MIBG scintigraphy in patients with inherited monogenic electric heart diseases

The clinical heterogeneity within families with the Long QT syndrome (LQTS),
Brugada syndrome (Brs) and cathecholaminergic polymorphic ventricular
tachycardia (CPVT) can be enormous, varying from asymptomatic family-members to
family members who experience multiple syncope*s and die ultimately at a young
age due to ventricular fibrillation. Risk-stratification in these patients is
difficult, as there are no risk-stratification tools available. Dysfunction of
the cardiac pre-synaptic activity appears to play an important role in the
symptomatic patients. Therefore, we hypothesize that cardiac MIBG scintigraphy
(a tool to asses the cardiac sympathetic pre-synaptic function) is an effective
risk stratification tool in these patients.

The main objective of the study is to establish whether cardiac MIBG
scintigraphy can be used as a risk-stratification tool in LQTS, Brs and CPVT.

Observational study in which a single cardiac MIBG scintigram is made. This
scintigram will be related to disease severity.

MIBG scintigraphy is a diagnostic test, using a radiopharmacon, which is safely
used in routine clinical practice for various conditions, including cardiac
disease (but not for the conditions described in this application). Because the
method used in this study is identical to the methods used in established
routine clinical tests, we anticipate that the risks of this study are equally
small as in these routine clinical tests. The extra burden for all patients is
a single injection of the radiopharmacon. The patients require a hospital
visit for scintigraphy.