Risk Stratification in Children and Adolescents With Primary Cardiomyopathy

German Heart Institute200 enrolled

Overview

RIKADA is a prospective study performing systematic family screening including clinical and genetic testing in pediatric patients with primary cardiomyopathy and their first-degree relatives with the aim to facilitate risk stratification.

RIKADA is a long-term prospective study performing in-depth phenotype and genotype characterization in children and adolescents with primary cardiomyopathy and their first-degree family members. Family screening contains complete cardiac work-up with medical history, physical examination, 12-lead-/Holter-electrocardiogram, cardiopulmonary exercise testing, echocardiography, cardiovascular magnetic resonance (CMR) and laboratory including genetic testing. The aim is to facilitate early identification of at-risk individuals and contribute to patient-specific follow-up and therapy regimes preventing progressive heart failure and arrhythmia in pediatric CMP.

Study Type

OBSERVATIONAL

Enrollment

200

Conditions

Primary Cardiomyopathy Dilated Cardiomyopathy Hypertrophic Cardiomyopathy Left Ventricular Noncompaction Restrictive Cardiomyopathy Arrhythmogenic Right Ventricular Cardiomyopathy

Eligibility

Sex: ALLMax age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: 1. Index patients: * Age ≤18 years * written informed consent of parents/legal guardians * diagnosis of primary cardiomypathy: * DCM: left ventricular (LV) systolic dysfunction and dilatation greater than two standard deviations (SD) above the mean of a normal population * HCM: LV hypertrophy and septal wall thickness above two SD * RCM: diastolic dysfunction and concordant atrial enlargement * LVNC: separation of the myocardium into a compacted (C) and a non- compacted (NC) layer with an NC/C ratio \>2 in echocardiography and/or \>2.3 in CMR * ARVC: according to the revised Task Force Criteria 2. First-degree family members (parents and siblings): * Age ≥3 years * written informed consent of parents/legal guardians and siblings ≥18 years Exclusion Criteria: * unwillingness to give consent * myocardial inflammation / myocarditis * systemic disease with cardiac involvement (secondary cardiomyopathy) * structural congenital heart disease

Locations (2)

Charité - Universitätsmedizin Berlin

Berlin, Germany

RECRUITING

German Heart Institute

Berlin, Germany

RECRUITING

Outcomes

Primary Outcomes

major cardiovascular events

death, need for mechanical circulatory support or heart transplantation

Time frame: from date of enrollment until the date of death, mechanical circulatory support or heart transplantation, assessed up to 8 years

Central Contacts

Sabine Klaassen, MD

CONTACT

+49 30 450 klaassen@mdc-berlin.de

Nadya Al-Wakeel-Marquard, MD

CONTACT

+49 30 4593 alwakeel@dhzb.de

Data from ClinicalTrials.gov

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