Sympathetic innervation of the left ventricle is impaired in arrhythmogenic right ventricular disease

Abstract

Arrhythmogenic right ventricular disease (ARVD) is a disease of unknown origin that primarily affects the right ventricle and is characterized by ventricular tachyarrhythmias which may lead to syncope and even, though rarely, sudden cardiac death. In 25 patients with ARVD, sympathetic innervation of the left ventricle was assessed by iodine-123 metaiodobenzylguanidine single photon emission tomography (123I-MIBG SPET). In addition, thallium-201 SPET was performed. The diagnosis of ARVD was made by an electrophysiological study and right and left heart catheterization including right ventricular endomyocardial biopsy. Ischaemic heart disease was excluded by coronary angiography. A group of seven patients without any evidence of heart disease served as a control group. Twenty-two of the 25 patients showed reduced uptake of 123I-MIBG. The abnormal areas were located predominantly in posterior and posteroseptal segments of the heart. No focus of increased 123I-MIBG activity could be demonstrated. No patient had signs of left ventricular involvement on left ventricular angiography. In contrast to the results of the 123I-MIBG SPET, those of 201T1 SPET were normal in 16 patients. The remaining nine patients showed areas of slight hypoperfusion not correlated with the reduced 123I-MIBG uptake. 123I-MIBG scintigraphy allows detection of left ventricular adrenergic dysinnervation in ARVD patients without morphological or functional abnormalities of the left ventricle.

Arrhythmogenic Right Ventricular Dysplasia: MRI Findings

“…The authors elaborated on 4 theories on the etiology of ARVD, potentially explaining the development and occurrence of the disease; the dysontogenic, degenerative, inflammatory, and apoptotic theory. Involvement of the left ventricle in ARVD had already been evidenced by Lerch et al [38] who showed impaired sympathetic innervation of the left ventricle in patients with ARVD. Accordingly, the disease should be regarded as a serious congenital heart disease.…”

Cardiac Radionuclide Imaging to Assess Patients With Heart Failure

“…) right ventricular dysplasia. 171 There is no established method for scoring SPECT 123 I-mIBG images, but analysis can be performed similar to the conventional 17-segment method used for MPI with generation of a summed score. 155 Nevertheless, an important consideration for 123 I-mIBG images is that when there is globally decreased uptake, that is, low HMR, homogeneous tracer uptake cannot be scored as normal as, unlike the custom for perfusion images, one cannot assume a "normal" region.…”

Imaging of myocardial autonomic innervation in patients with congestive heart failure: Methods and clinical implications

“…Myocardial ischemia and infarction produce regional efferent sympathetic denervation, which can be visualized by MIBG scintigraphy and may influence arrhythmias [134][135][136][137][138][139][140][141][142][143]. Abnormalities of sympathetic innervation, demonstrated by MIBG imaging, are frequent in patients with ventricular arrhythmias without apparent heart disease [144][145][146][147], in patients found with the congenital long-QT syndrome [148], and in those with arrhythmogenic right ventricular disease [149,150].…”