An 80 year old patient named Mr. QRS, has suffered his third stroke in several years.

His first
two strokes resulted in hemiparesis. He has been in a nursing home since his first stroke and is
totally dependent on others. However, this stroke has resulted in a coma. He is unresponsive
even to painful stimuli. His wife of 60 years requests he is not to be resuscitated and that he is
not fed or hydrated artificially, either through tube feedings or intravenously. He does not have a
living will or a durable power of attorney but the wife states shortly before his first stroke, he
verbalized his wishes not to be kept alive artificially.
Several nurses feel he should receive tube feedings and IV hydration and say they cannot "watch
someone starve to death". Others believe this patient is comatose and therefore would not be
suffering any discomfort associated with dehydration or starvation.

A 22-year-old Woman with Ischemic Stroke and Left Ventricular Dysfunction

Case Presentation - October 2011
Shikhar Agarwal MD MPH CPH, Cardiology Fellow, Cleveland Clinic
A 22-year-old female was transferred to Cleveland Clinic from an outside hospital for evaluation of acute ischemic
stroke. She has presented to an outside hospital two days prior with acute onset numbness and tingling of bilateral
upper extremities and mild expressive aphasia.

Past Medical History

Her past medical history was significant for migraines with aura. Six months prior to her current presentation, the
patient had a transient episode of forgetfulness and confusion for four hours and one month ago the patient had
reported an episode of numbness and tingling of her left lower extremity lasting three hours. She did not seek medical
attention for these episodes.

Initial physical examination at the outside hospital

On initial presentation, the patient had a blood pressure of 128/84mmHg, heart rate of 75
beats per minute and respiratory rate of 16 breaths per minute saturating at 98%, while
breathing ambient air. Her lungs were clear to auscultation. Cardiac auscultation did not reveal
any murmurs or gallops. Abdomen was non-tender, non-distended without any
hepatosplenomegaly. Skin examination was significant for three circular macules ranging 1-3
mm on the right hand. Neurological examination was significant for focal weakness in the
distal left upper extremity.

Hospital course prior to presentation to Cleveland Clinic

Computed tomography of the brain was performed which failed to reveal any evidence of
cerebral hemorrhage. Magnetic resonance imaging of the brain demonstrated left sided acute
infarcts in the middle cerebral artery and posterior inferior cerebellar artery territories.
Ultrasonography of bilateral carotid arteries was reported to be normal. An echocardiogram
performed at the outside hospital, demonstrated presence of a left atrial mass with a severely
reduced left ventricular systolic function (ejection fraction 20%). One day later, the patient
became lethargic and had multiple episodes of emesis. She became febrile (core body
temperature 38.2 C), hypotensive with systolic blood pressure 60mmHg and developed
significant degree of leucocytosis (WBC: 25200). She was electively intubated for airway
protection. She was given empiric broad-spectrum antibiotics consisting of vancomycin,
gentamicin and ceftriaxone. She developed a generalized erythematous rash to vancomycin
and subsequently it was switched to daptomycin. In addition to antibiotics, she was also
started on phenylephrine and norepinephrine infusions to maintain mean arterial blood
pressure of 65 mm Hg. Laboratory work up was significant for positive cardiac biomarkers [CK-

MB: 51 ng/mL (normal <8.8 ng/mL) and Troponin I: 4.8 ng/mL (normal <0.4 ng/mL)]. A repeat
echocardiogram demonstrated reduction in the left ventricular ejection fraction to 10%. At this
point, a decision was taken to transfer the patient to our hospital.

Hospital course at Cleveland Clinic

Figure 1: Axial section of the computed tomography of the brain demonstrating areas of subacute
infarction in the left fronto-parietal area.

