A Look Inside the Inherited Cardiovascular Disease Clinic

The Heart Arrhythmia Center at The Heart Hospital Baylor Plano’s Center for Advanced Cardiovascular Care is a specialized setting where patients with heart rhythm disorders can be evaluated. The center supports and supplements the services provided in traditional cardiology office settings by offering additional resources for evaluation and care. 

The clinic is staffed by a team of electrophysiologists dedicated to the treatment of cardiac arrhythmias. Such treatments may include medication therapy, non-invasive testing, or catheter-based/surgical procedures. Services offered include comprehensive, on-site imaging services (if needed), a multidisciplinary approach to care (including surgical consultation), and coordination of care before complex ablation procedures. 

A unique aspect of the Heart Arrhythmia Center is the Inherited Cardiovascular Disease Clinic. During the past ten years, it has become more common to consider genetic testing as an integral part of cardiovascular care. Last year, new guidelines were released and published on the state of genetic testing for channelopathies and cardiomyopathies.¹ These guidelines reflect an improved understanding of many of the rare cardiovascular diseases that can result in sudden death as well as the ability to detect the genetic predisposition for similar diseases in family members.

Based upon the model of the Center for Inherited Heart Disease at Johns Hopkins Hospital, the Inherited Cardiovascular Disease Clinic at the CACC is a referral center for cardiologists and electrophysiologists dealing with suspected inherited causes of sudden cardiac death. The clinic officially started in October 2012 and is currently seeing ten to twelve patients per month as we grow; some of these cases include patients seen at THE HEART HOSPITAL Baylor Plano while inpatient, or, in some cases, some have been seen at outlying clinics with results sent back to and coordinated at the clinic. The goal is to continue to grow steadily and to help as many people as possible. The Inherited Cardiovascular Disease Clinic is staffed by a cardiologist and electrophysiologist with experience gained in academic inherited disease centers; therefore, the clinic can evaluate and treat if necessary a variety of inherited diseases. Typical referrals include suspected or confirmed electrophysiologic diseases such as long QT syndrome, Brugada syndrome, and short QT syndrome. The center evaluates and can treat hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia (ARVD/C) and dilated cardiomyopathy when a familial component is suspected. The clinic also works closely with the surgeons at the CACC when evaluating patients with Marfan syndrome and suspected familial thoracic aortic aneurysm.

Most importantly, our clinic has a dedicated cardiovascular genetic counselor, Laura Panos, MS, CGC. This is critical when developing an inherited cardiovascular disease center, and is the feature that delineates academic cardiovascular genetics programs from general medical genetics clinics. The genetic counselor is able to spend one-on-onetime understanding a multigenerational family history and providing a full explanation of the risks, benefits, and limitations of testing the patients in the clinic. The presence of our dedicated genetics counselor alleviates some of the publicized concerns regarding a potential conflict of interest when cardiologists must rely on genetic counselors supplied by commercial genetic testing companies.

One of the most powerful aspects of combining a team of experienced cardiovascular physicians with a genetics counselor is that a referral is not simply a request for genetic testing, but rather a full clinical evaluation prior to testing that can better ascertain the likelihood and appropriateness of genetic testing as well as confirm or rule out a clinical diagnosis. For example, a clinical diagnosis of Marfan syndrome or ARVD requires a complete exam meeting ever-changing clinical criteria; not all cardiology centers have the ability to fulfill the testing criteria prior to genetic testing. Further, the team takes responsibility to help find and screen family members who may also be affected. Such a responsibility is implied when a physician makes a diagnosis of a disease that is commonly inherited, such as hypertrophic cardiomyopathy.

Although there are academic centers in the state of Texas with interests in specific rare cardiovascular diseases and with access to medical genetics clinics, there are no other dedicated inherited cardiovascular disease clinics staffed with a cardiovascular genetics counselor offering a full range of services for rare inherited disease. Diagnostic modalities available include electrocardiograms (including SAECG), echocardiograms, stress tests, ambulatory telemetry monitors (Holter and event), CT scanning, and cardiac MRI. Additionally, the clinic is staffed to facilitate enrollment and follow-up of patients in the multiple trials taking place at THE HEART HOSPITAL Baylor Plano. Current trials taking place include the CABANA trial, the VERSATILE study, the STOP AF PAS study, and ICY-AVNRT. 

Summary

Family medical history plays an important role in the accurate diagnosis of arrhythmia and other disorders. Family-dominant heart conditions that could warrant genetic counseling include channelopathies (i.e., catecholaminergic polymorphic ventricular tachycardia, cardiomyopathies, and vasculopathies (i.e., Loeys/Dietz syndrome). The Heart Arrhythmia Center is proud to offer genetic counseling services and is one of very few outpatient settings in the North Texas region where such expertise is offered.

For more information, please click here to visit The Heart Hospital Baylor. 

Disclosures: Dr. Edwards has no conflicts of interest to report.  Ms. Panos reports employment with the Baylor Health Care System. Outside the submitted work, Dr. Shapira reports payment for development of educational presentations including service on speakers’ bureaus and travel/accommodations expenses covered or reimbursed by St. Jude Medical; he also reports employment with the Center for Advanced Cardiovascular Care - Heart Arrhythmia Center.   

Reference

1. Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm. 2011;8(8):1308-1339.

Disclaimer: Notice Regarding Physician Ownership: THE HEART HOSPITAL Baylor Plano is a hospital in which physicians have an ownership or investment interest. The list of the physician owners or investors is available to you upon request.

Physicians are members of the medical staff at one of Baylor Health Care System’s subsidiary, community or affiliated medical centers and are neither employees nor agents of those medical centers, THE HEART HOSPITAL Baylor Plano or Baylor Health Care System. © 2013 Baylor Health Care System.