect of the pericardium,”
Circulation,
26:114,
1962.
ROGGE,
J. D., MISHKIN,
M. E. AND GENOVESE,
P. D.:
“Congenital
partial
penicardial
defect
of
herniation
of the
left
atnial
appendage,”
Ann.
mt. Med.,
64:137,
1966.
For reprints, please write:
11th Street,
New
York
City
Dr. Grace,
10011.
153
West
POLYTOMOGRAPHY
cent
technically
successful
examinations
with
no significant
morbidity
or mortality.
It is emphasized
that
pathologic
processes,
grossly
similar
but
histologically
dissimilar,
cannot
be differentiated
by
method.
S.,
A.
P. M.
ilcINs,
88:519,
AND
bronchogenic
restoring
with
clinically
URSCHEL,
HIATAL
F.:
#{149}C0-O
for
the
pre-
Radiology,
carcinoma.”
HERNIA
who
demonstrate
Reconstruction
on the
technique
provement
53:21,
of
W.
BUGDEN,
polytomography
1967.
ment
refiux.
based
refiux
AND
with
evaluation
diagnosis
treat-
and
SUNDERLAND,
REFLUX
Cinefluorography
useful
R.:
heart
London,
operative
secondary
to cardioesophaor without
hiatal hernia.
the etiology
of esophageal
complications.
has
Indications
12
Thorac.
BOXALL,
cape
of
Soc.,
11
AND
BOLL,
of the pen-
deficiency
1960.
pneumomediastinography
suspected
GASTROESOPHAGEAL
Gastroesophageal
refiux
geal incompetence,
with
is a significant
factor
in
of the
esophagus
and
evaluation
10
BERNE,
has
J.
40:49,
J. S.
C.,
WILSON,
“Congenital
JR.:
candium,”
this
anatomy.
CO,-O2
pneumomedlastinography
formed
in this
fashion
in 64
bronchogenle
carcinoma.
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A.
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R. E.,
WITH
CO, pneumomediastlnograby the
Injection
of
injecting
Diseases
of
the Chest
GRACE
PNEUMOMEDIASTINOGRAPHY
technic
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and
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the gastroesophageal
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PAULSON,
hernia.
1967.
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