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Case
Report -
A Construction Worker with Fatigue
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Federico Viganego, MD
A 49-year-old construction worker with no significant past
medical history presented to his primary care physician
in September, 2003 with a five month history of fatigue
and dyspnea on exertion that worsened with high humidity
and after a day’s work. The patient’s job consisted of physical
labor, i.e., building log homes; however, he never had similar
symptoms in the past. The patient also described intermittent
episodes of mild left arm pain with an exertional quality,
lasting no longer than five minutes since the previous spring.
Also, occasional episodes of Raynaud-like features such as
“white hand” were described, especially after continuous
chain saw use. He denied having dizziness, light-headedness,
nausea, chest pain or palpitations. He had no previous history
of malignancy, cardiopulmonary, hematologic, neuro-muscular,
psychiatric or endocrine disorders. His past medical history
was significant for remote alcohol abuse and an episode
of spontaneous pneumothorax 13 years earlier. Past surgical
history was notable for testicular tubular ligation and
vasectomy. His medications included daily aspirin and multivitamin
complex. He denied current alcohol consumption, smoking
or illicit drug use. Family history was significant for
coronary artery disease and colon cancer.
On physical examination, patient appeared in no distress.
Vital signs were normal, as were lungs, heart, and abdomen
examinations. A significant delay in capillary refill was
noted with radial occlusion in both hands. The remaining
of the physical exam was unremarkable.
Laboratory studies and chest radiograph were normal. Electrocardiogram
showed sinus bradycardia at 48 beats per minutes with a
borderline prolonged, notched P wave in the inferior leads,
however not fulfilling criteria for a typical “P mitrale”
pattern.
In view of his atypical chest pains, the patient was referred
to a cardiologist for an echo stress test, which showed
a normal left ventricle with an ejection fraction of >60%,
left atrium dilation (51.5 mm) with a normal mitral valve
and no observed mitral valve gradient. A left atrial mass,
consistent with a myxoma, was noted attached to the interatrial
septum (Figure 1).
Figure 1. Echocardiogram showing a
left atrial mass.
A CT of the thorax (Figures 2a and 2b) showed a large, 4
x 6 x 4 cm left atrial mass, which was adherent to the inter-atrial
septum and extending inferiorly to abut the anterior leaflet
of the mitral valve as well as the common wall of the aorta.
The mass filled a large percentage of the left atrial cavity,
resulting in a significant narrowing of the mitral valve
outflow into the left ventricle. Evaluation of the coronary
arteries showed evidence of multiple calcified plaques consistent
with coronary artery disease.
Figure 2A.
Figure 2B.
Left heart catheterization with coronary angiography showed
single vessel disease with a 70-75% obstruction of the mid
left anterior ascending artery.
The patient underwent thoracotomy with successful excision
of the tumor and a single coronary artery bypass grafting
with anastomosis of the left internal mammary to the left
anterior descending artery. Histology of the excised mass
was consistent with atrial myxoma.
Following surgery, the patient returned to his regular life.
Follow-up echocardiography at 6 months showed no evidence
of recurrence of the atrial mass.
DISCUSSION
Primary tumors of the heart are rare, with an incidence between
0.0017 and 0.19% in unselected autopsy series1. Most of them are benign, and approximately
half of them are myxomas. Myxomas occur more frequently between the third
and sixth decade. Though most cases are sporadic, familial recurrences of
myxomas have been described; these usually present in association with skin,
breast, testicular tumors, pituitary or adrenal cortex adenomas, as well as
cutaneous hyperpigmentation1. Family studies of inheritance suggest
a possible dominant pattern of this rare syndrome2.
Myxomas are neoplasms of endocardial origin, generally polypoid, more often
pedunculated, that project into the cardiac chamber. They are constituted
of multi-potential mesenchymal cells, which usually have a rather high rate
of growth. About 75% of myxomas originate in the left atrium, while right
atrium involvement is uncommon. Diagnosis can be incidental on imaging studies
or, more often, after the patient presented with signs or symptoms that prompted
a cardiac work-up. Before the introduction of imaging methods such as angiocardiography,
echocardiography, CT or MRI, the diagnosis of atrial myxoma was made only
at postmortem examination. The first diagnosis of a left atrial myxoma made
during life with angiocardiography was described in 19524, while the first successful surgical
removal was reported in 19541.
Atrial myxoma (AM) may present clinically according to its
anatomical location, size and mobility. In a recent study
that analyzed clinical presentation of left AM in 112 patients3,
the most common initial symptoms were related to mitral
valve outflow obstruction: congestive heart failure, dizziness,
syncope, chest pain, palpitations, dyspnea, cough, or pulmonary
edema were observed in 52% of patients. Embolic manifestations,
either cerebral or peripheral were recognized in 16% of
patients. Constitutional symptoms, such as fever, fatigue,
muscle weakness, asthenia, weight loss, arthralgia and Raynaud’s,
were seen in 15% of cases. Incidental diagnosis was made
in ten percent of patients by echocardiography or CT scan
that were performed for another indication.
Abnormal
cardiac auscultation findings in patients with AM were found
to be significantly associated with constitutional symptoms,
such as fever, fatigue or weight loss3. Cardiac auscultation findings include
systolic or diastolic murmurs. Diastolic murmur are similar
to those observed in mitral stenosis and are due to obstructed
filling of the left or right ventricle, while systolic murmurs
occur when the mass interferes with the closure of the atrioventricular
valve or narrows the outflow tract. A “tumor plop”, that
is the presence of a protodiastolic murmur after the second
heart sound is audible in about 30% of patients.
Electrocardiographic findings are
described in about two thirds of patients, and are mostly
the result of atrial overload (left or right atrial abnormality)
or ventricular hypertrophy. Less frequently, nonspecific
ST-segment abnormalities or supraventricular arrhythmias
have been observed. Chest radiograms may occasionally reveal
left atrial enlargement or signs of pulmonary hypertension
or congestion.
M-Mode and, subsequently two-dimensional
echocardiography, which allow direct visualization of the
atrial chambers, have largely facilitated the diagnosis
of intracardiac tumors, also given their wide availability
and relatively low costs. About 85% of patients with AM
were diagnosed by echocardiography in the French study3. In addition, transthoracic echo
(TTE) can be completed with a transesophageal view in order
to obtain more detailed morphologic features of the tumor.
Despite histologically benign AM
is potentially life threatening due to its location and
ensuing hemodynamic consequences. Surgery, with excision
of the tumor, is usually curative, although recurrences
of AM have been described1. The possibility of an atrial mass
should be considered by the clinician especially in previously
healthy patients who present with cardiac, constitutional,
or even atypical symptoms such as Raynaud’s that are not
accounted for by more frequent pathologies. Family screening
with TTE might be appropriate if a genetic link is suspected.
Cardiac imaging may allow detection of the tumor and potentially
definitive cure through surgical excision. After surgery,
close follow-up with TTE is recommended to allow early recognition
of possible recurrences.
REFERENCES
1. Reynen
K. Cardiac myxomas. New England Journal of Medicine
1995; 333:1610-1617.
2. Carney
JH, Hrushka LS, Beauchamp GD, Gordon H. Dominant inheritance of the complex
of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin
Proc 1986; 61:165-172.
3. Pinede
L, Duhaut P, Loire R. Clinical presentation of left atrial
cardiac myxoma. A series of 112 consecutive cases. Medicine
(Baltimore) 2001; 80:159-172.
4. Goldberg
HP, Glenn F, Dotter CT, Steinberg I. Myxoma of the left atrium: diagnosis
made during life with operative and postmortem findings. Circulation
1952; 6:762-767.
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