She was admitted to the coronary intensive care unit of our hospital. The patient was intubated
but followed simple commands. Her vital signs were significant for fever (Temperature: 38.2 C),
hypotension (BP: 88/61) and regular tachycardia (heart rate: 110). Neurological examination
was significant for global weakness, worst in the left upper extremity. In addition, there was
impairment of leftward gaze in her left eye.
Her initial laboratory examination was significant for anemia (hemoglobin: 10.6), leucocytosis
(WBC: 21870) and acute kidney injury (serum creatinine: 1.5 mg/dL). Evidence of myocardial
injury was confirmed with elevated cardiac biomarkers (CK: 1352 ng/mL, CK-MB: 10.9 ng/mL
and Troponin T: 0.79 ng/mL). Repeat computed tomography of the brain demonstrated
evidence of subacute infarction involving multiple areas (Figure 1). Transthoracic
echocardiography confirmed the presence of severely reduced ejection fraction and a large left
atrial mass attached to the interatrial septum (Movie 1-2). There were regional wall motion
abnormalities noted in multiple territories, most prominent in the apical region (Movie 1-2).
Transesophageal echocardiography revealed a large mass in the left atrium 3.5 cm X 2.8 cm X
1.7 cm (Movie 3-4). The mass was noted to have a broad base of attachment to the interatrial
septum and multiple frond like projections into the left atrial cavity (Movie 3-4).
Due to the devastating embolic complications of this mass, urgent surgical treatment was
contemplated. It was evident that the patient had suffered cerebral embolic stroke and
possibly acute myocardial infarction as a result of embolic occlusion of coronary arteries. The
patient underwent an urgent coronary angiogram, which revealed completely normal coronary
arteries. Presence of normal coronary arteries in the setting of multi-territorial wall motion
abnormalities, predominantly involving the apex, suggested the diagnosis of Takotsubo
cardiomyopathy.
Within 24 hours of her presentation to our hospital, the patient was moved to the operating
room for resection of the atrial mass. Intraoperatively, the right atrium was opened and a large
amount of septum was removed, in order to excise the atrial mass without disturbing it. The
septotomy was closed using a piece of autologous pericardium. Subsequently, the patient was
weaned off the cardiopulmonary bypass after closing the right atrium.
Gross and histological examination of the mass was consistent with left atrial myxoma. Histological examination
revealed a myxoid tumor with lepidic cells arranged in cords and ring structures. The stroma showed mononuclear

infiltrates and hemosiderin-laden macrophages without neutrophils (Figures 2-3). The tumor was completely excised
with a portion of normal myocardium at the base.

Figure 2: H & E stained section of the excised mass under low power magnification. The photomicrograph
demonstrates a bluish stroma containing areas of extensive hemorrhage along with isolated and clustered
tumor cells.

Figure 3: H&E stained section of the excised mass under high power magnification. The photomicrograph
demonstrates presence of tumor cells in isolation and surrounding blood vessels. In addition, several
hemosiderin-laden macrophages are visible scattered in the myxoid stroma.

Post-operative course
Post-operatively, the patient had a relatively uncomplicated clinical course. She was extubated
on post-operative day two, following which it was realized that she had lost complete vision in
her left eye. Indirect ophthalmoscopy demonstrated presence of central retinal artery occlusion
of subacute duration, which was attributed to be embolic sequela of the atrial myxoma. Five
days after surgery, the left ventricular function had improved to 45%. She was discharged on
the eighth post-operative day. Prior to her discharge, she had regained complete motor
strength in all four extremities. However, the visual loss in the left eye was persistent.

Discussion
Cardiac myxoma is the most commonly encountered primary tumor affecting the heart
accounting for at least half of all primary cardiac neoplasms 1. Up to half of the cardiac
myxomas may produce systemic emboli 2. Emboli from the cardiac myxoma may lead to
cerebral ischemia or infarction, myocardial infarction or peripheral embolism 2,3,4. Of these
complications, cerebral ischemia is the most commonly encountered embolic complication
accounting for 0.5% of all strokes 5. Left atrial myxomas have been occasionally associated
with acute embolic occlusion of coronary arteries leading to acute myocardial infarction 3.
Our patient is unique due to her systemic presentation involving multiple organ systems. In
order to provide the best possible care to this patient, a multi-disciplinary approach was
warranted from the beginning. The initial differential diagnosis consisted of septic thrombus,
atrial myxoma or atrial sarcoma. Regardless of the exact histological diagnosis, it was certain
that the mass had potential to cause catastrophic embolic complications in this young patient.

