Clinicopathological

Case Conference

Shock and Pulmonary Edema

GERARD B. MARTIN, MD,* KIRK JENSEN, MD,t
RICHARD M. NOWAK, MD**

CASE PRESENTATION
Dr.Kirk Jensen:
A 6%year-old white woman was transferred to the University of Chicago Emergency Department
with the diagnosis of pulmonary edema and cardiogenic
shock.
She was well until approximately two weeks previously,
when she experienced several transitory episodes of weakness and palpitations, each lasting seconds to minutes. She
denied having chest pain, shortness of breath, diaphoresis,
or loss of consciousness.
On the day of admission the patient had an episode of
syncope lasting several minutes. There was no associated
chest pain, shortness of breath, or diaphoresis. Upon regaining consciousness,
the patient noted a generalized
feeling of weakness and called the emergency dispatch service. A Chicago Fire Department ambulance subsequently
transported her to a local community hospital.
Upon her arrival at the community hospital, her vital signs
were as follows: temperature,
38C; pulse, 100 beats per
minute; respiratory rate, 24 per minute; blood pressure, 901
60 mm Hg.
On examination the patient was awake, alert, and somewhat pale. There was jugular venous distension at 40 elevation. A loud midsystolic murmur was heard. Inspiratory
rales were heard over both lungs.
The electrocardiogram
was interpreted as showing left
ventricular hypertrophy, and the chest radiograph was read
as showing pulmonary edema (Fig. 1). The complete blood
count and levels of serum electrolytes, glucose, and cardiac
enzymes were reported as normal.
The patient had several episodes of what was believed to
be supraventricular tachycardia, marked by restlessness and

From the *Department

of Emergency Medicine, Henry Ford Hospital, Detroit, Michigan,
the TUniversity
of Chicago Hospitals,
Chicago,
Illinois,
and the *Education
committee,
Michigan
Chapter, American College of Emergency Physicians.
Presented at the Ninth Annual Michigan
Emergency
Assembly in Traverse City, Michigan, July 1982.

Medicine

Address reprint requests to Dr. Nowak: Education Committee,

Michigan Chapter, American College of Emergency Physicians,
1305 Abbott Road, Suite 109, East Lansing, Ml 48823.
Key Words: Cardiogenic
shock, hypertrophic
cardiomyopathy,
idiopathic
hypertrophic
subaortic stenosis (IHSS), mitral regurgitation.
232

obtundation. Approximately one hour after the patients arrival in the community emergency department, a sinus arrest
developed (Fig. 2). At that point a temporary transvenous
pacemaker was inserted, followed by infusions of dopamine
and norepinephrine for persisting hypotension. After an unsuccessful one-hour attempt to locate a cardiologist, a decision was made to transfer the patient.
Three hours after the patients arrival at the community
hospital, a call was placed to the University of Chicago
Emergency Department to inform the senior resident that
the patient was enroute via private ambulance; she arrived
half an hour later.
Further questioning of the patient revealed a history of
acute rheumatic fever in childhood. requiring extended hospitalization. There were no subsequent difficulties. Eight
years before the present illness, routine physical examination disclosed a heart murmur. Two years before the present
illness, she had an episode of weakness and diaphoresis. She
was hospitalized and a pacemaker was suggested, but the
patient refused. She had a forty pack/year history of smoking
and a history of hypertension,
but was currently not receiving any medications. There was a family history of hypertension but no history of diabetes mellitus or coronary
artery disease. There were no recent operative procedures.
When the patient arrived at the University of Chicago
Emergency Department, her temperature was 37.9C; pulse,
100 beats per minute; respirations, 22 per minute; and blood
pressure, 84/58 mm Hg.
On examination the patient was seen to be thin, awake,
alert, and somewhat pale. The skin was moist without rash
or petechiae. A temporary transvenous pacemaker and two
peripheral intravenous lines were in place. The neck was
supple, with a brisk carotid pulse. There was no evidence
of thyromegaly.
The jugular veins were distended 5 cm
above the sternal notch at 30 elevation. Inspiratory rales
were heard over the lower half of both lung fields. A prominent PM1 was palpable at the sixth intercostal space, 3 cm
to the left of the midclavicular line. There was a palpable
thrill, and a harsh, grade 4/6 holosystolic murmur was heard
best at the apex, with radiation to the axilla and to the left
upper sternal border. A soft, short, diastolic murmur (grade
2/6) was heard at the apex and left lateral sternal border. An
S, was heard at the apex, but no S, was appreciated. The
abdominal examination was noncontributory.
The extremities were warm, without clubbing, cyanosis, edema, or inflammation. The neurologic examination showed no abnormalities.

