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Circ Cardiovasc Imaging 2019 Apr;12(4):e009084

Division of Cardiology, Cardiothoracovascular Department, Azienda Sanitaria Universitaria Integrata of Trieste, Italy.

Neth Heart J 2019 Apr 17. Epub 2019 Apr 17.

Durrer Centre for Cardiovascular Research, Netherlands Heart Institute, Utrecht, The Netherlands.

Background: Clinical research on arrhythmogenic cardiomyopathy (ACM) is typically limited by small patient numbers, retrospective study designs, and inconsistent definitions.

Aim: To create a large national ACM patient cohort with a vast amount of uniformly collected high-quality data that is readily available for future research.

Methods: This is a multicentre, longitudinal, observational cohort study that includes (1) patients with a definite ACM diagnosis, (2) at-risk relatives of ACM patients, and (3) ACM-associated mutation carriers. Read More

Clin Case Rep 2019 Apr 19;7(4):686-688. Epub 2019 Feb 19.

Department of Electrophysiology, Heart Center University of Leipzig Leipzig Germany.

This case emphasizes the value of cardiac MRI and genetic testing in the early phase of ARVD/C. It also emphasizes the increased risk of SCD for patients with ARVD/C participating in competitive sports, even with immediate cardiopulmonary resuscitation. Read More

Ann Cardiol Angeiol (Paris) 2019 Jan 22. Epub 2019 Jan 22.

Centre de médecine et des sciences de sport, Tunis, Tunisia.

Introduction: Premature ventricular complexes (PVC) are generally considered as a benign electrocardiographic abnormality in the athletic population. However it may be indicative of underlying heart disease which may increase the risk of sudden death. This implies the need for cardiological evaluation before indicating the ability to practice competitive sports. Read More

Arq Bras Cardiol 2019 Jan;112(1):91-103

'Unité de Rythmologie de l'Institut de Cardiologie de l'Hôpital Pitié-Salpêtrière, Paris - France.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) was initially recognized as a clinical entity by Fontaine and Marcus, who evaluated a group of patients with ventricular tachyarrhythmia from a structurally impaired right ventricle (RV). Since then, there have been significant advances in the understanding of the pathophysiology, manifestation and clinical progression, and prognosis of the pathology. The identification of genetic mutations impairing cardiac desmosomes led to the inclusion of this entity in the classification of cardiomyopathies. Read More

Sci Rep 2019 Jan 14;9(1):100. Epub 2019 Jan 14.

University Heart Center, Department of Cardiology, Zurich, 8091, Switzerland.

The rationale of this paper is to investigate right ventricular (RV) hemodynamics in relation to changes in cardiac output, and in particular to study exercise-induced stresses at the RV outflow tract (RVOT), which is a common site of ventricular arrhythmias in the athlete's heart. We hypothesize that the thin-walled RVOT is exposed to high wall shear stresses (WSS) during physiological states associated with high cardiac output such as exercise, and therefore, may be particularly prone to substrate formation leading to ventricular tachyarrhythmias. 3D Particle Tracking Velocimetry (3D-PTV), an optical imaging method, has been performed in a novel anatomically accurate compliant silicone right heart model derived from a high resolution MRI heart scan of a healthy male proband. Read More

Heart Lung Circ 2019 Jan 24;28(1):164-177. Epub 2018 Oct 24.

Department of Cardiology, Westmead Applied Research Centre, Westmead Hospital, University of Sydney, Sydney, NSW, Australia. Electronic address:

Arrhythmogenic cardiomyopathy (ACM) is now commonly used to describe any form of non-hypertrophic, progressive cardiomyopathy characterised by fibrofatty infiltration of the ventricular myocardium. Right ventricular (RV) involvement refers to the classical arrhythmogenic right ventricular cardiomyopathy, but left ventricular, or bi-ventricular involvement are now recognised. ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Read More

Int J Cardiovasc Imaging 2019 Mar 31;35(3):529-538. Epub 2018 Oct 31.

Heart and Vascular Center, Semmelweis University, Budapest, Hungary.

