Download Tricuspid Valve

Sail Sound in Ebstein's Anomaly of the
Tricuspid Valve
By MARY E. FONTANA, M.D., AND CHARLES F. WOOLEY, M.D.
With the technical assistance of Richard S. Goodwin, MS., and George F. Rieser,
M.T.
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SUMMARY
Ebstein's anomaly represents an anatomic, pathologic, and physiologic spectrum.
There have been few hemodynamic correlates for the observed auscultatory events.
Multiple components of the first sound and "ejection" sounds are frequently described.
Cardiac catheterization, intracardiac sound-pressure studies (Telco), and cineangiograms were performed in three patients with Ebstein's anomaly who had a prominent
early systolic sound.
The right ventricular pressure pulse was abnormal in all; an initial delta-wave configuration, followed by a more rapid pressure rise, produced a prolonged rise to peak
pressure. The right ventricular pressure pulse is not that of a conduction defect alone;
rather it suggests that the altered pattern of ventricular contraction and abnormal
leaflet placement are contributing factors.
The early systolic sound was recorded in the atrialized right ventricle or right
ventricle in all. It occurred just after the peak of the c wave in the atrialized right
ventricle. In the right ventricle the sound occurred at the point where initial slow delta
portion of right ventricular pressure pulse gave rise to rapid upstroke. The early systolic
sound most likely occurs when the large, sail-like tricuspid valve reaches the limit of
systolic excursion. The sound has been designated as the "sail sound," and may be
the most specific auscultatory event in Ebstein's anomaly.
Additional Indexing Words:
Phonocardiography
Heart sounds
Heart murmurs
T HE DIAGNOSIS of Ebstein's anomaly
of the tricuspid valve has been made
during life with increasing frequency since the
early 1950s. Attempts have been made to
define a "typical" clinical picture and auscultatory pattern for this defect.'-25 The most
common findings are those of a variably
cyanotic patient with a multiplicity of heart
sounds with or without murmurs. Many
descriptions have been made of split first heart
sounds, early and midsystolic clicking or
metallic sounds, and ejection sounds as part of
9,20
the auscultatory complex.-7 9 1
Recently we have had the opportunity to
study three patients with Ebstein's anomaly
who have a prominent early systolic sound,
and we have made some observations regarding its origin. We have also noted striking
changes in the morphology of the right
ventricular pressure pulse, and will discuss
these changes together with the systolic sound
as they appear to be integrally related.
From the Department of Medicine, Division of
Cardiology, The Ohio State University College of
Medicine, Columbus, Ohio.
Supported in part by Graduate Training Grant
320327, Clinical Research Center Grant RR-34, and
Career Research Development Award HE-28,243
from the U. S. Public Health Service.
Address for reprints: Dr. Mary E. Fontana, Division of Cardiology, Room 203, University Hospital,
410 West 10th Avenue, Columbus, Ohio 43210.
Received March 29, 1971; revision accepted for
publication February 3, 1972.
Circulation, Volume XLVI, July 1972
Auscultation
Case Reports
Case 1
D. C., a 31-year-old white male, was admitted
in April, 1969. The diagnosis of Ebstein's
155
FONTANA, WOOLEY
156
Table 1
Catheterization Data
(2) a 4, c = 6, v =4
RA* (mm Hg)
22/0-5
RV (mm Hg)
MPA* (mm Hg)
19/5 (8)
(4)
PC* (mm Hg)
99/62 (75)
LBA* (mm Hg)
LA* (mm Hg)
95
Systemic 02 sat (%)
2.19
CI (liters/nin/M2)
Indicator
Small R->L shunt
curve-RA
Case 3
Case 2
Case 1
Parameter
(5) a-5, v = 7
22/6
6
(3) a = 8, c = 8, v
24/3
20/6 (9)
(4)
112/68 (82)
(6) a
110/65
= 12, v
=
9
92
1.80
86.5
2.78
Small R->L shunt
L-.R & R->L shunt
*Mean pressures in parentheses.
