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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

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

Medicine (Baltimore) 2020 Apr;99(15):e19817

5th Department of Internal Medicine, Cardiology-Rehabilitation.

Rationale: Two-dimensional echocardiography (2D echo) is a major tool for the diagnosis of Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However 2D echo can skip regional localized anomalies of the right ventricular wall. We aimed to determine whether transesophageal and intracardiac ultrasound can provide additional information, on the right ventricular abnormalities compared to 2D echo. Read More

Heart 2020 Apr;106(8):602-623

Department of Cardiology, University Hospital of Wales, Cardiff, UK.

Heart Lung Circ 2020 Apr 24;29(4):584-593. Epub 2019 Dec 24.

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

Imaging modalities are central to diagnosis and prognostication of confirmed or suspected inherited cardiomyopathies. The availability and use of cardiovascular magnetic resonance imaging (CMR) to supplement traditional modalities has increased substantially and has several advantages over traditional imaging techniques. CMR is unique in its ability to easily acquire images in any plane. Read More

Cardiol Rev 2020 Feb 3. Epub 2020 Feb 3.

From the Department of Medicine, Cardiology Division, New York Medical College/ Westchester Medical Center, Valhalla, NY.

Arrhythmogenic right ventricular cardiomyopathy (ARVC), formerly called 'arrhythmogenic right ventricular dysplasia', is an under-recognized clinical entity characterized by ventricular arrhythmias and a characteristic ventricular pathology. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore, consensus diagnostic criteria have been developed which combine electrocardiographic, echocardiographic, cardiac magnetic resonance imaging and histologic criteria. Read More

BMC Med Genet 2020 01 31;21(1):19. Epub 2020 Jan 31.

Blueprint Genetics, Helsinki, Finland.

Background: Dilated cardiomyopathy (DCM) is a condition characterized by dilatation and systolic dysfunction of the left ventricle in the absence of severe coronary artery disease or abnormal loading conditions. Mutations in the titin (TTN) and lamin A/C (LMNA) genes are the two most significant contributors in familial DCM. Previously mutations in the desmoplakin (DSP) gene have been associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) and more recently with DCM. Read More

Europace 2020 May;22(5):797-805

Cardiology and Arrhythmology Clinic, Marche Polytechic University, University Hospital "Ospedali Riuniti", Ancona (AN), Italy.

Aims: To provide long-term outcome data on arrhythmogenic cardiomyopathy (ACM) patients with non-classical forms [left dominant ACM (LD-ACM) and biventricular ACM (Bi-ACM)] and an external validation of a recently proposed algorithm for ventricular arrhythmia (VA) prediction in ACM patients.

Methods And Results: Demographic, clinical, and outcome data were retrieved from all ACM patients encountered at our institution. Patients were classified according to disease phenotype (R-ACM; Bi-ACM; LD-ACM). Read More

Neurol India 2019 Nov-Dec;67(6):1528-1531

Department of Cardiology, Amrita Institute of Medical Sciences, Kochi, Kerala, India.

Arrhythmogenic right ventricular dysplasia (ARVD) is an underdiagnosed cardiomyopathy which commonly presents in young adults with ventricular tachycardia or sudden cardiac death. We report a case of ARVD presenting with features of acute ischemic cerebrovascular stroke. The suspicion of ARVD came only when the echocardiogram revealed dilatation and abnormal wall motion of the right ventricle in the presence of certain ECG findings consistent with ARVD. Read More

Am J Cardiol 2020 02 19;125(4):613-617. Epub 2019 Nov 19.

Fuwai Hospital, National Center for Cardiovascular Diseases, Beijing, China; Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address:

Less is known about pregnancy in women with arrhythmogenic right ventricular cardiomyopathy (ARVC). From April 1995 to May 2018, 157 women with ARVC were retrospectively enrolled. Data on pregnancy and cardiac outcomes were analyzed. Read More

J Cardiovasc Magn Reson 2019 12 12;21(1):76. Epub 2019 Dec 12.

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

Background: The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. Read More

Eur Heart J Cardiovasc Imaging 2020 Apr;21(4):378-386

Cardiology Department, Hospital Universitario Virgen de las Nieves, Avda. De las Fuerzas Armadas 2, 18014 Granada, Spain.

