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Circulation. 2017;136:e60–e122. DOI: 10.1161/CIR.0000000000000499 August 1, 2017 e89 CLINICAL STATEMENTS AND GUIDELINES

result in a true loss of consciousness, but it is included in the present document because patients appear to exhibit syncope and therefore are referred for evaluation of syncope. Several key clinical features are suggestive of the diagnosis of psychogenic pseudosyncope. None alone, however, provides a definitive diagnosis. Patients with psychogenic pseudosyncope are often young females with a higher prevalence of preexisting V VS or a history of physical and/or sexual abuse.229,525 The appar- ent duration of loss of consciousness is often long (5 to 20 minutes), and episodes are frequent.525 Some common characteristics include closed eyes, lack of pallor and diaphoresis, and usually little physical harm.526 A normal pulse, blood pressure, or EEG during a psychogenic pseudosyncope episode can be documented.229 Although many patients with pseudosyncope can be diagnosed with a careful history, occasionally tilt-table testing with or without transcranial Doppler and monitoring of an EEG is helpful.

Recommendations for the Treatment of Pseudosyncope

COR LOE Recommendations IIb C-LD In patients with suspected pseudosyncope, a candid discussion with the patient about the diagnosis may be reasonable.30,527–529 See Online Data Supplements 38 and 39. Some reports suggest that patients benefit from being informed of the suspected diagnosis in a clear but sympathetic manner that also acknowledges the involuntary nature of the attacks.30,527,528

IIb C-LD Cognitive behavioral therapy may be beneficial in patients with pseudosyncope.530–532 See Online Data Supplements 38 and 39. Uncontrolled studies suggest that psychotherapy, particularly cognitive behavioral therapy, may be beneficial in conversion disorders.530–532 One RCT reported that cognitive behavioral therapy provided a non–statistically significant trend toward improvement in pseudosyncope at 3 months.530 There are no data that support significant benefit from pharmacotherapy.529

9 UNCOMMON CONDITIONS

ASSOCIATED WITH SYNCOPE

Syncope has been reported in many uncommon diseases, according to case reports. However, specific conditions may predispose the patient to various types of syncope. Table 9 provides a list of less common conditions associated with syncope. It is not intended as a reference for differential diagnosis or a complete synopsis of all conditions associated with syncope. Furthermore, it is not necessary to fully evaluate for all these causes when the etiology remains elusive. Most of these presentations rarely cause syncope, and data are sparse. If the cause for syncope is unclear, these conditions could be included in the differential diagnosis on the basis of other clinical characteristics and/ or historical features.

10 AGE, LIFESTYLE, AND SPECIAL

POPULATIONS: RECOMMENDATIONS

10.1 Pediatric Syncope: Recommendations

Syncope is common in the pediatric population. By 18 years of age, it is estimated that 30% to 50% of children experience at least 1 fainting episode, and syncope accounts for 3% of all pediatric ED visits.617–622 The incidence is higher in females and peaks between 15 to 19 years of age.617 Neurally mediated syncope accounts for 75% of pediatric syncope, followed by psychogenic or unexplained syncope in 8% to 15% of cases.623 Breath-holding spells are a form of syncope unique to the pediatric population. Cyanotic breath-holding spells typically occur from age 6 months to age 5 years and may be due to desaturation caused by forced expiration during crying. Pallid breath-holding spells are seen in the first 1 to 2 years of age and may be an early form of V VS. The latter episodes are associated with significant bradycardia and prolonged asystole. Pediatric cardiac syncope may result from obstruction to blood flow (HCM, aortic stenosis, pulmonary hypertension), myocardial dysfunction (myocarditis, cardiomyopathy, congenital coronary anomaly, or post–Kawasaki disease) or a primary arrhythmic etiology (LQTS, CPVT, Brugada syndrome, ARVC, or Wolff-Parkinson-White syndrome).

A detailed history with careful attention to the events leading up to the syncope and a complete physical examination can guide practitioners in differentiating the life-threatening causes of syncope (with potential for injury or SCD) from the more common and benign neurally mediated syncope. A detailed family history, with particular attention to premature SCD among first- and second-degree relatives and the manner in which those deaths occurred, is helpful. Given that many of the causes of non-CHD cardiac syncope in children who do not have a form of CHD are similar to those experienced in an adult cohort (LQTS, HCM, Wolff-Parkinson-White, Brugada, and ARVC), interventions recommended for adults with similar conditions presenting with syncope can be applied in children.

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