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While flecainide have a more favourable adverse effect profile regarding organ toxicity, increased mortality has previously been shown in patients with MI.556 For selected patients with suspected PVC-induced cardiomyopathy and PVC-aggravated cardiomyopathy, flecainide can still be considered, particularly if an ICD is in place (Table 9). A rare monogenetic cause of PVC-induced cardiomyopathy, referred to as multifocal ectopic Purkinje-related premature contractions, has been reported. It is characterized by a DCM phenotype and the presence of numerous PVCs with RBBB and/or left bundle branch block (LBBB) morphologies and an increased risk of SCD.627 Pathogenic mutations in the SCN5A gene lead to a gain of function of the sodium channel responsible for hyperexcitability of the fascicular Purkinje system.627,628 Limited data suggest that patients with multifocal ectopic Purkinje-related premature contractions do not respond to beta-blockers but might benefit from therapy with flecainide, quinidine, or amiodarone.627–631

N

N N

N Patient with PVC-Bu r den >10% and mild l y r educed or r educed L VEF

L L

CMR (Class IIa)

Suspected PVC induced

ca r dio m y opat h y SHD aggr a vated

a a b y f r equent PVC ’ s

Fu r ther e valuation

acco r ding to most li k e

k k l y diagnosis

(suspicious) C A D , valvular hea r t disease or inflammato r y hea r t disease L V dysfunction

L L explained b y under l ying

hea r t disease

Kn o wn SHD

LGE F oll o w-up

Y

Y Y

Y

Amioda r one (Class IIa)

Catheter ablation

(Class IIa)

Catheter ablation

(Class I) AAD (Class IIa)

Figure 18 Algorithm for the management of patients with premature ventricular complex-induced/-aggravated cardiomyopathy. AAD, anti-arrhythmic drug; CAD, coronary artery disease; CMR, cardiac magnetic resonance; LGE, late gadolinium enhancement; LV, left ventricular; LVEF, left ventricular ejection fraction; N, No; PVC, premature ventricular complex; SHD, structural heart disease; Y, Yes.

Recommendation Table 27 — Recommendations for the management of patients with premature ventricular complex-induced or premature ventricular complexaggravated cardiomyopathy

Recommendation Classa Levelb

Diagnostic evaluation In patients with an unexplained reduced EF and a PVC burden of at least 10%, PVC-induced cardiomyopathy should be considered.600,609,610 IIa C In patients with suspected PVC-induced cardiomyopathy, CMR should be considered.590,615 IIa B Treatment In patients with a cardiomyopathy suspected to be caused by frequent and predominately monomorphic PVCs, catheter ablation is recommended.535,600,609,612,617,618,620 I C

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