IC14 (Atibuclimab) in Arrhythmogenic Cardiomyopathy

Inclusion Criteria: Patients are eligible for the study if all of the following criteria are met: 1. Age ≥ 18 years 2. Diagnosis of arrhythmogenic cardiomyopathy and: 1) meeting the 2020 Modified Task Force Criteria as affected; 3. Left ventricular ejection fraction of ≥30% 4. Functioning implantable cardioverter/defibrillator with remote interrogation capability 5. One of the following: 1) a history of ventricular tachycardia or ventricular fibrillation (VF); 2) ≥500 ventricular premature contractions (VPCs) in 24 hours on the most recent 24-hour Holter monitor recording; or 3) C-reactive protein \>=1.5 mg/mL 6. 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 7. Capable and willing to provide informed consent 8. Capable of completing study visits 9. Females participating in the study must meet one of the following criteria: 1. 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 2. 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 10. 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: 1. Prior myocardial infarction 2. Receiving continuous infusion of antiarrhythmic medication at time of enrollment 3. Previous major vascular intervention within 4 weeks 4. NYHA heart failure class IV, except palpitations 5. Major surgery within 6 weeks 6. Patient has participated in any study using an investigational drug or device within 30 days 7. Life expectancy of less than 1 year due to non-cardiac pathology 8. 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) 9. 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 10. Current or planned use of continuous high-dose corticosteroids (short courses of corticosteroids are allowable) 11. Chronic immunosuppression with disease-modifying anti-rheumatic drugs (DMARDS) 12. 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 13. Patients who will be inaccessible due to geographic or social factors during treatment or follow-up