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Circulation. 2017;136:e60–e122. DOI: 10.1161/CIR.0000000000000499 August 1, 2017 e91 CLINICAL STATEMENTS AND GUIDELINES

Figure 5. Orthostatic Hypotension. Colors correspond to Class of Recommendation in Table 1. BP indicates blood pressure; and OH, orthostatic hypotension.

10.2 Adult Congenital Heart Disease: Recommendations

Recommendations for ACHD

COR LOE Recommendations IIa C-EO For evaluation of patients with ACHD and syncope, referral to a specialist with expertise in ACHD can be beneficial.

N/A The care of the expanding population of ACHD survivors is complex, especially in patients with moderate-to-severe ACHD. Care providers must be knowledgeable in the anatomy and repair; be vigilant in the recognition and management of HF, arrhythmias, and pulmonary hypertension; and have a deep understanding of noncardiac comorbidities. Delivery of ACHD care in highly specialized centers has been shown to reduce mortality rate.663 In a population-based retrospective study of 71 467 patients with ACHD from Quebec, Canada, between 1990 and 2005, care in a specialized referral center for ACHD care, compared with other care, was independently associated with reduced mortality rate, particularly in those with severe ACHD.663 IIa B-NR EPS is reasonable in patients with moderate or severe ACHD and unexplained syncope.664,665

See Online Data Supplement 40. SCD is a leading cause of death in the patient with ACHD. Unexplained syncope is a concerning event. In a cohort of 252 patients with repaired tetralogy of Fallot undergoing risk stratification with programmed ventricular stimulation, induction of either monomorphic or polymorphic VT predicted future clinical VT and SCD.664 Patients with tetralogy of Fallot and inducible monomorphic or polymorphic VT were more likely to have a history of syncope (42.9%) than were those without inducible VT (13.4%).664 In a cohort study of ICD recipients with transposition of the great arteries after an atrial baffle procedure, 35% of patients with primary-prevention ICDs presented with syncope. In 50% of patients receiving appropriate ICD shocks, atrial tachyarrhythmias preceded or coexisted with VT.665 It is reasonable to exclude atrial arrhythmias in patients with syncope and a CHD substrate at risk of atrial arrhythmias (eg, Mustard, Senning, Fontan, Ebstein anomaly, and tetralogy of Fallot).665

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