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anomaly had been confirmed by previous catheterization and cineangiographic studies. His
symptoms included only fatigue and dizziness.
On physical examination, he had a webbed neck,
pectus excavatum, and was mentally slow. His
blood pressure was 120/80, pulse 45 and regular.
There was no cyanosis or clubbing. The apical
impulse was not palpable. The first sound was
followed by a loud, clicking, nearly midsystolic
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sound. The second sound was persistently split. A
short grade II/ VI early systolic murmur was
present along the left sternal border. There was
an S3 at the apex. The remainder of his physical
examination was normal. ECG showed sinus
bradyeardia, left-axis deviation, complete right
bundle-branch block with a QRS of 140 msec,
and ST-T wave changes. Chest X-ray showed a
moderately enlarged, globular-shaped heart with
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Figure 1
External phonocardiogram of case 1. The microphones are placed at the fourth interspace
at the left sternal border and at the apex, both at a frequency cutoff of 100 Hz. The early
systolic sound (SS) is the loudest sound present, is recorded well by both microphones, and
occurs well after the first sound and the peak of the carotid pulse. The third heart sound (S3)
was only intermittently recorded. The QRS duration is 140 msec.
Circulation, Volume XLVI,
July
1972
SAIL SOUND IN EBSTEIN'S ANOMALY
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a
a
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Figure 2
External phonocardiogram of case 2. The microphones are at the third interspace at the
left sternal border and at the apex, with a frequency cutoff of 200 Hz. The early systolic
sound (SS) is the loudest sound and is well recorded by both microphones. It occurs on the
upstroke of the carotid pulse (allowing for delay in transmission of the carotid pulse). The third
sound (S3) and fourth sound (S4) are also weU shown. The delta wave of the Wolff-ParkinsonWhite syndrome can be seen in the QRS complex, which is of 110-msec duration.
right atrial enlargement. Pertinent catheterization
data are shown in table 1. His physical
appearance suggested Turner's syndrome, but
further studies were not done. There was no
family history of congenital anomalies.
Case 2
D. C. is a 35-year-old white male who was
admitted in April, 1970. The diagnosis of
Ebstein's anomaly was made in 1964 by
catheterization and cineangiography. Since then
he had had some increase in dyspnea and
cyanosis. He was taking digoxin for control of
paroxysmal tachycardia. On physical examination, his blood pressure was 102/84, pulse 76 and
regular. He appeared dusky, with cyanosis of his
nailbeds and early clubbing. There was no neck
vein distention. No precordial pulsations were
Circulation, Volume XLVI, July 1972
visible or palpable. Auscultatory findings, with
the help of phonocardiography for timing, were:
normal first sound, loud early systolic sound,
normal second sound, third sound, and fourth
sound. There were no murmurs. The remainder of
his physical examination was normal. ECG
showed Wolff-Parkinson-White syndrome, type
B, with a QRS duration of 110 msec. Chest X-ray
was normal. Catheterization results are shown in
table 1.
Case 3
R. K. was a 15-year-old white male admitted to
Ohio State University Hospital in August, 1967.
