Posted on July 8, 2009
Combined diagnostics useful for arrhythmogenic right ventricular cardiomyopathy/dysplasia
A combination of several diagnostic tests is optimal for
the evaluation of patients with suspected arrhythmogenic right ventricular
cardiomyopathy/dysplasia, according to a study in this month’s Heart
Rhythm Journal.
Researchers enrolled 108 newly diagnosed patients with
suspected arrhythmogenic right ventricular cardiomyopathy/dysplasia. Patients
underwent standardized testing that included 12-lead echocardiograms,
single-averaged ECGs, 24-hour Holter monitoring and electrophysiologic study
with programmed stimulation that resulted in ventricular arrhythmias. The right
ventricles were imaged with echocardiography, MRI and right ventricular
angiography. The results were interpreted first by the enrolling center and
then adjudicated by blind analysis in six core laboratories.
According to the study results, of the 108 probands
measured, 86 were classified as affected by arrhythmogenic right ventricular
cardiomyopathy/dysplasia in the original assessment. The researchers reported
that after blind interpretation of the diagnostic testing by core laboratories,
78% (67 of 86) who had the classification of “affected” at enrollment
maintained it upon final analysis of the diagnostic results, with 17 as
“borderline” and two as “unaffected.” Of the 19 patients
classified as borderline at enrollment, nine remained borderline at final
diagnosis, five were affected and five were unaffected. The final clinical
diagnosis revealed that 73 probands were classified as affected, 28 probands
were classified as borderline and seven probands were unaffected. The
researchers reported that the individual tests agreed with the final diagnosis
in 50% to 70% of the patients classified as affected in the final diagnosis.
“Echocardiography, right ventricular angiography,
single-averaged ECGs and 24-hour Holter monitoring provide
optimal clinical evaluation of patients suspected of having arrhythmogenic
right ventricular cardiomyopathy/dysplasia,” the researchers wrote in
their conclusion. “In the early stages of arrhythmogenic right ventricular
cardiomyopathy/dysplasia, overall right ventricular function may be normal,
with local or regional wall-motion abnormalities that are difficult to
quantify.”
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