Risk Stratification in Children and Adolescents With Primary Cardiomyopathy

Status

UNKNOWN

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT03572569

Acronym

RIKADA

Enrollment

200

Registered

2018-06-28

Start date

2013-01-31

Completion date

2020-12-31

Last updated

2018-06-28

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

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

Keywords

Pediatrics, Cardiomyopathy, Heart failure, Risk stratification, Family screening, Genetics

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.

Interventions

None listed

Study design

Observational model

FAMILY_BASED

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

No minimum to 18 Years

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

Design outcomes

Primary

Measure	Time frame	Description
major cardiovascular events	from date of enrollment until the date of death, mechanical circulatory support or heart transplantation, assessed up to 8 years	death, need for mechanical circulatory support or heart transplantation

Countries

Germany

Contacts

Primary ContactSabine Klaassen, MD

klaassen@mdc-berlin.de+49 30 450

Backup ContactNadya Al-Wakeel-Marquard, MD

alwakeel@dhzb.de+49 30 4593

Outcome results