He had been cyanotic at birth, but outgrew it. He
always fatigued easily, yet worked on a farm
doing hard labor. A diagnosis of Ebstein's
anomaly was entertained in 1960 on the basis of
FONTANA, WOOLEY
158
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physical examination, ECG, and X-ray. Catheterization studies were not done. In March, 1967, he
had a syncopal episode on exertion, and was
placed on digoxin. On physical examination, he
was well developed, well nourished, and only
faintly cyanotic. Blood pressure was 110/ 65,
pulse 72 and regular. A rolling apical impulse
extended from the sternum to the anterior axillary
line in the sixth intercostal space. An apical
systolic thrill was present. The sequence of heart
sounds was: first sound, early systolic sound,
second sound, third sound, fourth sound, with a
grade IV/VI holosystolic murmur and grade
II/VI diastolic rumble at the apex. There was also
a grade III/VI to-and-fro rublike murmur in the
second left intercostal space. There was no
clubbing. The remainder of his physical examination was normal. ECG showed broad, peaked P
waves, prolonged A-V conduction, bizarre RBBB
pattern with a QRS of 180 msec, and ST-T wave
changes. Chest X-ray showed massive cardiomegaly with right atrial and right ventricular enlargement, decreased pulmonary vascularity, and a
77
I
I
small pulmonary artery. Catheterization data are
shown in table 1. Right atrial cineangiography
confirmed the diagnosis of Ebstein's anomaly and
right-to-left shunt at the atrial level.
About 1 year after he was studied, he died
suddenly. An autopsy done at Columbus Children's Hospital demonstrated massive right atrial,
atrialized right ventricular, and right ventricular
enlargement, and patent foramen ovale. The
septal and posterior cusps of the tricuspid valve
were displaced well into the right ventricle, and
the anterior leaflet was huge (see fig. 7). The
right atrium and atrialized right ventricle were
paper thin.
Methods
Intracardiac sound and pressure recordings
were obtained in all three patients using a Telco
micromanometer-tipped catheter. Recordings
were made with a Sanbom 560 recorder and are
shown at a paper speed of 100 mm/sec. The
frequency response of the system was flat to 200
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External phonocardiogram of case 3. The microphones are at the third interspace at the left
sternal border, lower left sternal border, and at the apex. All are at a frequency cutoff of 200
Hz. The early systolic sound (SS) is well recorded by the lower two microphones, as well as
systolic (SM) and diastolic (DM) murmurs. The systolic sound occurs at the peak of the carotid
pulse in this patient. The third sound (S3) and fourth sound (S4) were recorded by the apical
and 3LSB microphones, rsepectively. Note the bizarre QRS configuration, which has a duration
of 180 rmec.
Circulation, Volume XLVI, July 1972
SAIL SOUND IN EBSTEIN'S ANOMALY
1.1
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right ventricle
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atrialized RV
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Figure 4
Intracardiac sound and pressure recording in the RV (left panel) and atrialized RV (right panel)
of case 1. The early systolic sound (SS) is recorded in both locations and occurs at the point
of transition from slow to rapid pressure rise of the RV pressure pulse and at the beginning
of an abrupt negative deflection following the "c" wave of the atrialized RV pressure pulse
(vertical dashed lines). The duration of pressure rise in the RV is markedly prolonged, although
the rise begins immediately after the R wave of the QRS complex.
Hz. External phonocardiograms were recorded
using Sanborn contact crystal microphones with
350-1700 B heart sound preamplifiers. External
carotid pulses were recorded with Sanborn
P23Db transducers. A transmission delay of about
30 msec is assumed in using the carotid pulse as
a reference tracing. The recording of a right
ventricular intracavitary ECG simultaneously
with an atrial pressure pulse, and demonstration
by cineangiography of the displaced tricuspid
leaflets were used to confirm the diagnosis.
Cineangiography was performed in the right
anterior oblique projection, injecting contrast into
the right atrium.
Results
The catheterization data are shown in table
1. All three patients had normal right heart
and pulmonary capillary pressures. The cardiac output was slightly depressed in case 1,
and very low in case 3. Case 2 had significant desaturation of 86.5% from right-toleft shunting across the atrial septum. The leftto-right shunt through the defect was 1.3:1,
determined by the nitrous oxide inhalation
Circulation, Volume XLVI, July 1972
method. Cases 1 and 3 also had right-to-left
shunting, demonstrated by indicator-dilution
curves performed within the right atrium. The
left atrium was entered in case 3.
