Cardiovascular Journal of Africa: Vol 23 No 1 (February 2012) - page 73

CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 1, February 2012
AFRICA
e15
The patient stayed in the intensive care unit for 15 days and
was discharged home on the 20th postoperative day. At the time of
discharge, she did not have any renal or respiratory problems but
had occasional minor convulsions on the phenobarbital treatment.
Discussion
Myxomas in any site are uncommon in childhood. They consti-
tute only 13.5% of benign tumours in children younger than
15 years.
3
The most common symptom of cardiac myxoma is
congestive heart failure, followed by embolisation.
4
These symp-
toms, usually based on the location of the tumour, may vary
with the size and shape of the tumour, and also with the physical
activity and position of the patient. In a right atrial myxoma,
the clinical presentations may include ascites, hepatomegaly or
peripheral oedema due to right heart failure. Pulmonary emboli-
sation may also occur. Other clinical symptoms are vague such
as malaise, low-grade fever and weight loss.
4
Due to the acute
onset of cardiac insufficiency, we were unable to observe any of
these symptoms.
In our case, the massive right atrial myxoma caused function-
al tricuspid stenosis by prolapsing into the right ventricle through
the tricuspid valve. This was also determined by auscultation of
the mid-diastolic murmur in the tricuspid region. The myxoma
caused a reduction in right ventricular inflow, with consequent
reduction in the stroke volume of the right ventricle. We believe
that the tricuspid obstruction due to the right atrial tumour and
subsequent elevation of the right atrial pressure had resulted in
significant right-to-left shunt through the patent foramen ovale.
The decompression of the right atrium through a right-to-left
shunt may have been the cause of delayed onset of right heart
failure in our case. If there was no interatrial communication, the
clinical status of the case may have deteriorated earlier.
To date, the clinical manifestations of and surgical guidelines
for treating myxomas in children have not been described in
detail. The symptoms observed may be related to tumour loca-
tion and size. Dyspnoea is usually the first clinical presentation
and it may be a sign of heart failure. As in our case, echocardi-
ography can confirm the diagnosis and need for urgent surgical
treatment. Additional non-invasive diagnostic imaging methods
are computed tomography and magnetic resonance imaging
(MRI). More recently, the use of 64-slice spiral computed
tomography (CT) scanning has become more frequent. Cardiac
catheterisation in patients with a known myxoma is potentially
hazardous for embolisation.
4
Most reviews in adults with myxomas advise urgent extirpa-
tion because of the frequency of embolisation, especially with
myxomas of left atrial origin.
3
In infants, there are very few
reports on myxomas and their clinical treatment. The widely
accepted treatment of myxomas in any cavity of the heart is
operative resection. The surgical strategy is to excise the tumour
completely, and every effort should be made to preserve the
original valve. If a large portion of the atrial septum is removed,
a cloth patch or parietal pericardium must be used to close the
defect.
Günteroth however suggested a conflicting approach.
3
He
reported on five infants with intracardiac masses where, in one
case, the obstruction resulted in heart failure and required surgi-
cal extirpation. In the subsequent four infants, whose condi-
tions were diagnosed with echocardiography, the appearance
was suggestive of myxomas. All four cases remained mildly to
moderately obstructive and then showed spontaneous regression
during a period of months to years.
3
On the contrary, Hong Wu reported on 15 children between
the ages of five months and 14 years (mean age 6.5 years) with
cardiac myxomas. Three patients presented with congestive
heart failure and underwent emergency operations because of
severe congestive heart failure due to mitral valve obstruction.
5
D’alfonso presented extended follow up of patients with intracar-
diac myxomas and showed that surgical excision of such tumours
was curative with low mortality and good long-term outcome.
6
Padalino
7
and Elderkin
8
reported that rapidly growing myxomas
in small-sized cavities frequently caused congestive heart failure
in childhood, and surgical treatment was lifesaving.
Conclusion
Whatever the clinical presentation, we advocate immediate
surgical extirpation of the tumour in order to avoid any unpre-
dictable consequences in its clinical course. As myxoma cases in
infancy increase, the term ‘benign tumour’ may be re-evaluated.
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