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Heart 2021 May 14. Epub 2021 May 14.

Cardiology, UMC Utrecht, Utrecht, The Netherlands

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by fibrofatty replacement of predominantly the right ventricle and high risk of ventricular arrhythmias and sudden cardiac death (SCD). Early diagnosis and accurate risk assessment are challenging yet essential for SCD prevention. This manuscript summarises the current state of the art on ARVC diagnosis and risk stratification. Read More

Curr Cardiol Rep 2021 05 7;23(6):56. Epub 2021 May 7.

Fondazione Toscana G.Monasterio, via Moruzzi 1, 56124, Pisa, Italy.

Purpose Of Review: Aim of the paper was to address all strengths and weakness of cardiac magnetic resonance (CMR) in arrhythmogenic cardiomyopathy, trying to highlight areas where further research and investigations should be carried out to fill current gaps in scientific knowledge.

Recent Findings: Arrhythmogenic cardiomyopathy represents a multifaceted clinical entity associated with arrhythmias and sudden death. Even though different diagnostic tools are available for appropriate identification and risk stratification, over the last few years cardiac magnetic resonance (CMR) has surfaced as an unmatched non-invasive imaging tool. Read More

Int J Mol Sci 2021 Apr 6;22(7). Epub 2021 Apr 6.

Erich and Hanna Klessmann Institute, Heart and Diabetes Center NRW, University Hospital of the Ruhr-University Bochum, Georgstrasse 11, 32545 Bad Oeynhausen, Germany.

About 50% of patients with arrhythmogenic cardiomyopathy (ACM) carry a pathogenic or likely pathogenic mutation in the desmosomal genes. However, there is a significant number of patients without positive familial anamnesis. Therefore, the molecular reasons for ACM in these patients are frequently unknown and a genetic contribution might be underestimated. Read More

Int J Cardiovasc Imaging 2021 Mar 24. Epub 2021 Mar 24.

Elettrofisiologia, U.O.C Di Cardiologia, Ospedale Civile Treviso, Treviso, Italy.

Three-dimensional (3D) reconstruction by means of electroanatomic mapping (EAM) systems, allows for the understanding of the mechanism of focal or re-entrant arrhythmic circuits along with pacing techniques. However, besides this conventional use, EAM may offer helpful anatomical and functional information. Data regarding electromechanical scar detection in ischaemic (and nonischaemic) cardiomyopathy are mostly consolidated, while emerging results are becoming available in contexts such as arrhythmogenic right ventricular dysplasia (ARVC/D) definition and Brugada syndrome. Read More

Am J Cardiol 2021 04 15;145:128-134. Epub 2021 Jan 15.

Division of Cardiology, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland. Electronic address:

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) classically initially present with ventricular arrhythmias or, less commonly, heart failure. Myocardial inflammation has been implicated in pathogenesis, but clinical myocarditis in ARVC is less described. We therefore studied clinical myocarditis as an initial ARVC presentation, and hypothesized that these patients have distinct clinical and genetic characteristics. Read More

Circ Genom Precis Med 2021 Feb 4;14(1):e003138. Epub 2020 Dec 4.

Cardiovascular Genetics Laboratory, Department of Medicine, Hatter Institute for Cardiovascular Research in Africa, University of Cape Town, South Africa (T.F.S., S.K., Z.B., N.A.B.N., G.S.).

J Am Heart Assoc 2020 12 21;9(23):e018866. Epub 2020 Nov 21.

Cardiovascular Division Department of Medicine University of Pennsylvania Philadelphia PA.

Arrhythmogenic right ventricular cardiomyopathy was first described as a right ventricular disease that is an important cause of death in young adults. However, with the advent of advanced imaging, arrhythmogenic right ventricular cardiomyopathy has been found to commonly have biventricular involvement, and a small portion of patients have left ventricular-dominant forms. On the other hand, a number of primarily left ventricular disease such as sarcoid and myocarditis can be arrhythmogenic and have right ventricular involvement. Read More

JACC Cardiovasc Imaging 2021 01 18;14(1):159-161. Epub 2020 Nov 18.

Clinical Research Domain, Baker Heart and Diabetes Institute, Melbourne, Australia; National Centre for Sports Cardiology, St. Vincent's Hospital Melbourne, Fitzroy, Australia. Electronic address:

Am J Emerg Med 2020 Nov 12. Epub 2020 Nov 12.

