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Updated: Mar 8, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
[Cardiomyopathy and ion channel diseases registry: the Szeged CardioGen Registry]
Péter Blazsó1, Kornél Kákonyi2, Tamás Forster2
1Gyermekgyógyászati Klinika és Gyermekegészségügyi Központ, Szegedi Tudományegyetem, Általános Orvostudományi Kar Szeged.
Insights
The Szeged registry established a patient database for cardiomyopathies and ion channel diseases. Analysis of hypertrophic cardiomyopathy patients showed consistent mortality and morbidity rates.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Cardiomyopathies and ion channel diseases represent significant cardiovascular challenges.
- A comprehensive registry is crucial for understanding disease progression and outcomes.
- The University of Szeged Cardiology Center follows numerous patients with these conditions.
Purpose of the Study:
- To establish a representative, disease-specific registry for cardiomyopathies and ion channel diseases.
- To collect and analyze patient data for improved understanding and management.
- To focus initial analysis on hypertrophic cardiomyopathy patient cohorts.
Main Methods:
- Patient recruitment from the University of Szeged Cardiology Center.
- Data collection on primary cardiomyopathies including hypertrophic, dilated, and restrictive types.
- Inclusion of ion channel diseases such as long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia.
Main Results:
- The registry includes diverse cardiomyopathy and ion channel disease patient groups.
- Hypertrophic cardiomyopathy patients (388) form the largest cohort.
- Dilated cardiomyopathy (310) and long QT syndrome (111) are also well-represented.
- Initial analysis of hypertrophic cardiomyopathy data reveals comparable mortality, morbidity, and clinical parameters.
Conclusions:
- The Szeged registry provides a valuable resource for studying cardiomyopathies and ion channel diseases.
- Hypertrophic cardiomyopathy patient data suggests consistent disease-related outcomes.
- Further analysis of registry data will enhance clinical understanding and patient care.
Abstract:
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia). Patients with hypertrophic cardiomyopathy (388 patients) make up the largest group of patients in the registry. Patients with dilated cardiomyopathy (310 patients) and patients with the long QT syndrome (111 patients) form two other sizable groups. Analyzed data of the group of patients with hypertrophic cardiomyopathy indicate similar figures with regard to disease related mortality and morbidity and clinical parameters. Orv. Hetil., 2017, 158(3), 101-105.
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