Elsevier

Heart Rhythm

Volume 12, Issue 6, June 2015, Pages e41-e63
Heart Rhythm

2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope

https://doi.org/10.1016/j.hrthm.2015.03.029Get rights and content

Introduction

This international consensus statement was written by experts in the field who were chosen by the Heart Rhythm Society, in collaboration with representatives from the American Autonomic Society (AAS), the American College of Cardiology (ACC), the American Heart Association (AHA), the Asia Pacific Heart Rhythm Society (APHRS), the European Heart Rhythm Association (EHRA), the Pediatric and Congenital Electrophysiology Society (PACES), and the Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE)-Latin American Society of Cardiac Pacing and Electrophysiology.

This document is intended to help front-line cardiologists, arrhythmia specialists, and other health care professionals interested in the care of patients who present with presumed postural tachycardia syndrome (POTS), inappropriate sinus tachycardia (IST), and vasovagal syncope (VVS). It is not intended to be a comprehensive narrative review, as excellent reviews, chapters, and entire volumes have appeared recently.1, 2, 3 This document has 3 objectives: (1) establish working criteria for the diagnosis of POTS, IST, and VVS; (2) provide guidance and recommendations on their assessment and management; and (3) identify key areas in which knowledge is lacking, to highlight opportunities for future collaborative research efforts.

To maintain this pragmatic focus, we excluded several related topics, including a detailed approach to syncope and other syndromes of transient loss of consciousness, the impact of syncope on other disorders, most orthostatic hypotension syndromes, the effects of the autonomic system on arrhythmias, the use of syncope scores or syncope units, and recommendations on training programs and staffing criteria. A number of sections contain very brief reviews, given that the material has recently been covered elsewhere. We refer readers to the excellent European Society of Cardiology guidelines2 and related recent reviews.1, 4

The writing group aimed to provide a succinct, evidence-based document at a uniform level, rather than a comprehensive narrative review. As much as possible, we made recommendations based on published evidence. There was a wide range in terms of the level of evidence available, and we included the highest-level evidence for each section. Inevitably, this led to heterogeneity in the level of evidence included. Each section, indeed the entire document, is a compromise among clinical need, succinctness, clarity, and level of evidence. The specific wording of definitions, recommendations, and the choice of references were the result of prolonged debate, consensus-seeking, and repeated votes.

Each section was drafted by compact writing groups with 3–5 members who completed the first versions and developed preliminary recommendations. The group assignments were based on individual interests and expertise. The recommendations and text underwent iterative revisions to resolve differences, increase clarity, and align the document format with that recommended by the American College of Cardiology.5 All members of the writing group and peer reviewers provided disclosure statements of all relationships that might present real or perceived conflicts of interest, as shown in the Appendices.

The recommendations and definitions in this document are based on the consensus of the full writing group following the Heart Rhythm Society’s process for establishing consensus-based guidance for clinical care. To identify consensus, we conducted surveys of the entire writing group, using a predefined threshold for agreement as a vote of >75% on each recommendation. An initial failure to reach consensus was resolved by subsequent discussion and re-voting. The final minimum consensus was 76% and the mean was 94%.

The consensus recommendations in this document use the commonly used class I, IIa, IIb, and III classifications and the corresponding language according to the most recent statement of the American College of Cardiology.6 Class I is a strong recommendation, denoting benefit greatly exceeding risk. Class IIa is a somewhat weaker recommendation, denoting benefit probably exceeding risk, and class IIb denotes benefit equivalent or possibly exceeding risk. Class III is a recommendation against a specific treatment, because either there is no net benefit or there is net harm. Level A denotes the highest level of evidence, usually from multiple clinical trials with or without registries. Level B evidence is of a moderate level, either from randomized trials (B-R) or well-executed nonrandomized trials (B-NR). Level C evidence is from weaker studies with significant limitations, and level E is simply a consensus opinion in the absence of credible published evidence.

When considering the guidance provided in this document, it is important to remember that there are no absolutes with regard to many clinical situations. The writing group was struck by the large number of issues lacking high-level evidence. To this end, the document provides evidence-informed recommendations, striking a balance between the need for recommendations and the availability of evidence. Health care providers and patients need to jointly make the final decision regarding care in light of their individual circumstances.