Aims: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a life-threatening entity with a highly heterogeneous genetic background. Cardiac magnetic resonance (CMR) imaging can identify fibrofatty scar by late gadolinium enhancement (LGE). Our aim is to investigate genotype-phenotype correlation in ARVC/D mutation carriers, focusing on CMR-LGE and myocardial fibrosis patterns. Read More

Fetal Pediatr Pathol 2019 Oct 18:1-12. Epub 2019 Oct 18.

Department of Pathology and Pediatrics, Children's Hospital of Michigan Foundation , Detroit , Michigan , USA.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited, progressive form of cardiomyopathy, which is characterized by fibrofatty replacement of the myocardium. While the gold standard for diagnosis remains pathologic evaluation of biopsy, advances in noninvasive imaging, including cardiac magnetic resonance imaging (CMRI), have led to improved clinical diagnosis. We report three additional cases of pediatric patients that have pathologically confirmed ARVC/D with CMRI images, demonstrating extensive macroscopic fatty infiltration of the right and left ventricular myocardium. Read More

Rofo 2020 Jan 9;192(1):27-37. Epub 2019 Oct 9.

Department of Radiology, University Medical Center Göttingen, Göttingen, Germany.

Backround:  Structured reports have numerous benefits through standardizing the way imaging findings are reported and communicated. Nevertheless, the adoption of structured reports in everyday radiological practice is still limited. In view of the irrefutable benefits, various national and international radiological societies have started initiatives which aim at promoting a broader use of structured reports. Read More

J Am Heart Assoc 2019 09 23;8(17):e012989. Epub 2019 Aug 23.

Department of CMR State Key Laboratory of Cardiovascular Disease Fuwai Hospital National Center for Cardiovascular Diseases Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China.

Background Left ventricular (LV) involvement is common in arrhythmogenic right ventricular cardiomyopathy (ARVC). We aim to evaluate LV involvement in ARVC patients by cardiovascular magnetic resonance feature tracking. Methods and Results Sixty-eight patients with ARVC and 30 controls were prospectively enrolled. Read More

Anatol J Cardiol 2019 Aug;22(2):60-67

Departments of Pediatric Cardiology,Sağlık Bilimleri University, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center; İstanbul-Turkey.

Objective: Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD. Read More

Circulation 2019 09 18;140(12):1015-1030. Epub 2019 Jul 18.

The Leon H. Charney Division of Cardiology (J.C.K., M.P.H., M.Z., X.L., C.V., M.C., M.D.), New York University School of Medicine.

Background: Plakophilin-2 (PKP2) is classically defined as a desmosomal protein. Mutations in PKP2 associate with most cases of gene-positive arrhythmogenic right ventricular cardiomyopathy. A better understanding of PKP2 cardiac biology can help elucidate the mechanisms underlying arrhythmic and cardiomyopathic events consequent to PKP2 deficiency. Read More

Circ Cardiovasc Imaging 2019 07 24;12(7):e009272. Epub 2019 Jun 24.

Cardiovascular Division, Department of Medicine, University of Minnesota Medical School, Minneapolis (C.S.).

Thromb Haemost 2019 Aug 10;119(8):1373-1378. Epub 2019 Jun 10.

University Heart Center Zurich, University Hospital of Zurich, University of Zurich, Zurich, Switzerland.

Background:  Thrombus formation within the left ventricle (LV) is a well-known clinical entity and is often associated with underlying myocardial disease, whereas right ventricular (RV) thrombi are rarely observed. This study aimed to investigate the clinical characteristics of patients with arrhythmogenic RV cardiomyopathy (ARVC) who developed an RV thrombus.

Methods And Results:  This study included patients with an RV thrombus from the ARVC databases of the University Heart Center in Zurich, Switzerland, and the Fuwai Hospital in Beijing, China. Read More

Int J Cardiol 2019 09 1;290:100-105. Epub 2019 May 1.

Cardiomyopathy Service, Royal Brompton Hospital, London, UK.

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disorder associated with an increased risk of life-threatening arrhythmias in some patients. Risk stratification remains challenging. Therefore, we sought a non-invasive, easily applicable risk score to predict sustained ventricular arrhythmias in these patients. Read More

Circ Cardiovasc Imaging 2019 04;12(4):e007693

Department of Pediatrics, Labatt Family Heart Centre (G.E.P., L.G.-W., M.H., M.F., P.C., C.S., W.H., C.-P.S.F., C.M., L.M., R.H., M.K.F.), Hospital for Sick Children, University of Toronto, ON, Canada.