MARTIN ET AL B CARDIOGENIC SHOCK AND PULMONARY EDEMA

An electrocardiogram
demonstrated a sinus rhythm with
a rate of 100 beats per minute, with evidence of left atria1
enlargement and left ventricular hypertrophy. The chest radiograph demonstrated pulmonary edema. The hematocrit
was 32%, the leukocyte count was 13,300lcu mm, and the
differential count was as follows: bands, 1%; polymorphonuclear cells, 87%; lymphocytes, 5%; monocytes, 5%, and
eosinophils, 2%. Coagulation; levels of serum electrolytes,
urea nitrogen, creatinine, glucose, calcium and magnesium;
and results of urinalysis were all normal. Serum enzyme
levels were as follows: creatine phosphokinase,
14 mIUlm1;
lactate dehydrogenase,
63 units; SGOT, 41 Karmen units/
ml; SGPT, 26 Karmen units/ml; and alkaline phosphatase,
64 units/dl. Blood for culture was obtained.
Analysis of arterial blood gases, with the patient breathing
room air and blood being taken from the radial artery,
showed a pH of 7.45, a Po2 of 53 mm Hg, and a Pco2 of 29
mm Hg.
In the Emergency Department, the norepinephrine
infusion was discontinued,
with no change in pulse or blood
pressure. The dopamine infusion was continued, and 40 mg
of furosemide was administered intravenously, with a rapid
onset of diuresis. Forty-five minutes after the patients arrival, atria1 fibrillation occurred, with a ventricular rate of
90 beats per minute, followed by atrial flutter (atria1 rate,
300; ventricular rate, 150). At this point the patients blood
pressure was 78/54 mm Hg. Digoxin, 0.125 mg, was administered intravenously,
with a subsequent conversion to a
normal sinus rhythm.
The patient was admitted to the coronary care unit, where
a Swan-Ganz catheter was inserted. Later that night an
intro-aortic balloon pump was used for persistent hypotension.
The diagnosis was confirmed the next day.

DISCUSSION
This case involves
the
Dr. Gerard Martin:
problem of acute pulmonary
edema and hypotension
in an elderly woman with syncope,
fever, and heart
An organized approach to this patients
murmurs.

problems with emphasis on differential diagnosis and

pathophysiology will be presented.
Syncope of cardiac origin can be divided into two
major groups: arrhythmia- and nonarrhythmia-related.
Arrhythmia-related
causes include supraventricular
tachycardias (SVT), ventricular arrhythmias, second
and third degree heart block, and sick sinus syndrome.
Aortic stenosis, hypertrophic
cardiomyopathy
(previously and less accurately known as idiopathic
hypertrophic subaortic stenosis),* and atria1 myxoma
are structural abnormalities that may also lead to syncope through a temporary decrease in cardiac output.
The history of transient episodes of weakness accompanied by palpitations is suggestive of an arrhythmia.
This is further supported by the episodes of SVT accompanied by restlessness and obtundation. At one
point there was sinus arrest of unspecified duration.
This combination of supraventricular tachycardia and

FIGURE 1. Initial radiograph demonstrating

pulmonary edema.

