Summary
Background
Syncopes are frequent in the paediatric population. Most are benign, but rare cases are caused by cardiac life-threatening diseases. Syncope units developed in the adult population have demonstrated improvement in evaluation and treatment, with a reduction in hospitalization.
Aims
We report our experience of paediatric syncope management in a dedicated unit, and analyse the value of different elements in the identification of cardiac causes.
Methods
This prospective study included 97 consecutive patients (mean age: 12.1 ± 3.3 years) referred between January 2011 and June 2013 to a syncope unit with a paediatric cardiologist, a nurse, a physiotherapist and a psychologist. Patients were classified into diagnostic categories after an initial evaluation that included history, physical examination, electrocardiography, echocardiography and Holter monitoring.
Results
The most common diagnosis was neurocardiogenic syncope ( n = 69, 70.4%). Fifty-two cases (81.3%) had no or less recurrence after specific management that included physiotherapy and psychological support (follow-up: 11.5 ± 5.4 months). Psychogenic pseudosyncopes affected 20 children (20.6%). Two patients had epileptic seizures. There were five cases of cardiac syncope (5.1%): two long QT syndromes and a catecholaminergic polymorphic ventricular tachycardia received beta-blockers; two atrioventricular complete blocks required pacemakers. One case was of indeterminate cause and received an insertable loop recorder after exhaustive investigations. Exercise-induced syncopes were significantly associated with cardiac origins ( P = 0.003), such as electrocardiographic abnormalities ( P < 0.001), whereas echocardiography was not contributive.
Conclusion
Syncope units in the paediatric population may be useful in the diagnostic process, to help identify rare cardiac aetiologies, and could decrease recurrence through specific management.
Résumé
Contexte
Les syncopes sont un problème fréquent en pédiatrie ; la plupart sont bénignes mais quelques cas sont dus à des pathologies cardiaques potentiellement menaçantes. Les unités syncope développées chez l’adulte ont montré un bénéfice dans la prise en charge diagnostique et thérapeutique en réduisant les hospitalisations.
Objectifs
Nous rapportons notre expérience de prise en charge des syncopes en pédiatrie par une unité spécialisée en analysant la valeur de différents éléments pour discriminer les causes cardiaques.
Méthodes
Cette étude prospective a inclus 97 patients (âge moyen : 12,1 ± 3,3 ans) consécutivement adressés entre janvier 2011 et juin 2013 à une unité impliquant cardiologue pédiatrique, infirmière, kinésithérapeute et psychologue. Les patients étaient classés en catégories de diagnostic après évaluation initiale comprenant histoire médicale, examen physique, électrocardiogramme, échocardiographie et holter rythmique.
Résultats
Le diagnostic le plus fréquent était les syncopes neurocardiogéniques : 69 patients (70,4 %). Cinquante-deux cas (81,3 %) présentaient moins de récidives après prise en charge spécifique comprenant kinésithérapie et support psychologique (suivi de 11,5 ± 5,4 mois). Les pseudosyncopes psychogéniques représentaient 20 enfants (20,6 %). Deux patients présentaient une épilepsie. Les syncopes cardiaques comprenaient 5 cas (5,1 %) : 2 syndromes du QT long et une tachycardie ventriculaire catécholergique traités par bêtabloquants, 2 blocs atrio-ventriculaires complets nécessitant un pacemaker. Un cas de syncope inexpliquée fut appareillé d’un holter implantable. Les syncopes à l’effort étaient significativement associées à une origine cardiaque ( p = 0,003), comme les anomalies électrocardiographiques ( p < 0,001) alors que l’échocardiographie s’avérait non contributive.
Conclusion
Une unité syncope pédiatrique apparaît intéressante dans la démarche diagnostique pour identifier les rares étiologies cardiaques et pourrait réduire les récidives via une prise en charge spécifique.
Background
Syncope is defined as a “transient loss of consciousness (LOC) due to transient global cerebral hypoperfusion characterized by rapid onset, short duration and spontaneous complete recovery”. Presyncope is “a state that resembles the prodrome of syncope, but which is not followed by LOC” .
Syncope is a common problem in children and adolescents; nearly 15% of this population will have at least one syncopal event before the age of 18 years, with a female predominance and an incidence peak in those aged 15–19 years . The recurrence rate ranged from 33% to 51% in a 5-year follow-up . While most cases of syncope are benign, around 5% are the initial manifestation of a life-threatening cardiac disease . Thus, diagnostic evaluation has to fulfil two nearly opposite objectives: to reassure large numbers of patients and anxious parents, without missing rare cardiac causes that have to be recognized promptly as they can be fatal.
Issues are similar in the adult population, and led to the development of specific syncope units, which have demonstrated improvement in the diagnostic and management processes, with reductions in duration of hospitalization and favourable long-term outcomes .
In this article, we aim to report our experience of syncope evaluation and treatment in children and adolescents in a dedicated syncope unit, with a focus on the specific management of reflex neurocardiogenic syncope (NCS), and the effect on recurrence rates.