Dept of Emergency Medicine, Sarasota Memorial Hospital, 1540 S. Tamiami Trl. Suite 101, Sarasota, FL 34239, United States of America. Electronic address:

Wide complex tachycardias are rare in the pediatric population and may be due to ventricular tachycardia, aberrant conduction or antidromic tachycardia each with multiple underlying etiologies. We present a 14 yo female in extremis with syncope at rest witnessed by her mother, found in ventricular tachycardia by EMS who challenged with IVF hydration and amiodarone. Consecutive adequate fluid challenges and antiarrhythmics in the emergency department failed requiring synchronized cardioversion for stabilization. Read More

Int J Cardiol 2021 03 17;327:155. Epub 2020 Nov 17.

Cardiology and Cardiovascular Pathology, Department of Cardiac Thoracic and Vascular Science, University of Padova, Italy.

Circ Arrhythm Electrophysiol 2020 12 15;13(12):e009005. Epub 2020 Dec 15.

Heart Rhythm Center (M.C., A.G., R.S., V.C., M.B., G.V., C.T.), Centro Cardiologico Monzino IRCCS, Milano.

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce.

Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Read More

Eur Heart J 2021 03;42(11):1117

Department of Cardiology, Concord Hospital and The University of Sydney, Hospital Road, Concord NSW 2139, Australia.

Int J Cardiol 2021 01 8;322:175. Epub 2020 Oct 8.

First Department of Cardiology, National and Kapodistrian University of Athens, Hippokrateion Hospital, 114 Vasilissis Sofias avenue, Attica, Athens 11527, Greece.

Pulse (Basel) 2020 Aug 11;8(1-2):21-30. Epub 2020 Feb 11.

Laboratory of Medical Genetics, B.V. Petrovsky Russian Research Center of Surgery, Moscow, Russian Federation.

Introduction: Commonly accepted clinical classification of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still not developed.

Objective: To study the clinical forms of ARVC.

Methods: Fifty-four patients (38. Read More

JACC Clin Electrophysiol 2020 09;6(9):1086-1088

Section of Cardiac Electrophysiology, Sanger Heart and Vascular Institute, Concord, North Carolina, USA.

Int J Mol Sci 2020 Sep 10;21(18). Epub 2020 Sep 10.

Department of Cardiology, Royal Papworth Hospital, Cambridge CB2 0AY, UK.

Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterised by ventricular arrhythmia and an increased risk of sudden cardiac death (SCD). Numerous genetic determinants and phenotypic manifestations have been discovered in ACM, posing a significant clinical challenge. Further to this, wider evaluation of family members has revealed incomplete penetrance and variable expressivity in ACM, suggesting a complex genotype-phenotype relationship. Read More

Circ Cardiovasc Imaging 2020 09 2;13(9):e010243. Epub 2020 Sep 2.

Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.).

Mil Med 2020 12;185(11-12):e2173-e2175

Department of Critical Care Medicine, Walter Reed National Military Medical Center, 8901 Rockville Pike, Bethesda, MD 20889.

Identifying the cause of palpitations and syncope in the healthy, active duty military population is important. Most often, the causes are benign, but more malignant etiologies should not be overlooked. In this case, we present a 22-year-old active duty female soldier who developed exercise intolerance, palpitations, and ultimately one episode of exercise-induced syncope. Read More

World J Cardiol 2020 Jun;12(6):285-290

Department of Cardiology, Saint Vincent Hospital, Worcester, MA 01604, United States.

Background: Cardiac lipoma and lipomatous hypertrophy of interatrial septum (LHIS) are very rare disorders with distinct pathological features. While cardiac lipoma is a well-circumscribed encapsulated tumor of mature adipocytes, LHIS is due to entrapment of fat cells in the interatrial septum during embryogenesis. Although a biopsy is the definitive diagnostic test, these disorders can be differentiated by a cardiac magnetic resonance imaging (MRI). Read More

Med J Malaysia 2020 07;75(4):452-454

Hospital Sultanah Aminah, Department of General Surgery, Johor, Malaysia.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited cardiomyopathy characterised by right ventricular dysfunction, ventricular arrhythmias and increased risk of sudden cardiac death. Due to the replacement of myocardium with fibro-fatty and fibrous tissue, patients with ARVC are prone to develop ventricular tachycardia. Histologically, it is often reported as the 'triangle of dysplasia' involving the inflow tract, outflow tract and apex of the right ventricle. Read More

J Pathol Transl Med 2020 Sep 29;54(5):396-410. Epub 2020 Jul 29.