sinus arrest is characteristic

of the sick sinus syndrome .
The term sick sinus syndrome was first used by
Lown3 to describe patients whose hearts failed to
achieve a stable sinus rhythm following cardioversion
for supraventricular arrhythmias. Since then this syndrome has come to denote a variety of arrhythmias
indicative of sinus node dysfunction, including the following:4
1. Chronic and persistent sinus bradycardia without
significant change in sinus rate during stressful situations and exercise.
2. Cessation of sinus activity for short periods of
time, with no escape rhythm, or for longer periods,
with atria1 or junctional escape rhythms.
3. Long periods of sinus arrest without appearance
of any escape rhythm, thus resulting in atrioventricular standstill.
4. Inability of the heart to resume sinus rhythm following cardioversion for chronic atria1 fibrillation or
flutter.
5. Episodes of sinoatrial exit block not related to
drug therapy.
6. Episodes of supraventricular
tachycardia in addition to, or terminating in, long asystolic pauses
(brady-tachy syndrome).
The patient in this case is a member of the bradytachy subgroup.
Moss and Davis5 reviewed the records of 74 patients with the brady-tachy syndrome and
found it to be a disease of the elderly with an average
age of 68 years. Coronary artery disease and idiopathic
causes together accounted for 85% of associated and
possibly etiologic cardiac diseases in this study. However, there is controversy concerning the relationship
between coronary artery disease and the sick sinus
233

AMERICAN JOURNAL OF EMERGENCY MEDICINE n Volume 2, Number 3 n May 1984

FIGURE 2.

Sinus arrest while patient was at community hospital.

syndrome. Engel et al6 found no angiographic evidence of sinus node artery involvement in five of six
patients with sick sinus syndrome and coronary artery
disease. Fibrosis from any cause has been proposed.,8
as a final common pathway in most patients. This
seems likely, since a wide variety of conditions have
been reported in association with sick sinus syndrome.
The symptomatology has been well documented; syncope and near-syncope
are the most common presenting symptoms. Palpitations and increased congestive heart failure occur less frequently.5*9
In this case, it is unlikely that sick sinus syndrome
alone was the cause of the acute pulmonary edema
and hypotension, since there was no improvement in
the patients clinical status once the cardiac rhythm
had stabilized. Instead, the patient had a continued
downhill course with findings indicative of another origin of her acute illness.
The causes of acute cardiogenic pulmonary edema
include arrhythmias, high-output states, severe hypertension, acute myocardial infarction, ventricular or arterial left-to-right shunts, cardiomyopathy,
and valvular disease. Arrhythmias, as mentioned, are not totally responsible
for this patients condition. The
common causes of high-output states include thyrotoxicosis, beri-beri, anemia, and systemic arteriovenous listulas. There is no evidence to suggest that the
first two conditions are responsible, and although the
patient is somewhat anemic, with a hematocrit of 32%,
the anemia is not severe enough to explain her clinical
findings. Systemic arteriovenous Iistulas often occur
secondary to trauma or surgery. Increased pulse pressure, a key tinding,iO is absent in this patient. Severe
hypertension cannot be the cause of the pulmonary
edema in this hypotensive patient without evidence of
severe, long-standing hypertensive heart disease.
Acute myocardial infarction or ischemia with resulting left ventricular dysfunction is unlikely as a
cause of the problems. The patient gives no history of
angina or chest pain prior to this episode, although
silent myocardial infarctions have been well documented, particularly in diabetic patients. The electrocardiogram shows only left atria1 enlargement with left
ventricular hypertrophy and no evidence of ischemia
or infarction. Initial cardiac enzyme levels are reported as normal; subsequent levels almost four hours
later are still within normal limits. Since the creatine
phosphokinase level begins to rise within four to six
234

hours after an infarct,* some increase would be expected.

Acute ventricular septum rupture presents with the
abrupt onset of pulmonary edema and hypotension.*
It usually occurs, however, during the first week after
an acute transmural myocardial infarction, an event
for which there is no evidence in this patient.
Cardiomyopathies
are considered in three major
groups based on abnormalities of structure and function: dilated congestive, restrictive/obliterative,
and
hypertrophic. The patient with congestive cardiomyopathy typically presents with increasingly severe
symptoms of left ventricular failure. Although these
patients may at times during their course present with
pulmonary edema, it is uncommonly the first manifestation of disease. Cardiomegaly on chest radiograph is
a characteristic finding, absent in this case.
Amyloidosis is the most common cause of restrictive cardiomyopathy in this part of the world. Amyloid
deposits in the heart extensive enough to cause such
cardiac dysfunction are virtually never isolated to the
heart. This patient has no evidence of systemic amyloidosis; thus, restrictive cardiomyopathy
is effectively eliminated.
Hypertrophic
cardiomyopathy
can mimic a large
number of cardiovascular disorders and is more difticult to exclude. However, several features make it less
likely in this case. The main symptoms of hypertrophic
cardiomyopathy
(HC) are chest pain and dyspnea;
syncope and palpitations are noted less frequently.
Also, patients with HC most commonly present with
symptoms at a young age. In their review of 85 patients with HC, Swan et d3 found the average age of
onset of symptoms to be 24 years.
Congestive heart failure occurs in a minority of patients with HC and is frequently related to an extensive myocardial infarct or the onset of atria1 fibrillation: two events for which there is no evidence in this
patient. Although compatible with HC, some of the
characteristic
electrocardiographic
findings are absent; these include left anterior hemiblock, Q waves
in the inferior and left precordial leads, and a short PR
interval. The midsystolic murmur present early in the
case is consistent with HC, but its progression to a
holosystolic murmur helps rule out that condition.
Therefore, acute valvular disease must be the cause
of this patients hypotension and pulmonary edema.
Patients with adult aortic valvular stenosis typically