Methods
This prospective study included patients aged from 4 to 18 years who were consecutively referred, between 01 January 2011 and 30 June 2013, for syncope evaluation to a paediatric syncope unit in our department of paediatric cardiology, after clinical assessment by general practitioners or within the paediatric emergency unit of our university hospital. This dedicated unit has experienced and trained personnel, including a paediatric cardiologist, a nurse, a physiotherapist and a psychologist. Children and adolescents with a history of structural heart disease, channelopathy or arrhythmia were excluded from the sample. The execution of the study conformed to the principles outlined in the Declaration of Helsinki on research in human subjects. Children were included after informed consent was obtained from their parents. The study was approved by the institutional ethics committee.
Initial assessment
All patients underwent an initial evaluation, which included medical history, physical examination, 12-lead electrocardiogram, echocardiography and 24-hour Holter monitoring. Standardized exercise stress testing and tilt testing (stabilization phase of 15 minutes in supine position, passive phase of 20 minutes at a tilt angle of 60 degrees and, if no symptom occurred, provocation phase of a further 15 minutes after 400 μg nitroglycerin delivered sublingually ; test was considered positive when symptoms were reproduced in association with hypotension and/or bradycardia) were very often proposed as systematic complementary investigations. In rare cases, other targeted specific tests were carried out according to suspected underlying cause: blood tests, genetic investigations, drug tests, such as isoproterenol infusion (45 μg/min in 3 min) and epinephrine infusion (0.05 μg/kg per min to 0.3 μg/kg per min in 20 min), and imaging, including computed tomography (CT) and resonance magnetic imaging (MRI).
Data systematically collected regarding medical history included family history of syncope or cardiac disease, previous episodes of fainting, prodomes, associated symptoms (such as palpitations or chest pain) and triggers (effort, stress, pain, etc.).
After initial assessment, patients were classified into five diagnostic categories, according to published data : NCS, cardiac syncope, psychogenic pseudosyncope (PPS), neurological disorder and indeterminate cause ( Table 1 ). A diagnosis of PPS was made after the exclusion of other causes, and was confirmed by the psychologist after clinical evaluation. The distribution of associated circumstances or signs and test results were examined in the different diagnostic categories, especially in order to compare cardiac syncopes versus other causes.
| Categories | Included conditions |
|---|---|
| NCS | Classical vasovagal syncopes and other neurally mediated syncopal syndromes, including situational syncope, breath-holding spells, postural orthostatic tachycardia syndromes |
| Cardiac syncopes | Arrhythmias, long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, Wolff-Parkinson-White syndrome, right ventricular arrhythmogenic cardiomyopathy, hypertrophic cardiomyopathy, pulmonary arterial hypertension, myocarditis, tumour, congenital heart disease or anomalous origin of a coronary artery |
| PPS | Panic attacks, anxiety, hysteria, depression, without clinical argument for NCS |
| Neurological disorders | Seizures, strokes, migraine, meningitis |
Management of NCS
Patients with a diagnosis of NCS underwent specific management in the syncope unit, consisting of information, reassurance, general recommendations (hydration, increasing salt intake, avoiding hot conditions and situations inducing syncope), physiotherapy training (isometric pressure movements) and consultation with the psychologist.
Individual physiotherapy sessions focused on education about recognizing early symptoms and preventive measures. Physical training included exercise and manoeuvres consisting of isometric muscle contractions, such as leg crossing and arm gripping . Manoeuvres were explained, demonstrated and then practised under supervision. Patients were instructed to maintain the manoeuvre as long as possible, and to move on to a second manoeuvre in the absence of complete disappearance of symptoms; they were encouraged to practise these manoeuvres and exercise regularly.
Patients and their parents were contacted in order to document recurrence of syncope and presyncope, and were asked to assess if there was a significant improvement in the situation in case of recurrent events.
Statistical analysis
Data are expressed as means ± standard deviations for continuous variables or as numbers and percentages for categorical variables. The paired t -test, Fisher’s test, the χ 2 test and linear regression were used to assess differences between subgroups, as appropriate. Statistical significance was set at P < 0.05. Data analysis was performed using STATA ® software (StataCorp LP, College Station, TX, USA).
Results
Population characteristics
Between 01 January 2011 and 30 June 2013, 97 patients were prospectively enrolled (mean age 12.1 ± 3.3 years; 47.4% males). The 13–15 age group was predominant ( Fig. 1 ). Seventy-eight patients (80.4%) had typical syncopes, most of the other 19 patients presented only symptoms of presyncope, and the few remaining cases had another clinical presentation. Medical history revealed at least one previous episode for 80 patients (82.5%). Prodomes concerned 67 children (69.1%), and consisted of lightheadedness, blurred vision, sweating, nausea, abdominal pain and/or tinnitus. LOC caused trauma in nine cases, and symptoms were exercise-induced in eight cases. Other variables are reported in Table 2 .
| Characteristic | |
|---|---|
| Females | 51 (52.6) |
| Mean age (years) | 12.1 ± 3.3 |
| Syncope | 78 (80.4) |
| Recurrent episode | 80 (82.5) |
| Trauma | 9 (9.3) |
| Prodomal symptoms | 67 (69.1) |
| Palpitations | 14 (14.4) |
| Chest pain | 8 (8.2) |
| Exercise-induced | 8 (8.2) |
| Hospitalization | 84 (86.6) |
| Number of days | 1.1 ± 1.0 |
Effort tests were done in 76 cases (78.0%), as well as tilt tests in a similar number of patients ( n = 74; 76.3%). Other examinations were rarely performed ( Table 3 ).