The phonocardiograms of the three patients
are illustrated in figures 1, 2, and 3, respectively. The pertinent feature of all three
is the prominent early systolic sound (SS),
transmitting widely, occurring well after the
first heart sound. This sound bears no constant
relationship to the simultaneously recorded
carotid pulse.
Intracardiac sound recordings demonstrated
that this early systolic sound was recorded
within the right ventricle and atrialized right
ventricle in case 1 (fig. 4), but not within
the true right atrium. The sound was recorded
within the right ventricle, atrialized right
ventricle, and pulmonary artery in case 2
(fig. 5), and within the atrialized right
ventricle only in case 3 (fig. 6).
FONTANA, WOOLEY
160
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Figure 5
Pressure-sound correlates in the RV, atrialized RV, and main pulmonary artery (left to right
panels, respectively) of case 2. From top to bottom-external phonocardiogram with the
microphone at the lower left sternal border with frequency cutoff of 200 Hz; intracardiac sound
in the respective locations, corresponding pressure pulse, and ECG. The external carotid pulse
is also included in the right panel. The early systolic sound (SS) is well recorded by both external microphone and catheter in all three locations. It coincides with a change in rate of rise
in the markedly prolonged upstroke of the RV pressure pulse, the abrupt negative deflection
following the "c" peak in the atrialized RV, and the change in rate of pressure rise in the
pulmonary artery. The beginning of RV pressure pulse rise is at the peak of the R wave of the
QRS, which has a "delta" configuration of the Wolff-Parkinson-White syndrome.
The right ventricular pressure pulse was
abnormal in all three patients (figs. 4-6).
There was a very slow rise to peak pressure. In cases 1 and 2, there was a slow
initial rise in pressure, followed by a more
abrupt rise, coincident with the occurrence of
the early systolic sound (figs. 4 and 5). This
change in the rate of pressure rise was not
present in case 3 (fig. 6), in whom a
holosystolic murmur consistent with tricuspid
regurgitation was recorded within the atrialized right ventricle. Pertinent relationships of
the QRS, early systolic sound, and RV
pressure pulse are indicated in table 2.
The timing of the early systolic sound
relative to the atrialized right ventricular
pressure pulse is also pertinent. In all three
patients the sound occurred at the beginning
of an abrupt negative deflection following the
peak of the c wave (figs. 4-6). There
is considerable distortion of the atrialized
right ventricular pressure pulse by tricuspid
regurgitation in case 3 (fig. 6), so that the
negative deflection is less prominent.
Discussion
Other authors have ascribed the early systolic sound in patients with Ebstein's anomaly
to a splitting of the first sound with a delayed
tricuspid closure.2 47, 9-11, 16, 17, 19, 20, 23 No
simultaneous intracardiac sound and pressure
studies are present in the literature to support
or disprove this impression. The studies presented here provide a more direct approach
to delineate the source of the early systolic
sound.
Circulation, Volume XLVI, July 1972
SAIL SOUND IN EBSTEIN'S ANOMALY
161
Recording the sound within the right
ventricle and/or atrialized right ventricle in all
three patients suggests that the sound is
related to the tricuspid valve. Defining the
event responsible for the sound requires analysis of the right ventricular and atrialized
right ventricular pressure pulses and their
relationship to the sound.
The right ventricular pressure pulse showed
a prolonged duration of pressure rise varying
from 135 msec in case 2 to 214 msec in
case 3. In two cases there was an abrupt
increase in the rate of pressure rise coincident
with the occurrence of the early systolic
sound. This change in the rate of rise of the
right ventricular pressure pulse has been
described previously by Van Lingen et al.25
and Pocock et al.19 Pocock also related a
"third component of the first heart sound" to
the change in pressure rise, but did not do
intracardiac sound and pressure studies. This
third component is very likely equivalent to
our sail sound.
