Cardiac Involvement in Wilson's Disease (WIL-HEART)

Fondation Ophtalmologique Adolphe de Rothschild

Status: Enrolling

Conditions: Wilson's Disease

Treatments: Procedure: Delivery of a long-term Holter 21 days Or placement of an implantable holter from the outset so syncope

Study type: Interventional

Funder types: NETWORK

Identifiers

NCT05493605

APS_2021_9

Details and patient eligibility

About

Heart damage by copper accumulation has been reported in Wilson's Disease. However, the disease epidemiology is still poorly understood. A number of studies on pediatric populations have not shown any significant cardiac involvement apart from early dysautonomia. This could suggest that the clinical manifestations related to the copper accumulation in the heart appears with the duration of the disease. Case-control studies on adult populations have highlighted various electrocardiographic (ECG) abnormalities more frequent in patients with Wilson's Disease than in healthy volunteers, but all these studies involved small number of patients (maximum 60). The hypothesis is that there is cardiac involvement in Wilson's Disease, requiring screening, follow-up and appropriate support.

Enrollment: 150 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Adult patient with Wilson's disease confirmed by a Leipzig score ≥ 4
• Express consent to participate in the study by the patient or legal guardian in the case of patients under guardianship or by the patient assisted by his curator in the case of patients under guardianship
• Member of or beneficiary of a Social Security scheme

Exclusion criteria

• Absolute or relative contraindication to MRI or contrast media
• Pregnant, parturient or breast-feeding women: a urine pregnancy test will be carried out in women of childbearing age
• Patient with hepatic decompensation (Child-Pugh score stage C)
• Patient in neuro-psychiatric decompensation

Trial design

Primary purpose: Other

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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150 participants in 1 patient group

Cases

Other group

Description:

The intervention consists in setting up a morphological and rhythmological cardiological follow-up patients with confirmed Wilson disease. It will require the wearing of a long Holter duration (21 days) and for some patients the installation of an implantable cardiac monitor.The long-time ECG holter is used to record heart rhythm for 21 days and detect possible arrhythmias (accelerations of the heart) or conduction disorders (slowdowns of the heart).

Treatment:

Procedure: Delivery of a long-term Holter 21 days Or placement of an implantable holter from the outset so syncope