Background: The usefulness of echocardiographic indices, including those already used by modified Task Force Criteria (mTFC), and others such as strain imaging, to identify arrhythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established.

Methods: Echocardiograms from 120 adolescents investigated for ARVC (13±4 years) were retrospectively analyzed. According to the mTFC, patients were classified into definite (n=38), borderline (n=39), or possible (n=43) ARVC. Read More

Circ Cardiovasc Imaging 2019 04;12(4):e009084

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

Neth Heart J 2019 Oct;27(10):480-486

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

Monaldi Arch Chest Dis 2019 Mar 27;89(1). Epub 2019 Mar 27.

San Giovanni Addolorata Community Hospital, Cardiology and Rehabilitation Unit, Department of Medicine, Rome.

We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Read More

Am J Cardiol 2019 05 23;123(10):1690-1695. Epub 2019 Feb 23.

Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, PR China. Electronic address:

Less is known about bradyarrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). This cross-sectional study aimed to assess the prevalence and clinical significance of bradyarrhythmias in ARVC. From May 1995 to December 2017, bradyarrhythmias including sick sinus syndrome, atrioventricular block, and intraventricular conductional block (ICB) were investigated in 522 ARVC patients. Read More

Echocardiography 2019 04 18;36(4):666-670. Epub 2019 Mar 18.

Harry Perkins Institute of Medical Research and Fiona Stanley Hospital, The University of Western Australia, Perth, Western Australia, Australia.

Background: There are discrepancies in the quantitative echocardiographic criteria for the right ventricle (RV) between the revised task force criteria (TFC) for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) and the guidelines for RV assessment endorsed by American Society of Echocardiography (ASE). Importantly, these criteria do not take into account potential adaptation of the RV to exercise. The goal of this study was to compare the revised TFC quantitative echocardiographic parameters in patients with ARVC/D, athletes and matched controls. Read More

JACC Cardiovasc Imaging 2019 08 13;12(8 Pt 1):1580-1582. Epub 2019 Mar 13.

Clin Res Cardiol 2019 Oct 13;108(10):1147-1162. Epub 2019 Mar 13.

Department of Clinical Radiology, University Hospital Muenster, Muenster, Germany.

Objectives: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS.

Methods: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT. Read More

Heart Fail Rev 2019 07;24(4):511-520

Semmelweis University Heart and Vascular Center, Városmajor St. 68, Budapest, H-1122, Hungary.

Right ventricular (RV) function has proven to be a prognostic factor in heart failure with reduced and preserved ejection fraction and in pulmonary hypertension. RV function is also a cornerstone in the management of novel clinical issues, such as mechanical circulatory support devices or grown-up congenital heart disease patients. Despite the notable amount of circumferentially oriented myofibers in the subepicardial layer of the RV myocardium, the non-longitudinal motion directions are often neglected in the everyday assessment of RV function by echocardiography. Read More

Clin Radiol 2019 03 23;74(3):228-234. Epub 2019 Jan 23.

Bristol Heart Institute, Bristol NIHR Cardiovascular Biomedical Research Unit (BRU), University of Bristol, UK. Electronic address:

Aim: To assess the role of cardiovascular magnetic resonance imaging (CMRI) in patients referred for suspected arrhythmogenic right ventricular cardiomyopathy (ARVC), its ability to identify ARVC mimics, and subsequent clinical impact.

Materials And Methods: The CMRI registry of the year 2014 was analysed to identify all consecutive patients referred for suspected ARVC. A comprehensive CMRI protocol that included anatomy, bi-ventricular function modules, and late gadolinium enhancement (LGE) was performed in all patients. Read More

Ann Cardiol Angeiol (Paris) 2019 Jun 22;68(3):175-180. 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

Ulster Med J 2019 Jan 22;88(1):15-16. Epub 2019 Jan 22.

Cardiology Department, Belfast HSC Trust.

We report a patient who first presented during childhood in the early 1960's with several episodes of ventricular tachycardia (VT) and we describe her management which reflected the best medical knowledge at the time. She then presented more than 50 years later, again with VT, at which time a definitive diagnosis of the underlying cause was made. Her case illustrates the evolution in the understanding and management of VT over the past 50 years. Read More

Arq Bras Cardiol 2019 01;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 01 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

J Cardiovasc Electrophysiol 2019 03 6;30(3):366-373. Epub 2019 Jan 6.

