CARDIOVASCULAR JOURNAL OF AFRICA • Vol 22, No 5, September/October 2011
248
AFRICA
complications are more common in patients with a greater than
5% annual increase in aortic dimensions, serial aortic root meas-
urements have prognostic importance in this patient population.
1
Two of the patients diagnosed with dilatation of the aortic root
had accompanying AR, which has no prognostic value in chil-
dren because of its late occurrence.
10
Mitral valve prolapse and MR occur before aortic valve
involvement and show early progression in children with MFS.
1
Consistent with this finding, MVP was detected in all our
patients. In patients with MFS, unlike other causes of MVP,
deformation of both mitral leaflets is more common and the need
for surgical intervention is usually earlier.
1-3
In our group, three patients had anterior leaflet prolapse while
eight had prolapse of both mitral leaflets. Nine patients had
marked MR and seven had TVP. Diagnosis of MVP and TVP was
based not only on systolic prolapse of the leaflets but also thick-
ness of the leaflets. Consequently, only classical MVP patients
were assessed,
6
however, marked mitral valve involvement and
frequency of TVP was significant. Therefore, in patients with
marfanoid phenotype, echocardiographic examination not only
focuses on aortic root measurements, but must also include
evaluation of the mitral and tricuspid valves from at least two
different echocardiographic planes.
In our patients, systolic and diastolic ventricular functions
were within normal limits. However, some studies have shown
that even with apparently normal systolic or diastolic function
using conventional echocardiographic examination, impairment
of these functions can be detected with tissue Doppler imaging.
These findings are explained as early manifestations of cardiac
involvement.
13
In addition, tissue Doppler imaging can be useful
in evaluation of the aortic wall thickness.
14
Therefore, supple-
mentary use of tissue Doppler imaging in patients with MFS is
important.
Cardiac symptoms in MFS are usually silent until adulthood.
1
Although our patient group had marked cardiac involvement
on echocardiographic evaluation, none had cardiac symptoms;
therefore no therapeutic interventions were needed. However,
early diagnosis of cardiac abnormalities and regular follow up
of patients is important. Therapy with beta-blockers is suggested
in these patients. With beta-blocker therapy, aortic stiffness is
reduced and aortic distensibility increased. In young patient
subgroups and patients with smaller aortic diameters, beta-
blockers reduce the risk of sudden death and improve survival
rates.
1-3
Conclusion
Even with no detected cardiac abnormality on first evaluation,
echocardiographic follow up at six- or 12-month intervals is
suggested, particularly in children with MFS.
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