Section snippets

Definition

POTS is a clinical syndrome usually characterized by (1) frequent symptoms that occur with standing, such as lightheadedness, palpitations, tremor, generalized weakness, blurred vision, exercise intolerance, and fatigue; (2) an increase in heart rate of ≥30 beats per minute (bpm) when moving from a recumbent to a standing position (or ≥40 bpm in individuals 12 to 19 years of age); and (3) the absence of orthostatic hypotension (>20 mm Hg drop in systolic blood pressure). The symptoms associated

Definition

The syndrome of IST is defined as a sinus heart rate >100 bpm at rest (with a mean 24-hour heart rate >90 bpm not due to primary causes) and is associated with distressing symptoms of palpitations.

Definition: Inappropriate Sinus Tachycardia

The syndrome of inappropriate sinus tachycardia is defined as a sinus heart rate >100 bpm at rest (with a mean 24-hour heart rate >90 bpm not due to primary causes) and is associated with distressing symptoms of palpitations.

Epidemiology and Natural History

The prevalence of IST was

Definition

Syncope is defined as a transient loss of consciousness, associated with an inability to maintain postural tone, rapid and spontaneous recovery, and the absence of clinical features specific to another form of transient loss of consciousness such as epileptic seizure. “Clinical features” indicates all the information obtained from the history, physical signs, and feasible, reasonable, limited investigations such as an ECG.

Definition: Syncope

Syncope is defined as a transient loss of

Section 4: Postural Tachycardia Syndrome and Vasovagal Syncope in the Young

The relatively limited pediatric literature on this topic has meant that most insights and treatments for POTS and vasovagal syncope have come from the adult medical literature. This section provides a high-level view of the features shared by adults and children and comments on the evidence, research, and treatments specific to children and adolescents.

Section 5: Future Opportunities

The writing group identified numerous opportunities for better understanding the causes, diagnosis, risk stratification, and treatment of these disorders. The first and formative step is to agree on uniform definitions for the syndromes, which the Heart Rhythm Society provides in this document. Once validated, these definitions will provide uniform criteria for inclusion of patients in clinical studies. Many subsequent studies will require multiple centers, and the formation of standing

References (147)

  • P.A. Low et al.

    Comparison of the postural tachycardia syndrome (POTS) with orthostatic hypotension due to autonomic failure

    J Auton Nerv Syst

    (1994)
  • D. Wallman et al.

    Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: a relationship study

    J ournal Neurol Sci

    (2014)
  • X.L. Wang et al.

    Autoimmunoreactive IgGs against cardiac lipid raft-associated proteins in patients with postural orthostatic tachycardia syndrome

    Transl Res

    (2013)
  • Q. Fu et al.

    Cardiac origins of the postural orthostatic tachycardia syndrome

    J Am Coll Cardiol

    (2010)
  • F.A. Gaffney et al.

    Effects of long-term clonidine administration on the hemodynamic and neuroendocrine postural responses of patients with dysautonomia

    Chest

    (1983)
  • E.M. Garland et al.

    Chiari I malformation as a cause of orthostatic intolerance symptoms: a media myth?

    Am J Med

    (2001)
  • P.A. Chiale et al.

    Inappropriate sinus tachycardia may be related to an immunologic disorder involving cardiac beta adrenergic receptors

    Heart Rhythm

    (2006)
  • A. Castellanos et al.

    Heart rate variability in inappropriate sinus tachycardia

    Am J Cardiol

    (1998)
  • N.F. Marrouche et al.

    Three-dimensional nonfluoroscopic mapping and ablation of inappropriate sinus tachycardia. Procedural strategies and long-term outcome

    J Am Coll Cardiol

    (2002)
  • R. Cappato et al.

    Clinical efficacy of ivabradine in patients with inappropriate sinus tachycardia: a prospective, randomized, placebo-controlled, double-blind, crossover evaluation

    J oAm Coll Cardiol

    (2012)
  • L.Y. Chen et al.

    Prevalence and clinical outcomes of patients with multiple potential causes of syncope

    Mayo Clin Proc

    (2003)
  • F. D’Ascenzo et al.

    Incidence, etiology and predictors of adverse outcomes in 43,315 patients presenting to the Emergency Department with syncope: an international meta-analysis

    Int J Cardiol

    (2013)
  • G. Glick et al.

    Hemodynamic changes during spontaneous vasovagal reactions

    Am J Med

    (1963)
  • D.E. Manyari et al.

    Abnormal reflex venous function in patients with neuromediated syncope

    J Am Coll Cardiol

    (1996)
  • B. Verheyden et al.

    Steep fall in cardiac output is main determinant of hypotension during drug-free and nitroglycerine-induced orthostatic vasovagal syncope

    Heart Rhythm

    (2008)
  • J. Gisolf et al.