Department of Pathology, Sejong Hospital, Bucheon, Korea.

Background: The definitive pathologic diagnosis of cardiac sarcoidosis requires observation of a granuloma in the myocardial tissue. It is common, however, to receive a "negative" report for a clinically probable case. We would like to advise pathologists and clinicians on how to interpret "negative" biopsies. Read More

Minerva Med 2021 Apr 22;112(2):269-280. Epub 2020 Jul 22.

Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Padua, Italy -

Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease caused by mutations of desmosomal genes in about 50% of patients. Affected patients may have defective non-desmosomal genes. The ACM phenotype may occur in other genetic cardiomyopathies, cardio-cutaneous syndromes or neuromuscular disorders. Read More

Circ Cardiovasc Imaging 2020 07 14;13(7):e010164. Epub 2020 Jul 14.

Department of Cardiology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, China.

Int J Cardiol 2020 11 4;319:119. Epub 2020 Jul 4.

Harzklinikum Dorothea Christiane Erxleben GmbH, Cardiology, Germany. Electronic address:

ESC Heart Fail 2020 10 27;7(5):3174-3178. Epub 2020 Jun 27.

Department of Cardiovascular Medicine and Diagnostic Radiology, Faculty of Life Sciences, Graduate School of Medical Sciences, Kumamoto University, 1-1-1, Honjo, Chuo-ku, Kumamoto, 860-8556, Japan.

A 74-year-old man had abnormal left ventricular (LV) function according to a perioperative test at a local hospital and was transferred to our institution for further evaluation and treatment. His electrocardiogram demonstrated the presence of premature ventricular contraction with a QRS complex of the right bundle branch block type and superior axis. His echocardiography showed systolic dysfunction of the LV (LV ejection fraction, 44. Read More

Medicine (Baltimore) 2020 Jun;99(25):e20279

Department of Cardiology, The Second Xiangya Hospital, Central South University, Changsha, Hunan Province, China.

Rationale: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary cardiomyopathy disease discovered in 1994. Though there are advances in diagnosis of arrhythmogenic right ventricular cardiomyopathy, early diagnosis is still difficult especially when it is combined with other diseases with similar pathophysiologic changes, such as left to right shunt congenital heart disease (CHD). In this paper, we reported a case of CHD combined with ARVC. Read More

Int J Cardiol 2020 11 16;319:106-114. Epub 2020 Jun 16.

Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, Italy.

The original designation of "Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy"(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias. Subsequently, autopsy investigations, genotype-phenotype correlations studies and the increasing use of contrast-enhancement cardiac magnetic resonance showed that the fibro-fatty replacement of the myocardium represents the distinctive phenotypic feature of the disease that affects the myocardium of both ventricles, with left ventricular involvement which may parallel or exceed the severity of right ventricular involvement. This has led to the new designation of "Arrhythmogenic Cardiomyopathy" (ACM), that represents the evolution of the original term of ARVC. Read More

J Interv Cardiol 2020 29;2020:4386841. Epub 2020 May 29.

Teknon Medical Center, Barcelona, Spain.

Background: Voltage mapping allows identifying the arrhythmogenic substrate during scar-related ventricular arrhythmia (VA) ablation procedures. Slow conducting channels (SCCs), defined by the presence of electrogram (EGM) signals with delayed components (EGM-DC), are responsible for sustaining VAs and constitute potential ablation targets. However, voltage mapping, as it is currently performed, is time-consuming, requiring a manual analysis of all EGMs to detect SCCs, and its accuracy is limited by electric far-field. Read More

Int J Cardiol 2020 Oct 3;317:152-158. Epub 2020 Jun 3.

Center for Cardiological Innovation, Department of Cardiology, Oslo University Hospital, Rikshospitalet, Norway; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. Electronic address:

Aims: In Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), electrophysiological pathology has been claimed to precede morphological and functional pathology. Accordingly, an ECG without ARVC markers should be rare in ARVC patients with pathology identified by cardiac imaging. We quantified the prevalence of ARVC patients with evidence of structural disease, yet without ECG Task Force Criteria (TFC). Read More

J Am Coll Cardiol 2020 06;75(22):2753-2765

Cardio-thoraco-vascular Department, University of Trieste, Trieste, Italy.

Background: Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered. Read More