MARTIN

have the onset of symptoms when the valve area has

become critically reduced from the normal 4 cm* to
about 0.7 cm* or less, with a systolic gradient exceeding 50 mm Hg or more if the cardiac output is
normal.14 The patient in this case is noted to have a
brisk carotid upstroke, which virtually eliminates
aortic stenosis as a possibility.
In contrast to aortic stenosis, the natural history of
mitral stenosis is marked by a gradual deterioration
over several years after the onset of symptoms. The
symptomatic decline of these patients commonly begins with the onset of atria1 fibrillation. In some patients, this event can precipitate pulmonary edema
with or without hypotension. The patient in this case
arrives in sinus rhythm, and atria1 fibrillation develops
only later; mitral stenosis is thus eliminated as a cause
of for her sudden decline.
Acute mitral regurgitation (AMR) is a well-recognized clinical entity with a characteristic picture similar to that in our patient. The abrupt onset of severe
congestive heart failure or sudden worsening of preexisting symptoms is typical. Acute mitral regurgitation is often associated with the new appearance of,
or an increase in the intensity of, a previously noted
pansystolic murmur. The persistence of sinus rhythm
and radiographic
evidence of a nearly normal-size
heart with severe pulmonary vascular congestion are
characteristic. I5
Acute mitral regurgitation
may be classified according to the part of the mitral apparatus involved
and the pathogenesis of the involvement. Almost all
cases of acute severe mitral regurgitation are a consequence of rupture of either the papillary muscles or
the chordae tendineae. Disorders affecting the mitral
leaflets are commonly associated with chronic mitral
insufficiency, but rarely with the acute severe regurgitation evident in this case.t5
The mitral valve consists of two leaflets anchored
by chordae tendineae to two papillary muscle groups
arising from the left ventricular myocardium. Approximately two thirds of the closure area of this valve is
contributed by the more mobile anterior, or aortic,
leaflet. The smaller, less mobile posterior leaflet is responsible for closing approximately one third of the
mitral orifice. The chordae tendineae, whose numbers
vary, are tough fibrous structures of fixed length that
attach the mitral valve leaflets to the papillary muscles. These muscles are located anterolaterally
and
posteromedially
and are firmly anchored to the left
ventricular wall by numerous muscular bands. The
chordae tendineae and associated papillary muscles
are parallel to the left ventricular wall and insert into
the mitral valve leaflets at nearly right angles, thus
providing maximal mechanical advantage. Mitral valvular competency depends on the anatomic, spatial,
and temporal integrity of the entire mitral valvular apparatus.