Both, arrhythmogenic right ventricular cardiomyopathy (ARVC) and regular training are associated with right ventricular (RV) remodelling. Cardiac magnetic resonance (CMR) is given an important role in the diagnosis of ARVC in current task force criteria (TFC), however, they contain no cut-off values for athletes. We aimed to confirm the added value of feature tracking and to provide new cut-off values to differentiate between ARVC and athlete's heart. Read More

Circ Cardiovasc Imaging 2018 Sep;11(9):e008271

The Labatt Family Heart Centre, Department of Paediatrics (M.d.C.V.A., L.G.-W.), The Hospital for Sick Children, University of Toronto, Ontario, Canada.

Circ Cardiovasc Imaging 2018 Oct;11(10):e008204

Istituto Auxologico Italiano, IRCCS, Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital, Milan, Italy (C.T., G.B.P., G.P.).

Pediatr Int 2018 Oct;60(10):987-989

Department of Pediatric Cardiology and Pediatric Dermatology, Marmara University Faculty of Medicine, Istanbul, Turkey.

Asian Cardiovasc Thorac Ann 2018 Sep 9;26(7):563-565. Epub 2016 Oct 9.

3 Department of Pediatric Cardiology, Fortis Hospital, Jaipur, India.

A 17-year-old boy presented with facial puffiness and swelling in the lower limbs for 6 months and one episode of syncope 15 days earlier. Transthoracic echocardiography showed a dilated right atrium and right ventricle with right ventricular systolic dysfunction. The free wall of the right ventricle was thinned out and devoid of myocardium and trabeculations. Read More

Med Sci (Basel) 2018 Sep 19;6(3). Epub 2018 Sep 19.

Department of Radiology, Tehran University of Medical Sciences, Tehran 1995614331, Iran.

Arrhythmogenic right ventricular dysplasia (ARVD) is an abnormality in the right side of the heart that may lead to sudden death. The study aims to compare cardiac MRI (magnetic resonance imaging findings) with echocardiography in patients with ARVD. For the cross-sectional study, patients with ARVD that were diagnosed using Task Force criteria were included, and their cardiac MRI findings were evaluated. Read More

J Vet Cardiol 2018 Oct 20;20(5):343-353. Epub 2018 Aug 20.

Department of Quantitative Health Sciences, University of Massachusetts Medical School, Worcester, MA, USA, 01605.

Objectives: To determine whether there are differences in measures of longitudinal right ventricular (RV) systolic function among Boxers with arrhythmogenic right ventricular cardiomyopathy (ARVC) compared with healthy control Boxer dogs. To explore relationships between markers of RV systolic function and age, body weight, gender, arrhythmia frequency, and markers of left ventricular (LV) systolic function in Boxer dogs.

Animals: The study included 50 client-owned Boxer dogs. Read More

J Am Coll Cardiol 2018 Aug;72(7):784-804

APHP, Pitié-Salpêtrière University Hospital, Institute of Cardiology, Paris, France; Centre de Référence des Maladies Cardiaques Héréditaires, Paris, France; Institute of Cardiometabolism and Nutrition (ICAN), Paris, France.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiomyopathy that can lead to sudden cardiac death and heart failure. Our understanding of its pathophysiology and clinical expressivity is continuously evolving. The diagnosis of ARVC/D remains particularly challenging due to the absence of specific unique diagnostic criteria, its variable expressivity, and incomplete penetrance. Read More

J Nucl Cardiol 2018 Jul 26. Epub 2018 Jul 26.

Department of Cardiology, Royal Melbourne Hospital, Parkville, Australia.

Indian Heart J 2018 May - Jun;70(3):421-426. Epub 2017 Oct 20.

Department of Cardiology, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, J&K, India.

Objective: Arrhythmogenic cardiomyopathy (ACM) is not an uncommon cause of cardiac morbidity in Kashmir valley. This study was designed to document various clinical features and to sequence exons 11 and 12 of plakophilin 2 (PKP2) gene in these patients.

Methods: ACM patients who attended cardiology outpatient department of our institute from January 2014 to April 2015 were included in the study. Read More

Int J Cardiol 2018 Nov 31;271:81-86. Epub 2018 May 31.

Hôpital Universitaire Necker Enfants Malades, Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes, M3C, 149, rue de Sèvres, 75743 Paris Cedex 15, France; Hôpital Universitaire Necker Enfants Malades, Service de Radiologie pédiatrique, 149, rue de Sèvres, 75743 Paris Cedex 15, France.