Department of Medicine, Section of Cardiac Electrophysiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Ripple mapping displays every deflection of a bipolar electrogram and enables the visualization of conduction channels (RMCC) within postinfarction ventricular scar to guide ventricular tachycardia (VT) ablation. The utility of RMCC identification for facilitation of VT ablation in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVC) has not been described.

Objective: We sought to (a) identify the slow conduction channels in the endocardial/epicardial scar by ripple mapping and (b) retrospectively analyze whether the elimination of RMCC is associated with improved VT-free survival, in ARVC patients. Read More

J Am Heart Assoc 2018 11;7(22):e009855

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

Background The new designation of arrhythmogenic cardiomyopathy defines a broader spectrum of disease phenotypes, which include right dominant, biventricular, and left dominant variants. We evaluated the relationship between electrocardiographic findings and contrast-enhanced cardiac magnetic resonance phenotypes in arrhythmogenic cardiomyopathy. Methods and Results We studied a consecutive cohort of patients with a definite diagnosis of arrhythmogenic cardiomyopathy, according to 2010 International Task Force criteria, who underwent electrocardiography and contrast-enhanced cardiac magnetic resonance. 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

Ann Noninvasive Electrocardiol 2019 05 9;24(3):e12619. Epub 2018 Nov 9.

Department of Cardiology, Dr. Suat Gunsel University of Kyrenia Hospital, Kyrenia, Mersin, Turkey.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and specific ventricular pathology. Repolarization abnormalities, the significant contributor to life-threatening arrhythmias and mortality, are frequently observed ECG changes in patients with ARVC. This study aimed to evaluate the changes in Tp-e interval, Tp-e/QT, Tp-e/QTc ratio, and traditional electrocardiographic features of electrical dispersion in patients with ARVC. Read More

Int J Cardiol 2019 06 26;284:99-104. Epub 2018 Oct 26.

Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK; NIHR University College London Hospitals Biomedical Research Centre, UK.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease that causes sudden cardiac death in the young. Inflammatory myocardial infiltrates have been described at autopsy and on biopsy, but there are few data on the presence of myocarditis in living patients with ARVC using non-invasive imaging techniques. FDG-PET is a validated technique for detecting myocardial inflammation in clinically suspected myocarditis. Read More

Anatol J Cardiol 2018 Nov;20(5):296-302

Scientific Laboratory of Molecular Genetics, Riga Stradins University; Riga-Latvia.

Objective: The Latvian arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD-C) registry was established to determine the genetic background of ARVD-C for analyzing discovered genetic variation frequencies in the European and Latvian populations.

Methods: In total, 38 patients with suspected ARVD-C were selected. The clinical parameters were defined according to the ARVD-C guidelines, PKP2 and DSG2 gene analysis was performed using the Sanger sequencing. 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 09;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 09;11(9):e007546

Precision Medicine Center of Excellence for ARVC and Complex Ventricular Arrhythmias, Johns Hopkins University School of Medicine, Baltimore, MD (T.Z., F.R.A., J.C., A.K., S.M., R.B., H.C., H.T.).

Background: Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy characterized by fibrofatty replacement of right ventricular myocardium resulting in reentrant ventricular tachycardia (VT). Cardiac magnetic resonance imaging (CMR) can noninvasively measure regional abnormalities using tissue-tracking strain as well as late gadolinium enhancement (LGE). In this study, we examine arrhythmogenic substrate using regional CMR strain, LGE, and electroanatomic mapping (EAM) in arrhythmogenic right ventricular cardiomyopathy patients presenting for VT ablation. Read More

Circ Cardiovasc Imaging 2018 09;11(9):e007344

Department of Radiology, University Medical Center Utrecht, The Netherlands (B.K.V.).

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that is predominantly known to affect the ventricles. Evidence for atrial involvement remains limited. Therefore, we aimed to characterize atrial involvement in ARVC using functional cardiac magnetic resonance, define the extent of atrial size and function variation attributable to ventricular variables, and identify cardiac magnetic resonance-based predictors of atrial arrhythmias (AA) in ARVC. Read More

Circulation 2018 08;138(6):642-645

Department of Cardiology (I.R., B.B.).

Circ Cardiovasc Imaging 2018 10;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