ET AL n CARDIOGENIC

SHOCK AND PULMONARY

EDEMA

Rupture of a papillary muscle most commonly OCcurs in the setting of a recent myocardial infarction.
The posteromedial muscle is more frequently involved
because of its less abundant collateral supply.* Rupture occurs within the first few days after an acute
infarct, after necrosis has developed but before the
muscle has become fibrotic. Transection of an entire
muscle almost always occurs in the setting of an inferior or posterior infarct.*.t6 This is incompatible
with survival, since each muscle has chordae to adjacent halves of both leaflets, and complete papillary
muscle rupture leads to massive mitral regurgitation
through approximately one half of the valvular orifice.
In immediate survivors, only one or a few of the several heads of a papillary muscle are ruptured. Acute
mitral regurgitation due to papillary muscle dysfunction from ischemia or infarction without rupture has
been described, with identical clinical pictures.17 In
this patient, there is no evidence to suggest infarction
or ischemia; papillary muscle rupture or infarction is
therefore unlikely.
Patients with hypertrophic
cardiomyopathy
frequently have some degree of mitral regurgitation because of the papillary muscle dysfunction that occurs
with the distortion of normal ventricular contraction
in this disease.s Abnormal bending caused by the
bulging ventricular septum results in excessive tension
on the chordae tendineae, preventing complete closure
of the mitral value orifice. Acute severe mitral regurgitation occurs infrequently with hypertrophic cardiomyopathy, making it a less likely possibility, as previously noted.
Rupture of the chordae tendineae was first described by Couvisart in the nineteenth century. Several published studies have appeared over the past two
decades, leading to a clearer understanding
of this
clinical entity. 19-*j Bacterial endocarditis is considered
by some22*26to be the single most common cause of
rupture chordae tendineae, while others believe that
spontaneous rupture is more common.27 Osmundson
et al** reported a series of 20 patients with ruptured
chordae tendineae in whom there was a clinical history
of bacterial endocarditis with positive blood cultures
or pathologic evidence of healed endocarditis in ten
patients. Three other patients had diagnostic findings,
and three had evidence suggestive of infective endocarditis at autopsy. Rheumatic endocarditis results in
shortened, thickened, fused chordae tendineae, which
are more susceptible to bacterial infection. Pre-existing rheumatic heart disease has been cited as a
cause of ruptured chordae tendineae in both the absence or presence of active or healed bacterial endocarditis.19*24,28The patient in this case has had a prior
episode of rheumatic fever, probably with some resultant mild mitral valvular disease, as indicated by
the left atria1 enlargement and left ventricular hypertrophy on electrocardiogram.
The absence of atria1 fi235

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brillation until late in her course and the lack of symptomatology rule out a severe, long-standing mitral valvular pathologic condition. The presence of fever, mild
leukocytosis, and anemia in this patient are compatible
with bacterial endocarditis.
The physical findings associated
with ruptured
chordae tendineae have been well described.*9,20 Examination of the jugular venous system reveals increased pressure with abnormally tall a waves.
There is a prominent left ventricular impulse and a
hyperactive precordium. An apical systolic thrill is frequently palpated, as in this case. There may be a systolic thrill at the base of the heart as well.
The character of the heart sounds are not noted in
this case but are important. The first heart sound (St)
is usually normal, but there is an increase in intensity
in the pulmonic component of the second heart sound
(P2). Physiologic splitting of the second heart sound is
present. Third heart sounds (S,) are typically present,
as in this case. Cohen et a129 found an atria1 gallop
sound (S,) in nine of 11 patients with ruptured chordae
tendineae and believed that it was a very important
diagnostic sign. The absence of an S, gallop in this
case indicates that other pathologic conditions may be
involved.
An apical systolic murmur is always present but
may have variable characteristics. The murmur is generally described as grade IV/VI, although softer murmurs may occur. Holosystolic murmurs are commonly
noted, but the murmur may be interpreted as starting
after S, and ending before S,, even though it may be
phonocardiographically
holosystolic.
The murmur
characteristically
is a plateau with a late systolic decrease in intensity. It may, however, appear as a crescendo-decrescendo,
and when associated with a basal
thrill, it may simulate aortic stenosis. The murmur frequently radiates to either the base of the heart and
cervical vessels or to the left axilla and posterior left
hemithorax .
Diastolic murmurs in cases of severe mitral regurgitation have been described and are thought to result
from a relative obstruction to flow of the large regurgitant volume across the finite mitral valvular orifice.
Since most studies of patients with ruptured chordae
tendineae do not report diastolic murmurs, other
pathologic conditions must be considered in this case.
Acute pulmonary edema is typically present on the
chest radiograph, as in this case. Sanders et d9 believe that the most useful sign in the diagnosis of ruptured chordae tendineae is the radiographic demonstration of a small left atrium in the presence of gross
mitral regurgitation.
The electrocardiogram
of the patient with ruptured
chordae tendineae provides no diagnostic signs. The
presence of left atria1 enlargement and left ventricular
236