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. Read More

Int J Cardiol 2018 Nov 20;271:75-80. Epub 2018 May 20.

Barts Heart Centre, Barts Health NHS Trust, West Smithfield, London EC1A 7BE, UK; Institute of Cardiovascular Science, University College London, Gower Street, London WC1E 6BT, UK. Electronic address:

Background: The re-entry vulnerability index (RVI) is a recently proposed activation-repolarization metric designed to quantify tissue susceptibility to re-entry. This study aimed to test feasibility of an RVI-based algorithm to predict the earliest endocardial activation site of ventricular tachycardia (VT) during electrophysiological studies and occurrence of haemodynamically significant ventricular arrhythmias in follow-up.

Methods: Patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) (n = 11), Brugada Syndrome (BrS) (n = 13) and focal RV outflow tract VT (n = 9) underwent programmed stimulation with unipolar electrograms recorded from a non-contact array in the RV. Read More

J Interv Card Electrophysiol 2018 Aug 26;52(3):307-313. Epub 2018 May 26.

Cardiology Division, Policlinico Casilino, Via Casilina 1049, 00169, Rome, Italy.

Right bundle branch block is relatively frequent in the general population and is generally considered benign in healthy individuals. However, it has been associated with increased cardiovascular risk and all-cause mortality in various cohort studies. Indeed, the presence of right bundle branch block in subjects affected by cardiomyopathies or channelopathies is not rare and may conceal underlying electrocardiographic changes, thus making the diagnosis of such conditions more challenging. Read More

Card Electrophysiol Clin 2018 06;10(2):413-429

Multimodality Cardiac Imaging Section, Policlinico San Donato, San Donato Milanese, Piazza Edmondo Malan, 2, 20097 San Donato Milanese MI, Italy.

Cardiovascular imaging has radically changed the management of patients with arrhythmogenic cardiomyopathies. This article focuses on the role of echocardiography and MRI in the diagnosis of these structural diseases. Cardiomyopathies with hypertrophic pattern (hypertrophic cardiomyopathy, restrictive cardiomyopathies, amyloidosis, Anderson-Fabry disease, and sarcoidosis), cardiomyopathies with dilated pattern, inflammatory cardiac diseases, and right ventricular arrhythmogenic cardiomyopathy are analyzed. Read More

Pediatr Cardiol 2018 Aug 12;39(6):1156-1164. Epub 2018 May 12.

Department of Diagnostic and Interventional Radiology, University Medical Center, Georg-August-University, Goettingen, Germany.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a potentially lethal disease that is well described in adults. In pediatric patients, however, identification of patients at risk of adverse events of ARVC remains a challenge. We aimed to determine which criteria of the revised Task Force Criteria (rTFC), alone or combined, have an impact on diagnosis of ARVC when compared to disease-specific genetic mutations in pediatric patients ≤ 18 years. Read More

Heart Lung Circ 2018 Nov 4;27(11):1310-1317. Epub 2018 Apr 4.

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia. Electronic address:

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that predominantly affects the right ventricle. With a prevalence in the range of 1:5000 to 1:2000 persons, ARVC is one of the leading causes of sudden cardiac death in young people and in athletes. Although early detection and treatment is important, the diagnosis of ARVC remains challenging. Read More

Clin Cardiol 2018 Mar 25;41(3):300-306. Epub 2018 Mar 25.

Service Rythmologie, Hôpital Cardiologique Louis Pradel, Hospices Civils de Lyon, Lyon, France.

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare cardiomyopathy characterized by the progressive replacement of cardiomyocytes by fatty and fibrous tissue in the right ventricle (RV). These infiltrations lead to cardiac electrical instability and ventricular arrhythmia. Current treatment for ARVD is empirical and essentially based on treatment of arrhythmia. Read More

Europace 2018 06;20(FI1):f46-f56

First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.

Aims: Our aim is to investigate the arrhythmogenic mechanism in arrhythmogenic right ventricular cardiomyopathy (ARVC)-patients by using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs).

