This document describes a genetic arrhythmia center (GAC) that takes a multidisciplinary approach to caring for patients and families with genetic predispositions for sudden cardiac death. The GAC provides comprehensive care and screening for familial cardiac conditions. It aims to properly diagnose genetic causes of sudden cardiac arrest and provide targeted treatment and family screening. Creating the GAC required internal resources and developing relationships with external experts. It has evaluated over 500 patients/families, diagnosed several rare genetic conditions, and helped identify treatment and risk stratification for affected family members.

1. GENETIC ARRHYTHMIA CENTER
Jay Sengupta, MD Cardiac Electrophysiology
Raed Abdelhadi, MD FACC Cardiac Electrophysiology
Mosi Bennett, MD Advanced Heart Failure
William Katsiyiannis, MD FACC Cardiac Electrophysiology
Minneapolis Heart Institute Abbott Northwestern Hospital
Minneapolis, Minnesota
Creating a multidisciplinary approach to care for patients and families
with genetic predisposition for sudden cardiac death

2. Disclosures
• I participate in industry-sponsored trials and studies in
association with the Minneapolis Heart Institute
Foundation and have no personal conflicts.

3. Sudden Cardiac Arrest
• Cessation of cardiac activity resulting in
unresponsiveness, abnormal breathing, and lack of
circulation
• AHA 2015 incidence of out-of-hospital cardiac arrest
326,200
– Average survival rate is 10.6%
– Nearly 1 in 3 survive when witnessed by a bystander
– Approximately 6328 individuals < 18 years old in U.S.
experienced out-of-hospital cardiac arrest
American Heart Association Heart Disease and Stroke Statistics 2015 Update

4. Causes of Sudden Cardiac Arrest
• Cardiovascular
– Ischemic heart disease
– Non-ischemic Heart Disease
• Structural abnormalities
• Electrical abnormalities
• Non-cardiac causes

5. Why do we need GAC and SCA network?
- There is limited experience with potentially lethal familial/genetic cardiac conditions due
to rare nature and lack of evidence-based guidelines
- Care for family members with similar cardiac presentation is often scattered in the
community making it difficult to reach a specific diagnosis
- Familial/genetic conditions can have various expression among family members and
therefore can have various clinical presentations (asymptomatic, tachy-arrhythmia,
brady-arrhythmia, heart failure, death)
- Survivors of unexplained sudden cardiac death often get an ICD without adequate
workup
- Even when diagnosed, screening of family members is often scattered and non-uniform
- Families of victims of sudden cardiac death who had an abnormal autopsy need
counseling and screening

6. More than a genetic test…

7. Concept behind GAC
- Provide comprehensive care for patients with genetic/familial cardiac conditions
- Provide appropriate screening for families of patients with with genetic/familial
cardiac conditions
- Consolidate the care for families with genetic/familial cardiac conditions
- Identify potential genetic/familial cause for aborted sudden cardiac death and
provide targeted therapy for these patients as well as family screening
- Identify potential genetic/familial cause for unexplained sudden death in order to
screen family members
- Create a database and research platform for various genetic/familial cardiac
conditions

8. Requirements/challenges for creating GAC
1- Requires internal resources, interest, motivation and support:
- Financial constraints
- Time consuming (each patient and family member gets 60 minutes for
counseling and to bring understanding and closure)
- Multiple subspecialties with unique skills (cardiac imaging, electrophysiology,
pediatric electrophysiology, and advanced heart failure)
- Non-physician support team that understands the mission and uniqueness of this
center (scheduling, screening, testing, counseling, and coordination between family
members)

9. Requirements/challenges for creating GAC
2- Requires close relationship and cooperation with other experts
in the community outside the physician group (Sudden Cardiac
Arrest network)

10. Patient and data flow within the Sudden Cardiac Arrest
Network

11. Our Model
- Dedicated electrophysiology and advanced heart failure/cardiac transplant
specialist to manage and follow patients based on diagnosis
- Protocols for follow up and screening were created for various conditions (ARVC,
LQT, Brugada, LVNC, familial cardiomyopathy,…etc)
- Screening tests (imaging, tests, ..etc) are performed prior to clinic visit when
appropriate
- Advanced imaging with on-site experienced imaging cardiologist
- Targeted genetic screening and testing only after further discussion and
counseling
- Dedicated nurse, clinic coordinator/scheduler and research coordinator

12. Achievements
- We have seen more than 500 patients/family members
- Multiple family members were diagnosed and treated for potentially lethal
conditions (Last year: LQT2, LQT7, ARVC, LVNC, LMNAC,…)
- Two separate families were diagnosed with very rare genetic condition (LQT7:
variant of Andersen Tawil). One of them was the family of a young girl who dies
suddenly at the age of 19 with negative autopsy who was referred via the sudden
cardiac death network. Both were presented at the Heart Rhythm Society annual
meeting
- A very large Minnesota family was diagnosed with a novel mutation that can
manifest as arrhythmogenic right ventricular cardiomyopathy. Identifying this
mutation has led to risk stratification and treatment of several family members and
brought family members together

13. Conclusion
The GAC and SCA network deliver an integrated care
model that provides family members and patients with
appropriate testing and screening where traditional care
can be suboptimal in the discovery and management of
rare genetic diseases.