hypertrophy are not specific and suggest some preexisting mild mitral valvular disease and hypertension.
The diagnosis of ruptured chordae tendineae can be
suspected clinically but is best confirmed with cardiac
catheterization.
Pressure measurements of the right
side of the heart with a Swan-Ganz catheter will show
the striking V wave, which may equal left venntricular
systolic pressure. The cardiac output and index are
uniformly low. Left ventricular angiography reveals
mitral regurgitation.
Two findings in this patient are especially notable:
the absence of an S, gallop and the presence of a diastolic murmur. The new appearance of a diastolic
murmur not initially heard suggests an ongoing destructive process. It may be secondary to severe mitral
regurgitation, as mentioned, but may also be due to
aortic insufficiency.
Acute mitral insufficiency
secondary to aortic valvular endocarditis has been reported by Edwards.30
The tendency for secondary mitral valvular involvement to occur is predicated on the close anatomic relationships between the aortic and mitral valves. The
anterior mitral leaflet lies immediately subjacent to the
aortic valve and may become infected by either of two
mechanisms. There may be direct extension of infection from the aortic valve onto the mitral leaflet or
impingement of regurgitant blood through the aortic
valve onto either the ventricular surface of the mitral
valve or the chordae tendineae. In either case, the
destructive process may have a greater effect on the
mitral valve than on the aortic valve, and the patient
may present with signs and symptoms primarily
suggestive of mitral regurgitation.
In the presence of both aortic and mitral regurgitation, an S, gallop would not be expected, since there
is no left ventricular presystolic tilling from the left
atrium. A short, soft diastolic murmur is common in
acute aortic insufficiency (AI), in contrast to chronic
AI. Tachycardia
is an extremely important sign in
acute AI, and its absence calls the diagnosis into question. In contrast to chronic severe AI, the pulse pressure widens little in acute AI.
The chest radiograph in acute AI provides important
negative information. The lack of left ventricular dilatation in this patient argues against long-standing AI.
Again, the electrocardiographic
findings of left atria1
enlargement and left ventricular hypertrophy do not
aid in the diagnosis.
The patient in this case thus appears to have acute
mitral regurgitation, apparently secondary to ruptured
chordae tendineae from acute valvular endocarditis.
There may well be aortic valvular involvement as well.
Such patients are difficult to treat. In this case, pulmonary edema and hypotension
should be initally
treated with diuretics to decrease the preload and do-

MARTIN ET AL W CARDIOGENIC SHOCK AND PULMONARY EDEMA

pamine to increase cardiac contractility. Sodium nitroprusside, a direct vasodilator, has proved beneficial in
the treatment of severe mitral regurgitation.32933 The
systemic vascular resistance is increased in these patients and augments the regurgitation. Reduction of
aortic impedance to left ventricular ejection by nitroprusside decreases the regurgitant flow and increases
the forward cardiac output. Norepinephrine,
a strong
vasoconstrictor
that therefore increases the afterload,
is contraindicated
because it serves only to increase
the regurgitant fraction. There was no change in blood
pressure after discontinuation
of the norepinephrine
infusion-further
evidence of its ineffectiveness
in
this case.
An intra-aortic balloon pump (IABP) has proven
useful in the treatment of intractible hypotension and
pulmonary edema secondary to acute mitral regurgitation.34p35Although the cited studies included patients
with papillary muscle rupture secondary to acute MI,
the basis of the beneficial effect of the IABP is similar
to that in patients with rupture of the chordae tendineae. The obvious difference between the groups is
the associated myocardial damage in patients with
papillary muscle rupture.
In systole, the balloon deflates just prior to aortic
valve opening and abruptly reduces aortic pressure,
thereby allowing the left ventricle to eject against a
greatly reduced arterial impedance. This reduces the
regurgitant flow across the mitral valve. By inflating
in diastole, the balloon displaces a volume of blood
equal to its own volume, thereby raising aortic diastolic pressure and increasing the regurgitant flow
across an incompetent aortic valve. Thus, the use of
an IABP in the present of aortic insufficiency is contraindicated .
Urgent cardiac catheterization with left ventricular
angiography is indicated in this case to confirm the
diagnosis and to assess the severity of valvular and
left ventricular dysfunction.
Once the diagnosis of
acute mitral regurgitation
secondary
to ruptured
chordae tendineae is made, the therapeutic options are
prosthetic valve replacement or repair of the ruptured
chordae. Although favorable long-term results after
valvuloplasty have been reported, these studies have
involved patients with spontaneous chordae tendineae
rupture, involving primarily the posterior cusp.36737
Most authorities agree that mitral valve replacement
is currently
the treatment
of choice for ruptured
chordae tendineae secondary to bacterial endocarditis,
particularly if it involves the anterior cusp.
Correct timing is the essence of surgical management of endocarditis. If the operation can be safely
delayed, antibiotic therapy should eradicate or at least
greatly reduce the population of organisms on the
valve, thus decreasing the chance of infection of the