Methods And Results: Human-induced pluripotent stem cell-derived cardiomyocytes were generated from human skin fibroblasts of two healthy donors and an ARVC-patient with a desmoglein-2 (DSG2) mutation. Patch clamp, quantitative polymerase chain reaction, and calcium imaging techniques were employed for the study. Read More

Arq Bras Cardiol 2018 Feb;110(2):201-202

Clinica Obstétrica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, SP - Brazil.

Int J Cardiol 2018 05;258:172-178

Sorbonne Universités, UPMC Univ Paris 06 Faculté de Médecine, Paris, France; Service de Gynécologie Obstétrique, APHP, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France.

Introduction: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children.

Methods: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Read More

Cardiovasc Pathol 2018 May - Jun;34:15-21. Epub 2018 Feb 14.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167A Beilishi Road, Xi Cheng District, Beijing, 100037, P.R. China. Electronic address:

Arrhythmogenic right ventricular cardiomyopathy (ARVC) and dilated cardiomyopathy (DCM), despite being two dramatically different entities, have overlapping phenotypes. As it is easy to misdiagnose between ARVC and DCM, there is a need to establish a new differential diagnostic parameter to differentiate the two. We investigated the utility of endomyocardial biopsy (EMB) for the differential diagnosis, and our study had three aims. Read More

Heart Fail Clin 2018 Apr;14(2):201-213

Department of Cardiac, Thoracic, and Vascular Sciences, University of Padova Medical School, Padova, Italy.

Arrhythmogenic cardiomyopathy (AC) is an inherited heart muscle disease characterized by myocardial atrophy and fibrofatty replacement of the ventricular myocardium, at risk of sudden cardiac death, particularly in the young and athletes. Because there is no "gold standard" to reach the diagnosis of AC, multiple categories of diagnostic information have been combined, including imaging, electrocardiographic changes, arrhythmias, tissue characterization, and family history. However, the routine use of contrast-enhanced cardiac magnetic resonance increasingly revealed left dominant AC, a variant that is not well addressed in the diagnostic criteria and still escapes clinical identification. Read More

Int J Cardiol 2018 04;257:371-377

Heart Department, University Hospital, Salerno, Italy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the most common phenotype described within the spectrum of arrhythmogenic cardiomyopathies. It usually presents in early adolescence with severe ventricular arrhythmias along with cardiac structural and functional alterations mainly of the right ventricular myocardium. Though the estimated prevalence of ARVC in the general population is only 1:5000, it represents one of the most common causes of juvenile sudden death. Read More

Biomed Res Int 2018 9;2018:5610347. Epub 2018 Jan 9.

Department of Radiological, Oncological and Anatomo-Pathological Sciences, Policlinico Umberto I, Sapienza University of Rome, Viale Regina Elena 324, 00161 Rome, Italy.

Ectopic cardiac fatty images are not rarely detected incidentally by computed tomography and cardiac magnetic resonance, or by exams focused on the heart as in general thoracic imaging evaluations. A correct interpretation of these findings is essential in order to recognize their normal or pathological meaning, focusing on the eventually associated clinical implications. The development of techniques such as computed tomography and cardiac magnetic resonance allowed a detailed detection and evaluation of adipose tissue within the heart. Read More

Heart Rhythm 2018 07 2;15(7):987-993. Epub 2018 Mar 2.

Section of Cardiac Electrophysiology, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. Electronic address:

Background: Criteria for identification of anatomic ventricular tachycardia substrates in arrhythmogenic right ventricular cardiomyopathy (ARVC) on late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) are unclear.

Objective: The purpose of this study was to define (1) the association of regional right ventricular (RV) epicardial voltage amplitude with the distribution of LGE; and (2) appropriate image signal intensity (SI) thresholds for ventricular tachycardia substrate identification in ARVC.

Methods: Preprocedural LGE-CMR and epicardial electrogram mapping were performed in 10 ARVC patients. Read More

Oxf Med Case Reports 2018 Jan 25;2018(1):omx088. Epub 2018 Jan 25.

Cardiology-Rehabilitation, 5th Department of Internal Medicine, 'Iuliu Hatieganu' University of Medicine and Pharmacy, Cluj-Napoca, Romania.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a genetic disorder characterized by fibrofattty replacement of the right ventricular myocardium. In the revised 2010 Task Force Criteria, a major criteria for ARVD/C is the presence of RV aneurysm by 2D echo. Our report demonstrates that intracardiac ultrasound can detect RV aneurysms and also focal absence of trabeculations which brings additional value to the diagnosis of ARVD/C. Read More

J Interv Card Electrophysiol 2018 Jan 5;51(1):25-33. Epub 2018 Jan 5.