It was interesting to note the occurrence of Takotsubo cardiomyopathy in our patient. The
syndrome is characterized by transient left ventricular dysfunction, perhaps attributable to the
catecholamine surge in stressful situations 6. An acute drop in her ejection fraction was
concerning for embolic occlusion of coronary arteries. However, multi-territorial wall motion
abnormalities along with presence of typical apical ballooning were very suggestive of this
syndrome, which were subsequently confirmed with coronary angiography.
The origin, progression and risk factors of atrial myxomas remain unclear. Although considered
benign, there has been description of recurrence of myxoma after surgical excision 7. However,
it is interesting to note that there are eight cases described in the literature reporting
association between cardiac myxoma and migraine with aura 7. There have been hypothetical
elucidations of mechanisms by which cardiac myxomas may trigger cortical spreading
depression leading to migraine with aura. However, the exact mechanism remains unknown.

References
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2.
3.

4.
5.
6.

7.

Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001: 85: 218 22.
Rodreigues D, Matthews N, Sccones D, Azis F, Nath F. Recurrent cerebral metastasis from a
cardiac myxoma: case report and review of literature. Br J Neurosurg 2006. 20: 318 20.
Konagai N, Cho M, Shigematsu H. Left atrial myxoma associated with acute myocardial
infarction and multiple cerebral infarctions: Report of a case. Surg Today. 2010 Dec;40(12):
1159 63.
Uner A, Dogan M, Sal E, Peker E. Stroke and recurrent peripheral embolism in left atrial
myxoma. Acta Cardiol. 2010 Feb;65(1): 101 3.
Ekinci EI, Donnan GA. Neurological manifestations of cardiac myxoma: a review of the
literature and report of cases. Intern Med J. 2004; 34: 243 9.
Koulouris S, Pastromas S, Sakellariou D, Kratimenos T, Piperopoulos P, Manolis AS.
Takotsubo cardiomyopathy: the "broken heart" syndrome. Hellenic J Cardiol. 2010 SepOct;51(5): 451 7.
Amano J, Kono T, Wada Y, Zhang T, Koide N, Fujimori M, Ito K. Cardiac myxoma: its origin
and tumor characteristics. Ann Thorac Cardiovasc Surg. 2003 Aug;9(4): 215 21. 8. de
Ceuster L, van Diepen T, Koehler PJ. Migraine with aura triggered by cardiac myxoma: case
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8. Staff commentary
9. Dr. James Thomas, Tomsich Family Department of Cardiovascular Medicine, Section of Imaging
10. This case illustrates the importance of a multidisciplinary approach to cardiac masses and the
utility of three-dimensional transesophageal echocardiography in guiding therapy. Several
aspects of this case deserve comment. First is the prolonged prodrome of migraine and at least
two transient ischemic events undoubtedly related to emboli from the growing mass. She
subsequently presented with a devastating syndrome of aphasia and a mixture of cardiogenic
and (apparently) septic shock. Given the clear-cut cerebral emboli, it was quite appropriate to
be concerned that coronary emboli were responsible for her profound LV dysfunction.
Fortunately, her coronaries were normal, indicating Takotsubo syndrome as the cause of her
depressed EF, which subsequently improved to 45% just four days post-operatively. The timing
of surgery in patients with large cerebral emboli is always challenging, due to the fear of
hemorrhagic conversion with the large heparin doses needed intraoperatively. In this case, the
clear danger of further emboli mandated urgent surgery, which was conducted successfully
without any further neurologic complications. By the time of discharge, her speech was greatly
improved and her right arm weakness was resolving.
11. The 3D TEE shown here is dramatic, showing a large, friable mass with multiple mobile
protuberant fronds at risk for further emboli. Even more useful to those caring for her was the
ability to explore this 3D dataset interactively, to determine the precise location of attachment

to the interatrial septum and absence of any satellite lesions. Armed with this information, the
surgeon was able to completely excise the myxoma blindly by approaching it from the right
atrium with an excellent clinical outcome.