, Volume 38, Issue 8, pp 1500-1506,
Open Access This content is freely available online to anyone, anywhere at any time.
Date: 07 May 2011

Imaging of programmed cell death in arrhythmogenic right ventricle cardiomyopathy/dysplasia

Abstract

Background

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a myocardial disease that predominantly affects the right ventricle (RV). Its hallmark feature is fibrofatty replacement of the RV myocardium. Apoptosis in ARVC/D has been proposed as an important process that mediates the slow, ongoing loss of heart muscle cells which is followed by ventricular dysfunction. We aimed to establish whether cardiac apoptosis can be assessed noninvasively in patients with ARVC/D.

Methods

Six patients fulfilling the ARVC/D criteria were studied. Regional myocardial apoptosis was assessed with 99mTc-annexin V scintigraphy.

Results

Overall, the RV wall showed a higher 99mTc-annexin V signal than the left ventricular wall (p = 0.049) and the interventricular septum (p = 0.026). However, significantly increased uptake of 99mTc-annexin V in the RV was present in only three of the six ARVC/D patients (p = 0.001, compared to 99mTc-annexin V uptake in the RV wall of the other three patients).

Conclusion

Our results are suggestive of a chamber-specific apoptotic process. Although the role of apoptosis in ARVC/D is unsolved, the ability to assess apoptosis noninvasively may aid in the diagnostic course. In addition, the ability to detect apoptosis in vivo with 99mTc-annexin V scintigraphy might allow individual monitoring of disease progression and response to diverse treatments aimed at counteracting ARVC/D progression.