Department of Cardiology, Electrophysiology, Erasmus Medical Center, Rotterdam, The Netherlands.

Purpose: Coupling interval (CI) variability of premature ventricular contractions (PVCs) is influenced by the underlying arrhythmia mechanism. The aim of this study was to compare CI variability of PVCs in different myocardial disease entities, in order to gain insight into their arrhythmia mechanism.

Methods: Sixty-four patients with four underlying pathologies were included: idiopathic (n = 16), non-ischemic dilated cardiomyopathy (NIDCM) (n = 16), familial cardiomyopathy (PLN/LMNA) (n = 16), and post-MI (n = 16)-associated PVCs. Read More

Scand Cardiovasc J 2018 Feb 19;52(1):13-19. Epub 2017 Dec 19.

a Institute of Emergency for Cardiovascular Diseases "Prof.dr.C.C.Iliescu" , University of Medicine and Pharmacy "Carol Davila" Bucharest , Bucharest , Romania.

Objective: Changes in right ventricular (RV) structure and function following prolonged endurance training in athletes arise due to its unique anatomy and physiology. Arrhythmogenic cardiomyopathy (AC) should be differentiated from electrical, functional and structural adaptation of the heart in response to repetitive intense physical activity due to the negative contribution of exercise on AC progression and arrhythmic risk.

Design: For this review we performed a systematic search of the PubMed database up to October 2017 using terms and keywords pertaining to RV, athlete's heart (AH), AC, sudden cardiac death. Read More

Intern Med 2018 Mar 8;57(6):835-839. Epub 2017 Dec 8.

Division of Cardiovascular Medicine, Department of Medicine, Jichi Medical University School of Medicine, Japan.

The diagnosis of cardiac sarcoidosis (CS) has become easier due to advances in imaging modalities, but we sometimes encounter difficult-to-diagnose patients. We herein report the case of a 60-year-old Japanese woman who was diagnosed with isolated CS, although she also met the diagnostic criteria of arrhythmogenic right ventricular cardiomyopathy (ARVC). A histological examination by an endomyocardial biopsy of the right ventricle revealed the typical findings of granulomatous change for CS. Read More

BMC Cardiovasc Disord 2017 Dec 6;17(1):286. Epub 2017 Dec 6.

Department of Internal medicine, Faculty of Medicine, "J.J. Strossmayer" University of Osijek, Osijek, Croatia.

Background: There are still ambiguities existing in regard to left ventricular non-compaction (LVNC) diagnostic imaging. The aim of our study was to analyze diagnostic potential of late gadolinium enhancement (LGE) and ventricle geometry in patients with LVNC and controls.

Methods: Data on cardiac magnetic resonance imaging (CMR) studies for LVNC were reassessed from the hospital's database (3. Read More

Clin Physiol Funct Imaging 2018 Sep 6;38(5):779-787. Epub 2017 Nov 6.

Department of Clinical Physiology and Department of Medical and Health Sciences, Linköpings Universitet, Linköping, Sweden.

Purpose: To assess right ventricular (RV) regional and global systolic function using feature tracking (FT) in patients with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) and to investigate if changes in strain amplitude and mechanical dispersion indicate a propensity for arrhythmia.

Materials And Methods: Twenty-seven patients fulfilling Task Force Criteria for ARVC and 24 healthy volunteers underwent MR at 1·5 Tesla. Steady-state free precession cine of long-axis slices and a short-axis stack of the RV was acquired. Read More

Eur Heart J Cardiovasc Imaging 2018 01;19(1):120

AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Unité de Rythmologie, Institut de cardiologie, Hôpital Pitié-Salpetrière, Paris, France.

Glob Cardiol Sci Pract 2016 Dec 30;2016(4):e201639. Epub 2016 Dec 30.

Department of Cardiology, University of Bristol, Bristol, UK.

