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.

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

Department of pediatric cardiology including outpatient department