An explanation of this coincidence of sound
and pressure events is that with the
onset of ventricular systole the abnormal
tricuspid valve leaflets are carried in an atrial
direction, actually counteracting the decrease
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Figure 6
Case 3. From the top down, respirometer, external phonocardiogram from the lower left
sternal border and apex, respectively, both at a frequency cutoff of 200 Hz; intracardiac sound
and pressure within the RV (left panel), and atrialized RV (right panel), and ECG. The systolic
sound (SS) recorded by the external microphones is not recorded within the RV. The RV
pressure pulse shows a very slow rise to peak pressure beginning just after the peak of the R
wave of the QRS complex, but there is no abrupt change in the rate of rise of pressure at the
time that the systolic sound (SS) occurs. The sound (SS) is recorded within the atrialized RV
as well as a holosystolic murmur. The atrialized RV pressure pulse is distorted by the regurgitant jet, but the sound does occur at the time of an abbreviated negative deflection after the
"c" wave. The peaked P waves and bizarre QRS pattern are also evident.
Circulation, Volume XLVI, July 1972
FONTANA, WOOLEY
162
Table 2
Relationships of QRS, Sail Sound, and RV Pressure
Case
QRS
(msec)
Q-rise
Q-S1
Q-SS
1
2
3
140
110
180
39
54
64
150*
98
211
160
231
126*
Rise-SS
Rise-SS
rise-PP
184
172
93%
135
214
106
167
78%
78%
Rise-PP
*Beginning of first sound difficult to delineate.
Abbreviations: SS = sail sound; PP = peak pressure in RV; rise = beginning of pressure
rise in right ventricle.
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in size of the contracting functional right
ventricle. Therefore, the pressure rises slowly.
When the leaflets reach their limit of excursion
toward the atrium, the ventricle more efficiently ejects blood out the pulmonary artery, as
indicated by the abrupt rise in pressure
development in the RV pressure pulse. The
pressure is seen to rise more abruptly in the
pulmonary artery as well, following a notch at
the occurrence of the sound, as shown in
figure 5. Case 2 was the only one in which
the pulmonary artery was entered with the
manometer-tipped catheter. Although the
sound occurs on the upstroke of the pulmonary artery pressure pulse and, therefore,
could represent a pulmonic ejection sound, the
sound was consistently recorded in the RV or
atrialized RV, and was of maximum intensity
there, which would be more consistent with a
tricuspid valve origin.
The systolic sound, then, occurs when the
tricuspid leaflets reach their limit of atrial
excursion, are maximally tensed, and produce
a sharp sound, similar to that of a sail
snapping in the wind. The lack of abrupt rapid
pressure rise in the right ventricle following
the sound in case 3 is thought to be the
result of tricuspid regurgitation allowing
continued decompression of the ventricle
throughout systole. The sound may not
be a "closure" sound of the tricuspid
valve, since the sound is clearly present
in this case with considerable regurgitation, where the leaflets may not approximate at all. The sound is probably more
related to the termination of motion of the
leaflets. Crews et al.3 have recently support-
ed this impression by describing a sudden
halting of motion of the tricuspid valve, using
ultrasound, at the time of the occurrence of a
high-frequency early systolic sound, whihc we
believe is equivalent to the "sail sound"
described here.
Further support for the above theory is the
occurrence of the sound just after the peak
pressure of the c wave in the atrialized right
ventricle, the abrupt negative deflection being
produced by the recoil of the leaflets toward
the ventricle as the ventricle rapidly ejects
blood.
The cineangiographic findings are also
compatible with the hypothesis. The right
atrial cineangiogram performed on patient 2,
who had a normal-sized heart, was of
sufficient clarity that the abnormally placed
leaflets could be defined and their movement
timed. During atrial systole, the leaflets were
bowed well into the ventricle. With the onset
of ventricular contraction, the leaflets could be
seen to move toward the atrium. At the time
of the occurrence of the early systolic sound,
the leaflets appeared to have reached the
point of maximum excursion toward the
atrium, with no further movement during the
remainder of ventricular systole. This sequence could not be confirmed from the
cineangiograms of the other two patients
because of the marked cardiomegaly, contrast
material obscuring the leaflets, and the lack of
timing devices when the cineangiograms were
performed.
