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Combined diagnostic tests useful for characterizing, diagnosing suspected arrhythmogenic right ventricular cardiomyopathy/dysplasia
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A combination of several diagnostic tests is optimal for the evaluation of patients with suspected arrhythmogenic right ventricular cardiomyopathy/dysplasia.
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 electrocardiograms 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.” – by Eric Raible
Marcus F. Heart Rhythm. 2009;6:984-992.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is both a risk for sudden cardiac death and a cause of death in individuals without a known diagnosis. Accurate diagnosis is essential not only to allow for correct treatment, but also to avoid misdiagnosis and the adverse consequences of inappropriate therapy. The diagnosis is difficult, as highlighted in this paper by Marcus and colleagues. Importantly, MRI has become a benchmark imaging modality for ARVC/D screening, but the authors show that a false-positive diagnosis is more frequent in referring hospitals than at their core center, indicating the potential for over-diagnosis in the community. A combination of diagnostic tests may enhance the accuracy of diagnosis and decrease the risk of misdiagnosis. The optimal combination needs to be prospectively examined.
– Andrew E. Epstein, MD
Cardiology Today Editorial Board member