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 TaskForce Criteria as affected;

3. Left ventricular ejection fraction of ≥30%

4. Functioning implantable cardioverter/defibrillator with remote interrogationcapability

5. One of the following: 1) a history of ventricular tachycardia or ventricularfibrillation (VF); 2) ≥500 ventricular premature contractions (VPCs) in 24 hours onthe most recent 24-hour Holter monitor recording; or 3) C-reactive protein >=1.5mg/mL

6. Agreement by subject, physician, and cardiologist to not change concomitant ACMmedications or to conduct catheter ablation during study participation unless neededfor 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:

10. Surgically sterilized (e.g., hysterectomy, bilateral oophorectomy, or tuballigation) for at least 6 months or postmenopausal (postmenopausal females musthave no menstrual bleeding for at least 1 year) or

11. If not postmenopausal, agree to use a double method of contraception, one ofwhich is a barrier method (e.g., intrauterine device plus condom, spermicidalgel plus condom) until 30 days after the IC14 treatment and have negative humanchorionic gonadotropin (β-hCG) test for pregnancy at Screening

12. 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 drugproduct 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 aswell as alanine transaminase (ALT)/aspartate transaminase (AST) elevations ≥ 3xupper limit normal (ULN); isolated AST elevation is not considered an exclusioncriterion from study participation

10. Current or planned use of continuous high-dose corticosteroids (short courses ofcorticosteroids are allowable)

11. Chronic immunosuppression with disease-modifying anti-rheumatic drugs (DMARDS)

12. Any clinically significant abnormality identified at the time of Screening that inthe judgment of the Investigator or any sub-Investigator would preclude safecompletion of the study

13. Patients who will be inaccessible due to geographic or social factors duringtreatment or follow-up