Inherited Arrhythmias Program

DNA

Inherited arrhythmia syndromes result from disorders in the heart's electrical system and are being recognized with increased frequency in both children and adults. Often, the heart is structurally normal.

The most common form of an inherited arrhythmia is Long QT Syndrome (LQTS). The condition leaves patients at risk for developing a rapid chaotic arrhythmia called Torsades de Pointes. This condition may lead to fainting spells, or in some patients cardiac arrest and possibly sudden death. LQTS can be inherited and several members in multiple generations of a family can be affected. Congential LQTS is estimated to affect one in every 5,000 persons in the United States. LQTS also can be caused by common drugs and medications. Multiple gene (DNA) abnormalities have now been implicated in causing several inherited arrhythmia syndromes including LQTS, Brugada syndrome, catechomaminergic ventricular tachycardia, sinus node and conduction defects, atrial fibrillation, Andersen's syndrome, and short QT syndrome.

The Inherited Arrhythmia Clinic was established to meet the needs of individuals and families with possible inherited arrhythmia syndromes. The physicians and staff bring together expertise in the diagnosis and treatment of these syndromes. This specialized clinic is a part of the Cellular and Molecular Arrhythmia Research Program at the University of Wisconsin-Madison.

Patients referred to the Inherited Arrhythmia Clinic should bring with them past medical records and detailed family histories. Testing may need to be done including simple blood tests, EKGs, Holter monitors, echocardiograms, stress tests and potentially genotyping for genetic diagnosis.

Treatment for inherited arrhythmia syndromes, including LQTS, may involve limiting physical activity, taking medications to prevent the development of a chaotic heart rhythm or avoiding other types of medications. Some people also need a pacemaker to control heart rhythm or an implantable cardioverter-defibrillator (ICD) to prevent sudden death.

The links below provides more information to patients and physicians on these diseases:

For patients: http://www.hrspatients.org/
For drugs to avoid: www.azcert.org
Long QT Arrhythmia


Contact Information

Kathy Hill, RN
Cardiovascular Medicine
600 Highland Ave., G3/4 CSC
Madison, WI 53792
Phone: (608) 263-1530

Jayme Frank, RN, MS, CPNP
UW Children's Hospital
600 Highland Ave., H4/414 CSC
Madison, WI 53792
Phone: (608) 263-8776


Inherited Arrhythmias Program Faculty

L. Lee Eckhardt, MD
Craig January, MD, PhD
Timothy Kamp, MD, PhD
Kathleen Maginot, MD
Jonathan Makielski, MD