IC14 (Atibuclimab) in Arrhythmogenic Cardiomyopathy
The goal of this clinical trial is to test IC14 (atibuclimab) in patients with arrhythmogenic cardiomyopathy (ACM) and who have an implantable cardoverter/defibrillator in place. ACM is also called arrhythmogenic right ventricular dysplasia (ARV) or arrhythmogenic right ventricular cardiomyopathy (ARVC). The main questions the study aims to answer are the effect of treatment on blood markers of inflammation, safety, and pharmacokinetics. There will also be measurements of myocardial imaging of C-C chemokine receptor type 2 (CCR2+) immune cells (optional), monitoring of cardiac arrhythmias using the patient's pre-existing intracardiac cardioverter/defibrillator (ICD) and a Holter monitor, electrocardiogram (ECG), echocardiogram (ECHO), and blood tests. Results will be compared to baseline; there is no inactive placebo treatment group.

Participants will be asked to undergo screening and baseline testing, then receive 4 intravenous infusions with blood measurements before and after the infusion (including 24, 48, and 72 hours and 7, 14, and 28 days). Participants will be offered specialized scanning of the heart muscle, and will be asked to provide recordings from their ICD, undergo Holter monitoring twice, and have electrocardiograms (ECG), echocardiograms (ECHO) and blood tests.
- EligibilityCriteria: Inclusion Criteria:  Patients are eligible for the study if all of the following criteria are met:  Age ≥ 18 years Diagnosis of arrhythmogenic cardiomyopathy and: 1) meeting the 2020 Modified Task Force Criteria as affected; and 2) demonstrating a genetic marker associated with arrhythmogenic cardiomyopathy Left ventricular ejection fraction of ≥30% Functioning implantable cardioverter/defibrillator with remote interrogation capability One of the following: a history of ventricular tachycardia or ventricular fibrillation (VF) or ≥500 ventricular premature contractions (VPCs) in 24 hours on the most recent 24-hour Holter monitor recording Agreement by subject, physician, and cardiologist to not change concomitant ACM medications or to conduct catheter ablation during study participation unless needed for management of life-threatening conditions C-reactive protein ≥1.5 mg/mL Capable and willing to provide informed consent Capable of completing study visits  Females participating in the study must meet one of the following criteria:  Surgically sterilized (e.g., hysterectomy, bilateral oophorectomy, or tubal ligation) for at least 6 months or postmenopausal (postmenopausal females must have no menstrual bleeding for at least 1 year) or If not postmenopausal, agree to use a double method of contraception, one of which is a barrier method (e.g., intrauterine device plus condom, spermicidal gel plus condom) until 30 days after the IC14 treatment and have negative human chorionic gonadotropin (β-hCG) test for pregnancy at Screening Males who have not had a vasectomy must use appropriate contraception methods (barrier or abstinence) until 30 days after IC14 treatment  Exclusion Criteria:  A patient fulfilling any of the following criteria is to be excluded from enrollment in the study:  Prior myocardial infarction History of unplanned cardioversion Receiving continuous infusion of antiarrhythmic medication at time of enrollment Previous major vascular intervention within 4 weeks NYHA heart failure class IV Major surgery within 6 weeks Patient has participated in any study using an investigational drug or device within 30 days Life expectancy of less than 1 year due to non-cardiac pathology History of allergic reaction to atibuclimab (IC14) or any monoclonal antibody drug product or other CD14-derived drug product or any component used in the study (including contrast media) Known severe renal (creatinine clearance <30 mL/min/m2) or hepatic insufficiency as well as alanine transaminase (ALT)/aspartate transaminase (AST) elevations ≥ 3x upper limit normal (ULN); isolated AST elevation is not considered an exclusion criterion from study participation Current or planned use of continuous high-dose corticosteroids (short courses of corticosteroids are allowable) Chronic immunosuppression with disease-modifying anti-rheumatic drugs (DMARDS) Any clinically significant abnormality identified at the time of Screening that in the judgment of the Investigator or any sub-Investigator would preclude safe completion of the study Patients who will be inaccessible due to geographic or social factors during treatment or follow-up - HealthyVolunteers: No - Gender: All - MinimumAge: 18 Years - StdAgeList: Adult, Older Adult