The longQT-syndrome is a genetically detemined malfunction of ion channels. The identified genes in longQT-patients encode cardiac ion channels or accessory proteins affecting the function of different ion channels. A certain gene defect can be associated with different symptoms regarding to the severity of the disease. Additionally, an increased sympathetic tone is a major trigger for potentially life threatening tachyarrhythmias depending on the genetical subtype.
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 100 patients with longQT syndrome and 40 healthy control subjects (male and female, >18 years) will be enrolled in the study Are the trial subjects under 18? no Number of subjects for this age range: F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) yes F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: - pregnant or breastfeeding women (confirmed by pregnancy test before scanning) - patients and volunteers younger thyn 18 years - patients who are kept in a mental institution upon official directive - patients who already took part in another resaerch project
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: Aim of this project is to investigate the autonomic nervous system using positron emission tomography (PET) in genotyped longQT syndrome patients and corresponding mouse model and compare the results with the genetic subtype, the phenotype and the role of the autonomic nervous system in the onset of tachyarrhythmias. Norepinephrine recycling will be quantified using PET and carbon-11 labelled hydroxyephedrine (11C-HED, sympathetic tone). The muscarinic receptor densitiy will be quantified using PET and carbon-11 labelled methylqunuclidinylbenzilat (11C-MQNB, parasympathetic tone). ;Secondary Objective: ;Primary end point(s): The results of the study are continuously analyzed regarding significant differences. As soon as a significant or non-significant and reasonable result is observed there no more patients will be enrolled. There is no specific end point for each patient alone, because there will be no more treatment or follow up after the end of the scan. | — |
Countries
Germany