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Societies release consensus on diagnosis, management of inherited arrhythmias
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DENVER — The Heart Rhythm Society, European Heart Rhythm Association and Asia Pacific Heart Rhythm Society have collaborated to release the first comprehensive statement of recommendations for the diagnosis and management of patients with inherited primary arrhythmia syndromes.
This is the first guideline on ventricular arrhythmias and sudden death since 2006.
The HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes “deals with uncommon conditions with an estimated prevalence of one in 1,000 to one in 10,000,” writing group chair Silvia G. Priori, MD, PhD, director of molecular cardiology and electrophysiology laboratories, University of Pavia, Italy, said at a round table discussion.
New guidance on rare syndromes
The consensus statement is designed to provide clinical guidance for the diagnosis, risk stratification and management of patients with inherited arrhythmias that can increase risk for sudden cardiac death, especially in young individuals, according to a press release.
For the first time, the statement includes a consensus of recommendations for the following syndromes, according towriting group chair Arthur Wilde, MD, PhD, of the University of Amsterdam:
- Catecholaminergic polymorphic ventricular tachycardia (CPVT);
- Short QT syndrome;
- Early repolarization; and
- Progressive cardiac conduction disease (PCCD).
Also featured in the guidelines are recommendations for long QT syndrome, Brugada syndrome and unexplained cardiac arrest, including idiopathic ventricular fibrillation, sudden unexplained infant death syndrome and sudden unexplained death in infancy.
Changes from previous guidelines
One new primary recommendation is that young patients and first-degree relatives with diagnosed or suspected inherited CVD should be evaluated in a dedicated clinic with appropriately trained clinical staff and resources to provide support to the patient and family members.
According to Priori, dedicated referral centers should have at least one expert in inherited arrhythmias with experience in diagnosing and managing these patients and awareness of the literature and updated data.
Other changes include new diagnostic criteria for Brugada syndrome based on ECG alone. Now the recommendation is based on a “pure electrocardiographic diagnosis,” Wilde said.
For patients with long QT syndrome, those who wish to engage in competitive sports should be referred to a clinical expert in inherited arrhythmias for evaluation of risk. Traditionally, patients with long QT syndrome, particularly type 1, have been discouraged or not permitted to participate in competitive sports.
For use by all health care professionals
The recommendations are the summarized opinions of the expert writing group, based on literature review and personal experience. The guidance is designed for use by all health care professionals who manage patients who survived cardiac arrest at a young age, family members of those who died of sudden cardiac arrest, patients with diagnosed arrhythmias or young individuals with unexplained syncope, according to the release.
The statement does not address indications for genetic testing nor does it specify a list of US centers with arrhythmia services.
Andrea M. Russo
“This is a particularly important document that we have been waiting for,” said round table moderator Andrea M. Russo, MD, FHRS, director of cardiac ablation and arrhythmia services at Cooper University Hospital, Camden, N.J.
The document will be published simultaneously in Heart Rhythm, the Journal of Arrhythmias and EP Europace.