Skip to content
How It Works
Explore
Resources Ask a Question
Who It's For
Patients
Decode results, prep for appointments, weigh options.
Caregivers & advocates
Carry the research load for someone you love.
Providers & clinicians
Resources for patients in your practice.
Log In Ask a Question
PubMed This is a summary of 95 peer-reviewed journal articles Updated
Cardiology

Your Guide to Inherited Arrhythmogenic Cardiomyopathy (ACM/ARVC)

At a Glance

Inherited Arrhythmogenic Cardiomyopathy (ACM), also known as ARVC, is a genetic heart condition causing irregular heartbeats. Essential management includes genetic testing, strict exercise restriction, medications, and sometimes an ICD to protect the heart and prevent dangerous arrhythmias.

Receiving a diagnosis of Inherited Arrhythmogenic Cardiomyopathy (ACM) or Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a life-altering moment. It is completely normal to feel overwhelmed, scared, and uncertain about your future.

This guide was created to help you navigate this complex condition. It is designed to take you from the initial shock of a diagnosis to a place of empowerment. By understanding the biology of your heart, the diagnostic puzzle, and the standard of care, you can become an active participant in your treatment and a strong advocate for your health.

Here is an overview of what you will find in this guide:

01

Understanding Inherited Arrhythmogenic Cardiomyopathy (ACM/ARVC)

Understand inherited Arrhythmogenic Cardiomyopathy (ACM/ARVC). Learn about fibrofatty replacement, exercise restrictions, and how to manage this condition.

02

Signs, Symptoms, and Warning Signs of ACM

Learn the hidden signs of Arrhythmogenic Cardiomyopathy (ACM). Understand early warning symptoms, exercise triggers, and the myocarditis-mimicking hot phase.

03

The Diagnostic Process: Putting the Puzzle Together

Learn how Arrhythmogenic Cardiomyopathy (ACM) is diagnosed. Understand the 2020 Padua Criteria, Cardiac MRI results, and the tests needed for a complete workup.

04

The Genetics of ACM: Understanding Your Mutation

Learn how gene mutations like PKP2 and DSP affect Arrhythmogenic Cardiomyopathy (ACM). Understand the types of ACM and what your genetic test results mean.

05

Treatment Options & The Standard of Care

Explore the standard treatment options for Arrhythmogenic Cardiomyopathy (ACM). Learn about exercise restrictions, beta-blockers, ICDs, and ablation procedures.

06

Living with ACM: Family, Pregnancy, and Emotional Support

Learn how to live with arrhythmogenic cardiomyopathy (ACM). Understand family cascade screening, pregnancy planning, building a care team, and mental health.

You do not have to read everything at once. Take your time, lean on your support system, and use these pages to prepare for your next cardiology appointment.

Common questions in this guide

What is the difference between ACM and ARVC?
ARVC (Arrhythmogenic Right Ventricular Cardiomyopathy) is an older term for the condition. It is now frequently called ACM (Arrhythmogenic Cardiomyopathy) because doctors have learned the disease can affect both sides of the heart, not just the right ventricle.
What are the warning signs of Arrhythmogenic Cardiomyopathy?
A primary warning sign of ACM is an irregular heartbeat, also known as an arrhythmia. However, the disease can remain hidden for years before symptoms suddenly appear during a period known as a 'hot phase'.
How is ACM/ARVC diagnosed?
Doctors diagnose ACM by combining results from multiple tests using a scoring system called the Padua Criteria. The diagnostic process typically involves cardiac MRIs, electrical tests like an ECG or Holter monitor, and genetic testing.
How is Inherited Arrhythmogenic Cardiomyopathy treated?
Treatment focuses on managing arrhythmias and preventing heart damage. The standard of care usually involves strict exercise restriction, specific heart medications, and often the surgical placement of an implantable cardioverter-defibrillator (ICD).
Who in my family needs to be tested if I am diagnosed with ACM?
Because ACM is an inherited condition, your close relatives should undergo cascade family screening. Your care team can help map out which family members need to be tested based on the specific genetic mutation discovered in your DNA.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my overall health and diagnosis, which specific pages of this guide should I focus on first?
  2. 2.Can we schedule a dedicated appointment to discuss my long-term care plan and family screening?
  3. 3.Are there any immediate lifestyle changes I need to make before my next round of testing?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

This guide is for informational purposes only and does not replace professional medical advice. Always consult your cardiologist regarding your ACM or ARVC diagnosis, symptoms, and specific care plan.

Get notified when new evidence is published on Inherited arrhythmogenic cardiomyopathy.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.