2020
Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy
Smith ED, Lakdawala NK, Papoutsidakis N, Aubert G, Mazzanti A, McCanta AC, Agarwal PP, Arscott P, Dellefave-Castillo LM, Vorovich EE, Nutakki K, Wilsbacher LD, Priori SG, Jacoby DL, McNally EM, Helms AS. Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation 2020, 141: 1872-1884. PMID: 32372669, PMCID: PMC7286080, DOI: 10.1161/circulationaha.119.044934.Peer-Reviewed Original ResearchConceptsLV late gadolinium enhancementLate gadolinium enhancementSevere ventricular arrhythmiasAcute myocardial injuryMyocardial injuryRight ventricular cardiomyopathyVentricular arrhythmiasGadolinium enhancementVentricular cardiomyopathyEjection fractionSevere arrhythmiasFrequent premature ventricular contractionsPathogenic plakophilin-2 mutationNormal LV systolic functionLeft ventricle ejection fractionRight ventricular ejection fractionPositron emission tomography scanArrhythmogenic right ventricular cardiomyopathyNormal ventricular functionLV systolic functionVentricle ejection fractionVentricular ejection fractionEmission tomography scanSmall case seriesPlakophilin-2 mutations
2019
Risk score for the exclusion of arrhythmic events in arrhythmogenic right ventricular cardiomyopathy at first presentation
Vischer AS, Castelletti S, Syrris P, Bastiaenen R, Miles C, Akdis D, Denhaerynck K, Jacoby D, Saguner AM, Krahn AD, Behr ER, McKenna WJ, Pantazis A. Risk score for the exclusion of arrhythmic events in arrhythmogenic right ventricular cardiomyopathy at first presentation. International Journal Of Cardiology 2019, 290: 100-105. PMID: 31104822, DOI: 10.1016/j.ijcard.2019.04.090.Peer-Reviewed Original ResearchConceptsArrhythmogenic right ventricular cardiomyopathyNon-sustained VTNegative T wavesRight ventricular cardiomyopathyVentricular arrhythmiasRisk scoreArrhythmic eventsFiltered QRS durationFirst presentationH ECGQRS durationVentricular cardiomyopathyLead aVRARVC Task Force CriteriaApplicable risk scoreCohort of patientsSustained ventricular arrhythmiasMultivariable logistic regressionTask Force criteriaDetailed clinical dataLife-threatening arrhythmiasHeart muscle disorderT waveNegative predictive valueSignal-averaged ECGPrediction of ventricular arrhythmia and sudden death in arrhythmogenic right ventricular cardiomyopathy
McKenna WJ, Asaad NA, Jacoby DL. Prediction of ventricular arrhythmia and sudden death in arrhythmogenic right ventricular cardiomyopathy. European Heart Journal 2019, 40: 1859-1861. PMID: 30977786, DOI: 10.1093/eurheartj/ehz195.Commentaries, Editorials and LettersConceptsArrhythmogenic right ventricular cardiomyopathyRight ventricular cardiomyopathyVentricular arrhythmiasVentricular cardiomyopathySudden deathCardiomyopathyArrhythmias