The use of the term "sail sound" can be
better understood by reviewing the anatomy
of the tricuspid valve in Ebstein's anomaly.
The artist's drawing of our third patient's
Circulation, Volume XLVI, July 1972
SAIL SOUND IN EBSTEIN'S ANOMALY
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tricuspid valve (fig. 7) shows that the anterior leaflet is a huge structure which is
attached to the true annulus, looks very much
like a sail, and has been described in these
terms by others.8' fl, 1,8 23 This huge structure
period is extremely short, or even nonexistent.
small functional right ventricle to move during
systole. If the systolic sound does indicate the
limit of systolic excursion of the leaflets, then
they are moving in an atrial direction 78% to
93% of the time that the ventricle takes to
reach peak pressure in our three patients, as
shown in table 2. Therefore, the isovolumic
period is extremely short, or even nonexistent.
Note that the pressure has already begun to
rise in the pulmonary artery of case 2
:; -,1
APM
Figure
7
Artists drawing of the heart of case 3, viewed anteriorly with the right ventricular wall reflected. The
anterior cusp (AC) is a huge, "sail-like" structure
originating from the true annulus. The small medial
(MC) and posterior (PC) cusps originated well below
the true tricuspid annulus. Abbreviations: CA
arteriosus;
conus
MB = moderator
band;
APM
papillary muscle; RA = right atrium; ARV
atrialized RV; RV right ventricle; AA = aortic
anterior
arch;
P
pulmonary artery; RPA,
LPA =
left pulmonary arteries; SVC = superior
Ra
right
atrial
pendage; LV
-
appendage;
left ventricle.
Circulition, Volume XLVI, July 1972
La
=
left
right and
vena cava;
atrial
ap-
163
(fig. 5) when the sound occurs. This could
be a major contributing factor in the inefficiency of right ventricular ejection common to
many patients with Ebstein's anomaly.
Several authors have commented upon the
slow rate of pressure rise in the right ventricle
of patients with Ebstein's anomaly, ascribing
the delay primarily to the right ventricular
conduction abnormalities often seen with this
lesion." 11, 15, 19, 23-25 However, Johnson and
co-workers26 performed micromanometer
studies in a patient with isolated intermittent
right bundle-branch block and demonstrated
the delay to be between the beginning of the
QRS and the beginning of pressure rise when
the bundle-branch block was present. In our
patients the Q-rise time (table 2) was actually
shorter than the normal values reported by
Johnson (39-64 msec vs 65-86 msec). The
prolongation was clearly during the upstroke,
which was of normal duration in Johnson's
patient. Our patients also exhibited similar RV
pressure pulses despite markedly different
QRS durations and configurations. Therefore,
we must conclude that the conduction defect
is not the sole determinant of the morphology
of the RV pressure pulse in Ebstein's anomaly.
The occurrence of the sail sound and the
right ventricular pressure pulse abnormalities
can both, then, be related to the movements of
the abnormal tricuspid leaflets. A direct cause
and effect relationship between the sail sound
and the behavior of the tricuspid leaflets
cannot be proved by these studies, but the
correlation of sound, pressure, and cineangiographic data makes the explanation here a
likely one.
Acknowledgment
The authors acknowledge Theodore H. Celet, M.D.,
for his help in performing the studies presented here,
and Jeffrey Fierra, M.D., for the drawing shown as
figure 7.
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164
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Circulation, Volume XLVI, July 1972
Sail Sound in Ebstein's Anomaly of the Tricuspid Valve
MARY E. FONTANA, CHARLES F. WOOLEY, Richard S. Goodwin and George
F. Rieser
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Circulation. 1972;46:155-164
doi: 10.1161/01.CIR.46.1.155
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