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....................................................................................................................................................................... 6.2 Syncope in paediatric patients 6.2.1 Diagnostic evaluation Diagnostic evaluation in paediatric patients is similar to that in adults.

Two specific conditions399 occur in early childhood: • Infantile reflex syncopal attacks (also called pallid breath-holding spells or reflex anoxic seizures), elicited by a brief unpleasant stimulus, caused by vagally mediated cardiac inhibition. • Cyanotic breath-holding spells, characterized by stopping breathing during crying, leading to cyanosis and usually TLOC. Careful taking of personal and family history and a standard ECG are the most important methods of distinguishing benign reflex syncope (also including reflex anoxic seizure or breathholding spells) from other causes. If the family history is positive, genetic causes of electrical disease of the heart should be considered first. Some children with reflex syncope also have a positive family history.400 Tilt testing seems to have high false-negative and false-positive rates and should be used with caution for the primary identification of reflex syncope. Since tilt protocols commonly used in adults may lack specificity in teenagers, in one study, a shorter tilt test duration of 10 min at 60 or 70 degrees was used and showed a specificity of >85%.401 In young patients, syncope can rarely be the initial manifestation of unusual but life-threatening conditions such as LQTS, Kearns–Sayre syndrome (external ophthalmoplegia and progressive heart block), Brugada syndrome, catecholaminergic polymorphic VT, Wolff-Parkinson-White syndrome, ARVC, HCM, pulmonary arterial hypertension, myocarditis, arrhythmia after repaired congenital heart disease, and anomalous origin of a coronary artery. Some aspects of the history can suggest a cardiac origin, and should prompt cardiac evaluation. • Family history: premature SCD at age <40 years and/or familial heart disease. • Known or suspected heart disease. • Event triggers: loud noise, fright, and/or extreme emotional stress. • Syncope during exercise, including swimming. • Syncope without prodromes, while supine or sleeping, or preceded by chest pain or palpitations.

6.2.2. Therapy The therapeutic approach is the same as in adults. However, it should be stressed that the effectiveness of pharmacological agents and tilt training for recurrent reflex syncope is undetermined in the absence of well-designed paediatric trials. Furthermore, even in the presence of VVS with prolonged asystole, pacemakers should be avoided due to the relatively transient and benign nature of the syndrome.402 In summary, the key points for the evaluation of syncope in paediatrics are as follows: • Syncope in childhood is common, the majority being of reflex origin, with only a minority having a potentially life-threatening cause. • Discriminating benign from serious causes is made primarily by history, physical examination, and ECG results. • Children with a history suggesting VVS, a normal ECG, and no family history of arrhythmia should not undergo further cardiac investigations. • The cornerstone of therapy for young patients with reflex syncope includes education and reassurance.

7 Psychogenic transient loss of

consciousness and its evaluation

In psychogenic TLOC there is no gross somatic brain dysfunction, but the attacks fulfil the criteria for TLOC (see section 3.1). There are two types: PPS and PNES. In PPS movements are absent, so PPS resembles syncope or longer-lasting LOC, whereas in PNES impressive limb movements mean the attacks resemble epileptic seizures. PPS and PNES differ pathophysiologically from the TLOC forms that they resemble: in PPS, BP and HR are normal or high rather than low, and the EEG is normal instead of showing the slowing or flattening typical of syncope; in contrast to epileptic seizures, the EEG in PNES shows no epileptiform brain activity during an attack.9,116 The frequency of PPS and PNES probably depends on the setting. The rate of PPS varies from 1% of patients referred to general syncope clinics94 to 8% of patients referred to specialist neurological clinics,116 but PPS is probably insufficiently recognized.154

7.1 Diagnosis

7.1.1 Historical criteria for attacks The presence of a psychological trauma is not a prerequisite for a diagnosis of conversion. The diagnosis of PPS rests on positive clues taken from the patient’s history and from documenting normal EEG results, HR, or BP during an attack. History taking in PPS usually reveals a combination of the following features116,154,403:

(1) In most cases, the duration of PPS is as short as in syncope, but a much longer duration is a useful diagnostic finding: patients may lie immobile on the floor for 15–30 min. (2) The eyes are usually open in epileptic seizures and syncope but are usually closed in psychogenic TLOC. (3) The attack frequency is high, with several attacks occurring over a week or in a day. (4) There is usually no recognisable trigger, and no sweating, pallor, or nausea beforehand. (5) Injury does not exclude PNES or PPS.

These features should occur together in most attacks. The presence of another pattern of features suggesting a true syncope type, usually VVS, does not argue against a diagnosis of PPS.

7.1.2 Documentation of key features during an attack The following features are relevant during an attack: • Video recording or clinical observation, including provocation of an attack during tilt testing. Primary features: sleep-like body position with closed eyes and lack of response to speech or touch, if Downloaded from academic.oup.com/eurheartj/article/39/21/1883/4939241 by guest on 07 October 2026