prosthetic valve. If surgery is delayed too long, the

patient may die suddenly or the hemodynamic status
may deteriorate so much that operation is no longer
feasible. The hemodynamic status of the patient in this
case makes her a candidate for urgent mitral valve
replacement after the diagnosis is confirmed.
It must be mentioned that left atria1 myxomas are
notoriously difficult to diagnose clinically and must
also be considered in this patient. In 1951, Prichard3*
commented on the belief of many that the diagnosis
of cardiac tumors is either impossible or a matter of
chance. Since widespread use of noninvasive cardiac
imaging with echocardiography
and radionuclide scanning, diagnosis has improved but remains difftcult if
not impossible on the basis of clinical findings alone.
In a recent review from the Mayo Clinic,39 only five
of 40 atria1 myxomas were diagnosed on the basis of
history, physical examination,
chest radiograph, or
electrocardiogram.
In three of the five, a calcified
intra-atria1 mass was seen on chest radiograph, while
in the remaining two patients, the diagnosis was made
by histologic examination
of a specimen from an
acutely ischemic limb. Myxomas should be considered
in the differential diagnosis of any patient with suspected mitral valvular disease who is in sinus rhythm
with congestive heart failure or constitutional symptoms. The Mayo Clinic study found fever present in
30%, anemia in 40%, congestive heart failure in 70%,
and syncope in 23% of patients with myxomas. Other
authors have reported similar findings.40-42 The vast
majority of clinical diagnoses included mitral valvular
disease, acute rheumatic carditis, and bacterial endocarditis .
Although atria1 myxomas have been regarded as
classic simulators of mitral stenosis, recent reviews
have reported findings of mitral stenosis in only 20%
to 40% of patients. Apical systolic murmurs were
present in 40% to 50%. Wise43 reported a patient with
severe mitral regurgitation
secondary to ruptured
chordae tendineae from a calcified atrial myxoma visible only on fluoroscopy, not routine chest radiograph.
In view of the findings in this case and the difficulty
in diagnosing atria1 myxomas clinically, this tumor
must be suspected. Echocardiography
has proven to
be an effective noninvasive diagnostic aid, particularly
in patients with less severe symptoms not warranting
catheterization.
The patients hemodynamic status in
this case is an indication for catheterization
and angiography emergently. Direct left atria1 catheterization
is best avoided in this situation because of the risk of
dislodging tumor tissue. Instead, injection of contrast
material into the pulmonary artery and delayed filming
to show the left atrium is the best diagnostic procedure .
Dr. Nowak:
Dr. Babbs, given your expertise in
237

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FIGURE 3. Simultaneous left ventricular and radial arterial pressure tracings in a patient with obstructive hypertrophic cardiomyopathy
show the Brockenbrough response to a ventricular premature beat (VPB). The gradient across the left ventricular outflow tract is about 80
mm Hg. Following the VPB (indicated by arrows on the ECG and the LV pressure tracing), there is a compensatory pause. In the beat
following the compensatory pause, the arterial pressure falls despite an increase in the left ventricular systolic pressure, and the radial pulse
pressure narrows. (Reproduced from DeSanctis RW. Cardiomyopathies. In: Rubenstein E (ed). SCIENTIFIC AMERICAN Medicine. New
York: Scientific American, Inc. 0 1984 SCIENTIFIC AMERICAN Mrdicine. All rights reserved.)

