Risk Stratification in Children and Adolescents With Primary Cardiomyopathy

Brief Title

Risk Stratification in Children and Adolescents With Primary Cardiomyopathy

Official Title

Risk Stratification in Children and Adolescents With Primary Cardiomyopathy

Brief Summary

      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.
    

Detailed Description

      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

Primary Outcome

major cardiovascular events

Condition

Primary Cardiomyopathy

Study Arms / Comparison Groups

 Index patients

Description:  Patients ≤18 years with primary cardiomyopathy

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Estimated Enrollment

200

Start Date

January 2013

Completion Date

December 2020

Primary Completion Date

December 2020

Eligibility Criteria

        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
      

Gender

All

Ages

N/A - 18 Years

Contacts

Felix Berger, MD, +49 30 450, [email protected]

Location Countries

Germany

Location Countries

Germany

NCT ID

NCT03572569

Organization ID

A30127

Secondary IDs

81Z3100331

Responsible Party

Sponsor

Study Sponsor

German Heart Institute

Collaborators

 Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK)

Study Sponsor

Felix Berger, MD, Principal Investigator, German Heart Institute

Verification Date

June 2018