    Sublingual nitroglycerin used in routine tilt testing provokes a cardiac output-mediated vasovagal response

    J Am Coll Cardiol

    (2004)
  • Q. Fu et al.

    Pathophysiology of neurally mediated syncope: Role of cardiac output and total peripheral resistance

    Auton Neurosci

    (2014)
  • R. Sheldon

    How to differentiate syncope from seizure

    Card Electrophysiol Clin

    (2013)
  • R. Sheldon et al.

    Historical criteria that distinguish syncope from seizures

    J Am Coll Cardiol

    (2002)
  • P. Alboni et al.

    Diagnostic value of history in patients with syncope with or without heart disease

    J Am Coll Cardiol

    (2001)
  • S. Sud et al.

    Predicting the cause of syncope from clinical history in patients undergoing prolonged monitoring

    Heart Rhythm

    (2009)
  • P. Saklani et al.

    Syncope

    Circulation

    (2013)
  • A. Moya et al.

    Guidelines for the diagnosis and management of syncope (version 2009)

    Eur Heart J

    (2009)
  • R. Schondorf et al.

    Idiopathic postural orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia?

    Neurology

    (1993)
  • P.A. Low et al.

    Postural tachycardia syndrome (POTS)

    Neurology

    (1995)
  • E.M. Garland et al.

    The hemodynamic and neurohumoral phenotype of postural tachycardia syndrome

    Neurology

    (2007)
  • P.A. Low et al.

    Postural tachycardia syndrome (POTS)

    J Cardiovasc Electrophysiol

    (2009)
  • R. Freeman et al.

    Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome

    Clin Auton Res

    (2011)
  • I. Lewis et al.

    Clinical characteristics of a novel subgroup of chronic fatigue syndrome patients with postural orthostatic tachycardia syndrome

    J Intern Med

    (2013)
  • A.C. Peltier et al.

    Distal sudomotor findings in postural tachycardia syndrome

    Clin Auton Res

    (2010)
  • W. Singer et al.

    Prospective evaluation of somatic and autonomic small fibers in selected autonomic neuropathies

    Neurology

    (2004)
  • E.R. Vogel et al.

    Blood pressure recovery from Valsalva maneuver in patients with autonomic failure

    Neurology

    (2005)
  • P. Sandroni et al.

    Mechanisms of blood pressure alterations in response to the Valsalva maneuver in postural tachycardia syndrome

    Clin Auton Res

    (2000)
  • G. Jacob et al.

    The neuropathic postural tachycardia syndrome

    N Engl J Med

    (2000)
  • J.M. Stewart et al.

    Decreased skeletal muscle pump activity in patients with postural tachycardia syndrome and low peripheral blood flow

    Am J Physiol Heart Circ Physiol

    (2004)
  • J.M. Stewart et al.

    Persistent splanchnic hyperemia during upright tilt in postural tachycardia syndrome

    Am J Physiol Heart Circ Physiol

    (2006)
  • J.M. Stewart et al.

    Regional blood volume and peripheral blood flow in postural tachycardia syndrome

    Am J Physiol Heart Circ Physiol

    (2004)
  • H.S.W. Tani et al.

    Splanchnic and systemic circulation in the postural tachycardia syndrome

    Clin Auton Res

    (1999)
  • H. Li et al.

    Autoimmune basis for postural tachycardia syndrome

    JAMA

    (2014)
  • S.R. Raj et al.

    Renin-aldosterone paradox and perturbed blood volume regulation underlying postural tachycardia syndrome

    Circulation

    (2005)
  • Cited by (0)

    Developed in collaboration with and endorsed by the American Autonomic Society (AAS), the American College of Cardiology (ACC), the American Heart Association (AHA), the AsiaPacific Heart Rhythm Society (APHRS), the European Heart Rhythm Association (EHRA), the Pediatric and Congenital Electrophysiology Society (PACES), and the Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE)-Latin American Society of Cardiac Pacing and Electrophysiology.

    ?>*

    Representative for the European Heart Rhythm Association (EHRA);

    ?>†

    Representative for the Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE);

    ?>‡

    Representative for the Asia-Pacific Heart Rhythm Society (APHRS);

    §

    Representative for the American Heart Association (AHA);

    ?>**

    Representative for the American Autonomic Society (AAS);

    ?>††

    Representative for the American College of Cardiology (ACC);

    ?>§§

    Representative for the Pediatric and Congenital Electrophysiology Society (PACES)

    View full text