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August 1, 2017 Circulation. 2017;136:e60–e122. DOI: 10.1161/CIR.0000000000000499 e80

4.2 Structural Conditions: Recommendations

Syncope occurs not infrequently in patients with underlying heart diseases. Comprehensive guidelines exist for diagnosis and management of many of these diseases, including sections on syncope. In this section, management of syncope is discussed in patients with underlying structural heart disease. The diseasespecific ACC/AHA guidelines were assessed first, and then a comprehensive review of literature published since publication of these disease-specific guidelines was performed to ensure that prior recommendations about syncope remained current. If new published data were available, they were incorporated into the present document.

4.1.3 Ventricular Arrhythmia: Recommendation

Recommendation for VA

COR LOE Recommendation

I C-EO In patients with syncope and VA, GDMT is recommended.12,13,220,264,271

N/A Patients with VA (monomorphic or polymorphic) can present with syncope, whether it is nonsustained or sustained. The mechanism of syncope from VA is multifactorial, including: rapid rate, abrupt change in rate, abnormal atrial and ventricular activation relationships, dyssynchrony of ventricular activation, changes in autonomic tone, and body position during the VA.272 One study of 113 patients with sustained VA showed that patients who had a mean VA rate of ≥200 bpm had a 65% incidence of syncope or near-syncope, compared with only 15% among patients with a rate <200 bpm.273 Of the patients with VA ≥200 bpm, 34% did not experience syncope or presyncope. The risk of recurrent syncope and the overall long-term prognosis of patients with VA depend on the severity of the underlying cardiac disease substrates. Indications for ICDs in patients with syncope and suspected VA are predicated on the documentation of or the risk of developing lethal VA.12

4.2.1 Ischemic and Nonischemic Cardiomyopathy: Recommendation

Recommendation for Ischemic and Nonischemic Cardiomyopathy

COR LOE Recommendation

I C-EO In patients with syncope associated with ischemic and nonischemic cardiomyopathy, GDMT is recommended.12,13

N/A Evaluation of syncope in patients with ischemic and nonischemic cardiomyopathy encompasses diagnosis and prognosis. Treatment of syncope is based on the specific cause of syncope, whereas treatment for the underlying cardiomyopathy impacts the longterm prognosis. A review of evidence supports previously published recommendations for patients with syncope in the presence of underlying cardiomyopathy. An ICD is recommended in patients with syncope of undetermined origin with clinically relevant and significant VA induced at the time of an EPS.28 ICD therapy is also reasonable for patients with unexplained syncope and nonischemic dilated cardiomyopathy with significant LV dysfunction.12,13,28

4.2.2 Valvular Heart Disease: Recommendation

Recommendation for Valvular Heart Disease

COR LOE Recommendation

I C-EO In patients with syncope associated with valvular heart disease, GDMT is recommended.11

N/A Patients with aortic stenosis may experience syncope during exertion. The mechanism is often hemodynamic, as opposed to arrhythmic, because of inability to augment and sustain cardiac output. In patients with valvular heart disease causing syncope, treatment is recommended by the latest guidelines.11 Specifically, aortic valve replacement is recommended in patients with severe aortic stenosis and syncope after other causes of syncope are also considered and excluded.

4.2.3 Hypertrophic Cardiomyopathy: Recommendation

Recommendation for HCM

COR LOE Recommendation

I C-EO In patients with syncope associated with HCM, GDMT is recommended.20

N/A A MEDLINE search and review of papers on syncope and HCM has been performed since the last guideline was published in 2011.20 There are no new data that would alter the 2011 recommendations. Thus, the writing committee supports the previous recommendations pertaining to syncope in patients with HCM. Although there are no randomized trials, data from registries have shown consistently that unexplained syncope is an independent predictor for SCD and appropriate ICD discharges. The present writing committee concurs that ICD implantation is reasonable in patients with HCM presenting with ≥1 recent episodes of syncope suspected to be of arrhythmic nature.

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