CPR, if this woman with presumably acute valvular

disease had experienced cardiac arrest, what specifically would you do during CPR to optimize blood
flow?
Dr. Charles F. Babbs (Purdue University, West Lafayette, Indiana):
Its interesting that in a case of
valvular insufficiency, if one believes in the thoracic
pump mechanism of CPR, it may not matter whether
the mitral valve is competent. However, in valvular
stenosis there may be impairment of flow. Further, this
is one diagnosis that may not be completely ruled out
in this case. I agree that sick sinus syndrome does not
explain the pulmonary
edema with normal sinus
rhythm. I also agree that there is no evidence for acute
myocaridal infarction or ischemic heart disease. Idiopathic hypertrophic
subaortic stenosis (IHSS) is an
interesting possibility that cannot be totally ruled out.
There is a clinical bedside test for IHSS that entails
listening to the murmur during leg elevation. Most
murmurs due to valvular stenosis will become louder
with this maneuver because there is increased venous
return to the heart and greater flow through the stenotic region. However, the murmur of IHSS will become softer because the stenosis caused by the hypertrophic muscle is decreased when the left ventricle
dilates. This would have been an interesting test to
perform on this patient.
Dr. Dennis C. Whitehead (Marquette General Hos238

Its interesting that the

pital, Marquette, Michigan):
diastolic murmur was not recorded until after the patient had been receiving morepinephrine,
which, as
Dr. Martin has noted, is contraindicated in a case of
suspected aortic insufficiency. If the patient had suffered only minor damage to the aortic leaflets during
her childhood episode of rheumatic fever, a soft
murmur of aortic insufficiency could have been easily
missed. Thus, norepinephrine
would be the perfect
way to accentuate that murmur-in
exactly the way
you wouldnt want to, since it would exacerbate the
insufficiency.
Id like to make one other point. Heres a patient
with poor color who has pulmonary
edema, and
someone has determined arterial blood gases with the
patient breathing room air. I would not have withheld
oxygen therapy when she was acutely ill in order to
obtain a baseline set of arterial blood gases.
Clinical Diagnoses
Brady-tachy
ease.

syndrome,

rheumatic

mitral valve dis-

Dr. Martins Diagnoses

Acute valvular endocarditis, acute chordae tendineae rupture, acute mitral regurgitation, possible acute
aortic insufficiency.

MARTIN

CLINICAL COURSE
The patient was admitted to the coronary care unit and
a Swan-Ganz catheter was inserted. The pulmonary artery
pressure was 55730 mm Hg (n = 40), and the capillary wedge
pressure was 25 mm Hg. (No comment was made regarding
the V wave.) An echocardiogram
demonstrated a normal
septum and a calcified mitral ring. Blood cultures were negative for pathogenic organisms.
Cardiac catheterization revealed a cardiac output of 6.0 Y
min, with a normal cardiac index. Right atrial, right ventricular, and pulmonary capillary wedge pressures all showed
moderate elevation. Careful pullback across the left ventricular outflow tract demonstrated a definite gradient between
the left ventricular apex (225/30 mm Hg) and the subaortic
valvular region (85/30 mm Hg); a simultaneous radial artery
pressure was 85/55 mm Hg. After premature ventricular contractions, the gradient increased, a classic finding in IHSS
(Fig. 3).
A left ventricular angiogram and coronary arteriogram revealed three plus mitral insufficiency, severe stenosis at the
right coronary artery, and moderate stenosis of the left anterior descending artery. There was poor left ventricular
function.
The patient was later taken to the operating room, where
her mitral valve was resected and a Bjork-Shiley prostetic
valve was inserted. The ventricular septum was not touched.
The left anterior descending artery was bypassed, but the
surgeon was unable to bypass the right coronary artery. A
permanent pacemaker was inserted.
Postoperatively, the systolic blood pressure promptly rose
to 120 mm Hg. No vasopressors were required. The patient
made an uneventful recovery and was discharged one month
later.

Final Diagnoses
Hypertrophic
cardiomyopathy
with subaortic
coronary atherosclerosis,
and mitral regurgitation.

stenosis,

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