Comparison of paediatric patients evaluated for postural orthostatic tachycardia syndrome with and without tachycardia.

Introduction: Postural orthostatic tachycardia syndrome is a debilitating disorder. We compared paediatric patients with this dysautonomia presenting with and without peak upright heart rate > 100 beats per minute.

Materials And Methods: Subjects were drawn from the Postural Orthostatic Tachycardia Syndrome Program database of the Children's Hospital of Philadelphia diagnosed between 2007 and 2018.

Long-Term POTS Outcomes Survey: Diagnosis, Therapy, and Clinical Outcomes.

Background: Limited data exist on long-term outcomes in individuals with postural orthostatic tachycardia syndrome (POTS). We designed an electronic questionnaire assessing various aspects of outcomes among patients diagnosed and treated in a single-center pediatric POTS clinical program.

Methods And Results: The LT-POTS (Long Term POTS Outcomes Survey) included questions about quality of life, symptoms, therapies, education, employment, and social impact of disease.

Assessment of comorbid symptoms in pediatric autonomic dysfunction.

Purpose: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning.

Methods: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI.

Emergency Department utilisation and diagnoses in adolescents with postural orthostatic tachycardia syndrome.

Introduction: Data for Emergency Department utilisation and diagnoses in adolescents with postural orthostatic tachycardia syndrome are lacking, making prevention of these visits more difficult to achieve.

Materials And Methods: We performed a retrospective study of patients with postural orthostatic tachycardia syndrome between ages 12 and 18 years seen in the Emergency Department at a large tertiary care children's hospital. These subjects were age- and sex-matched with controls, with volume of primary and total diagnoses assessed.

Creating a data dictionary for pediatric autonomic disorders.

Purpose: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders.

Methods: Our group created the list of terms for the dictionary.

Pediatric Postural Orthostatic Tachycardia Syndrome: Where We Stand.

Postural orthostatic tachycardia syndrome (POTS), first described in 1992, remains an enigmatic, yet severely and variably debilitating, disorder. The pathophysiology of this syndrome is still not understood, and there remains no biomarker indicating the presence of POTS. Although research interest has increased in recent years, there are relatively fewer clinical and research studies addressing POTS in children and adolescents compared with adults.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code () is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting - Part 2.

The National Institutes of Health hosted a workshop in 2019 to build consensus around the current state of understanding of the pathophysiology of postural orthostatic tachycardia syndrome (POTS) and to identify knowledge gaps that must be addressed to enhance clinical care of POTS patients through research. This second (of two) articles summarizes current knowledge gaps, and outlines the clinical and research priorities for POTS. POTS is a complex, multi-system, chronic disorder of the autonomic nervous system characterized by orthostatic intolerance and orthostatic tachycardia without hypotension.

Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting - Part 1.

Postural orthostatic tachycardia syndrome (POTS) is a chronic and often disabling disorder characterized by orthostatic intolerance with excessive heart rate increase without hypotension during upright posture. Patients often experience a constellation of other typical symptoms including fatigue, exercise intolerance and gastrointestinal distress. A typical patient with POTS is a female of child-bearing age, who often first displays symptoms in adolescence.

Prevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome.

Introduction: Patients with postural orthostatic tachycardia syndrome (POTS) have been shown to exhibit comorbid joint hypermobility manifested as Ehlers-Danlos syndrome (EDS) or hypermobility spectrum disorder (HSD). The prevalence of EDS and HSD in POTS has been demonstrated in smaller studies combining adult and pediatric patients. We examined a large series of pediatric patients to determine their prevalence in children with POTS.

Family history of associated disorders in patients with postural tachycardia syndrome.

Introduction: Postural tachycardia syndrome is more frequently being recognised in adolescents and adults. However, its pathophysiology remains undefined. We evaluated our database for patterns in family history of clinical symptoms and associated disorders in these patients.

Clinical Course of Transgender Adolescents with Complicated Postural Orthostatic Tachycardia Syndrome Undergoing Hormonal Therapy in Gender Transition: A Case Series.

Postural orthostatic tachycardia syndrome (POTS), an increasingly recognized dysautonomia, may affect as many as 3,000,000 Americans. Concurrently, prevalence estimates suggest 10% of individuals identify as lesbian, gay, bisexual, transgender, or questioning/queer. The preponderance of female POTS patients implies hormonal differences between natal sexes and their role in POTS.

Orthostatic heart rate does not predict symptomatic burden in pediatric patients with chronic orthostatic intolerance.

Purpose: Postural orthostatic tachycardia syndrome (POTS) in adults is defined as symptoms of chronic orthostatic intolerance (COI) and autonomic dysfunction (AD) with heart rate (HR) increase of 30 beats per minute (bpm), or HR > 120 bpm, during prolonged upright position. However, in adolescents, POTS is defined as symptoms of OI and AD with HR increase of ≥ 40 bpm, based on tilt table data. We assessed frequency of COI symptoms in pediatric patients versus HR criteria on prolonged standing to evaluate using criteria of increased HR of 30-39 bpm versus ≥ 40 bpm in our POTS Program.

Therapy for fatigue and cognitive dysfunction in postural orthostatic tachycardia syndrome.

IntroductionSevere fatigue and cognitive dysfunction are frequent symptoms in patients with postural orthostatic tachycardia syndrome. They can be debilitating, and often do not resolve despite improvement in haemodynamic symptoms. Our analysis was intended to assess clinical outcomes of medication treatment for these symptoms in a large, single-centre paediatric programme.

Utilisation of medications to reduce symptoms in children with postural orthostatic tachycardia syndrome.

Background: Postural orthostatic tachycardia syndrome encompasses multiple disabling symptoms that interfere with daily activities. Non-pharmacologic approaches can be insufficient and can require adjunctive medications to manage symptoms. Minimal data exist in the literature on medication outcomes in these patients.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases.

Activity and Exercise Intolerance After Concussion: Identification and Management of Postural Orthostatic Tachycardia Syndrome.

Background And Purpose: Postural orthostatic tachycardia syndrome (POTS) is increasingly recognized as a complication affecting recovery from concussion. Individuals with POTS demonstrate refractory dizziness, lightheadedness, cognitive dysfunction, fatigue, headache, chronic pain, nausea and gastrointestinal dysmotility, activity and exercise intolerance, syncope, and tachycardia. Subtypes of POTS may include hypovolemia, hyperadrenergic states, autonomic neuropathy, and underlying autoimmunity, which may variably impact response to rehabilitation in varying ways.

Postural orthostatic tachycardia syndrome in children and adolescents.

Postural orthostatic tachycardia syndrome (POTS) affects up to 3,000,000 people in the United States, with at least one-third of them developing POTS before the age of 18. POTS as a disorder is similar in adult and pediatric populations, but there are factors specific to pediatric patients that affect how it presents and how it is experienced that make pediatric POTS different. This review discusses the both the similarities in this population to their adult counterparts and the unique challenges faced by pediatric POTS patients, including management of schooling and education as well as the complex interactions between these pediatric patients and their parents.

Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program.

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.

Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11).

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many "short list" versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various "short lists".

Pediatric Disorders of Orthostatic Intolerance.

Orthostatic intolerance (OI), having difficulty tolerating an upright posture because of symptoms or signs that abate when returned to supine, is common in pediatrics. For example, ∼40% of people faint during their lives, half of whom faint during adolescence, and the peak age for first faint is 15 years. Because of this, we describe the most common forms of OI in pediatrics and distinguish between chronic and acute OI.