Arrhythmogenic right ventricular dysplasia (ARVD) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD). The natural history is predominantly related to ventricular electric instability which may lead to arrhythmic SCD, mostly in young people and athletes, but may progress with significant RV muscle disease and left-ventricular (LV) involvement and can result in right or biventricular heart failure. We report on a 54-year-old male with ARVD who underwent an epicardial ventricular tachycardia (VT) ablation using remote magnetic navigation (RMN) after functional imaging from a nuclear perfusion study was fused with a 3D segmentation from computed tomography (CT) imaging. Read More

Cardiovasc Res 2017 Oct;113(12):1499-1508

Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.

Exercise is associated with unequivocal health benefits and results in many structural and functional changes of the myocardium that enhance performance and prevent heart failure. However, intense exercise also presents a significant hemodynamic challenge in which the right-sided heart chambers are exposed to a disproportionate increase in afterload and wall stress that can manifest as myocardial fatigue or even damage if intense exercise is sustained for prolonged periods. This review focuses on the physiological factors that result in a disproportionate load on the right ventricle during exercise and the long-term consequences. Read More

Cardiovasc Res 2017 Oct;113(12):1486-1498

CARIM School for Cardiovascular Diseases, Maastricht University Medical Center, Maastricht, The Netherlands.

Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. Read More

Circ Res 2017 Sep;121(7):784-802

From the Department of Cardiac, Thoracic, and Vascular Sciences, University of Padova Medical School, Italy (D.C., C.B.); and Department of Medicine/Cardiology, Center for Inherited Heart Disease, Johns Hopkins University School of Medicine, Baltimore, MD (D.P.J.).

Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abnormalities of cardiac desmosomes, which leads to detachment of myocytes and alteration of intracellular signal transduction. Read More

J Cardiovasc Comput Tomogr 2017 Nov 5;11(6):497-498. Epub 2017 Sep 5.

Department of Radiology, Faculty of Medicine, Chulalongkorn University, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

Tex Heart Inst J 2017 Aug 1;44(4):290-293. Epub 2017 Aug 1.

Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle. Read More

Circ Heart Fail 2017 Sep;10(9)

From the Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD (N.A.G., A.B., C.A.J., B.M., C.T., C.S.O., D.P.J., S.D.R., H.C., R.J.T.); Division of Cardiology, University Medical Center Utrecht, The Netherlands (A.S.J.t.R.); and Division of Cardiology, Department of Medicine, University at Buffalo, State University of New York (A.S.).

Background: Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D.

Methods And Results: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. Read More

J Cardiovasc Magn Reson 2017 Sep 1;19(1):66. Epub 2017 Sep 1.

Department of Medicine, Division of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.

Background: Regional right ventricular (RV) dysfunction is the hallmark of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), but is currently only qualitatively evaluated in the clinical setting. Feature Tracking Cardiovascular Magnetic Resonance (FT-CMR) is a novel quantitative method that uses cine CMR to calculate strain values. However, most prior FT-CMR studies in ARVD/C have focused on global RV strain using different software methods, complicating implementation of FT-CMR in clinical practice. Read More

J Cardiovasc Med (Hagerstown) 2017 10;18(10):796-797

aDepartment of Internal Medicine, Division of Cardiology, University of Rome 'Tor Vergata' bDepartment of Cardiology, Policlinico Casilino, Rome, Italy.

Circ J 2017 12 30;82(1):78-86. Epub 2017 Aug 30.

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center.

Background: Risk stratification of ventricular arrhythmias is vital to the optimal management in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We hypothesized that 64-channel magnetocardiography (MCG) would be useful to detect isolated late activation (ILA) by overcoming the limitations of conventional noninvasive predictors of ventricular tachyarrhythmias, including epsilon waves, late potential (LP), and right ventricular ejection fraction (RVEF), in ARVC patients.Methods and Results:We evaluated ILA on MCG, defined as discrete activations re-emerging after the decay of main RV activation (%magnitude >5%), and conventional noninvasive predictors of ventricular tachyarrhythmias (epsilon waves, LP, and RVEF) in 40 patients with ARVC. Read More

World J Pediatr Congenit Heart Surg 2017 Jan 1:2150135117707459. Epub 2017 Jan 1.

1 Department of Cardiac Surgery, Madras Medical Mission, Chennai, India.

A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Read More