Comparison of patients with early-phase arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract ventricular tachycardia

Abstract

AimsDifferentiation between early-phase arrhythmogenic right ventricular cardiomyopathy (ARVC) and right ventricular outflow tract (RVOT)-ventricular tachycardia (VT) can be challenging, and correct diagnosis is important. We compared electrocardiogram (ECG) parameters and morphological right ventricular (RV) abnormalities and investigated if ECG and cardiac imaging can help to discriminate early-phase ARVC from RVOT-VT patients.Methods and resultsWe included 44 consecutive RVOT-VT (47 ± 14 years) and 121 ARVC patients (42 ± 17 years). Of the ARVC patients, 77 had definite ARVC and 44 had early-phase ARVC disease. All underwent clinical examination, ECG, and Holter monitoring. Frequency of premature ventricular complexes (PVC) was expressed as percent per total beats/24 h (%PVC), and PVC configuration was recorded. By echocardiography, we assessed indexed RV basal diameter (RVD), indexed RVOT diameter, and RV and left ventricular (LV) function. RV mechanical dispersion (RVMD), reflecting RV contraction heterogeneity, was assessed by speckle-tracking strain echocardiography. RV ejection fraction (RVEF) was assessed by cardiac magnetic resonance imaging (CMR). Patients with early-phase ARVC had lower %PVC by Holter and PVC more frequently originated from the RV lateral free wall (both P < 0.001). RVD was larger (21 ± 3 vs. 19 ± 2 mm, P < 0.01), RVMD was more pronounced (22 ± 15 vs. 15 ± 13 ms, P = 0.03), and RVEF by CMR was decreased (41 ± 8 vs. 49 ± 4%, P < 0.001) in early-phase ARVC vs. RVOT-VT patients.ConclusionPatients with early-phase ARVC had structural abnormalities with lower RVEF, increased RVD, and pronounced RVMD in addition to lower %PVC by Holter compared with RVOT-VT patients. These parameters can help correct diagnosis in patients with unclear phenotypes.

Management of asymptomatic arrhythmias: a European Heart Rhythm Association (EHRA) consensus document, endorsed by the Heart Failure Association (HFA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), Cardiac Arrhythmia Society of Southern Africa (CASSA), and Latin America Heart Rhythm Society (LAHRS)

“…The demonstration of PVCs of differing morphologies from the right ventricle (RV) in patients with normal LV function should prompt investigations to rule out arrhythmogenic cardiomyopathy with right ventricular dominance or sarcoidosis. 157 Similarly, multifocal PVCs of LV origin should trigger investigations for non-ischaemic cardiomyopathy.…”

Soluble ST2 is associated with disease severity in arrhythmogenic right ventricular cardiomyopathy

“…We assessed myocardial structure and function by cardiac imaging (echocardiography and cardiac magnetic resonance); conduction or rhythm disturbances by electrocardiograms (ECG), late potential ECGs and 24-hour ECGs; and family history and genetic testing as previously described (Saberniak et al 2017). A diagnosis of "definite" ARVC was made if the patient fulfilled at least two major criteria, one major criterion and two minor criteria, or four minor criteria covering different aspects of the disease.…”

“…Cine loops were digitally stored and later analysed off-line using Echo-Pac (GE Vingmed). Two-dimensional parameters and conventional Doppler measurements were obtained according to current recommendations (Lang et al 2015 (Saberniak et al 2017). Measurements of right ventricular function were obtained in focussed four-chamber views (Lang et al 2015).…”

Lower than expected burden of premature ventricular contractions impairs myocardial function

“…Cardiac magnetic resonance imaging (CMR) was performed in a subset of patients ( n = 22) as a part of the clinical evaluation and exclusion of ARVC, using a 1.5 Tesla unit (Magnetom Sonata, Vision Plus or Avanto Siemens, Erlangen, Germany) with a phased array body coil as previously described . LV and RV volumes and EFs were recorded, as well as the presence of late gadolinium enhancement or contraction abnormalities.…”