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Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program

Published online by Cambridge University Press:  23 January 2018

Jeffrey R. Boris  and
Thomas Bernadzikowski
Jeffrey R. Boris*
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia and The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
Thomas Bernadzikowski
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
*
Author for correspondence: J. R. Boris, MD, Division of Cardiology, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA 19104, United States of America. Tel. 215 590 3180; Fax: 267 426 5324; E-mail: borisj@email.chop.edu
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Abstract

Objective

The aim of this study was to identify and evaluate demographic and clinical features of paediatric patients with postural orthostatic tachycardia syndrome in a tertiary hospital speciality clinic.

Method

This is a retrospective review of clinical data obtained during initial outpatient evaluation.

Results

A total of 708 patients met the evaluation criteria. Female patients outnumbered males, 3.45:1. Caucasians were over-represented at 94.1% of patients. Median age at diagnosis was 15.7 years. Joint hypermobility occurred in 57.3% of patients; 22.4% had hypermobile Ehlers–Danlos syndrome; and 34.9% had hypermobility spectrum disorder. Median age of onset of symptoms was 12.6 years in patients with hypermobility versus 13.7 years in those without (p=0.0001). Median duration of symptoms was 3.3 years with hypermobility versus 1.5 years without (p<0.00001). Putative triggers included infection in 23.6% of patients, concussion in 11.4%, and surgery/trauma in 2.8%. Concurrent inflammatory disorders were noted in 5.2% of patients. Six symptoms comprised 80% of initial patient complaints. Overall, 66% of patients subsequently had at least 10 symptoms, 50% had at least 14 symptoms, and 30% reported at least 26 symptoms. Symptoms were largely cardiovascular, gastrointestinal, and neurological. Paediatric patients with postural orthostatic tachycardia syndrome seen in a large speciality clinic are predominantly female, are mostly Caucasian, have onset of symptoms in early adolescence, and have symptoms for over two years before diagnosis. Over half of patients have joint hypermobility. More than one-third of patients have a possible autoimmune or inflammatory trigger, including infection, concussion, or surgery/trauma. Patients experience symptoms that are highly variable and multi-system in origin over the course of illness.

Keywords

Ehlers–Danlosjoint hypermobilityconcussiondysautonomiaautoimmune
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 668 - 674
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Copyright
© Cambridge University Press 2018 

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Footnotes

*

These data were presented, in part, as an oral presentation at the 27th international meeting of the American Autonomic Society, San Diego, California, 2–5 November, 2016, and as a poster presentation at the 20th Annual Update on Paediatric and Congenital Cardiovascular Disease 2017 conference, Orlando, Florida, 22–26 February, 2017.

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