774 results match your criteria Imaging in Arrhythmogenic Right Ventricular Dysplasia ARVD


Assessment of premature ventricular beats in athletes.

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

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http://dx.doi.org/10.1016/j.ancard.2018.10.013DOI Listing
January 2019
3 Reads

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) - What We Have Learned after 40 Years of the Diagnosis of This Clinical Entity.

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

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http://dx.doi.org/10.5935/abc.20180266DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317628PMC
January 2019
1 Read

Hemodynamic Changes in the Right Ventricle Induced by Variations of Cardiac Output: A Possible Mechanism for Arrhythmia Occurrence in the Outflow Tract.

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

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http://dx.doi.org/10.1038/s41598-018-36614-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331649PMC
January 2019
3 Reads

Arrhythmogenic Cardiomyopathy in 2018-2019: ARVC/ALVC or Both?

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

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https://linkinghub.elsevier.com/retrieve/pii/S14439506183195
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http://dx.doi.org/10.1016/j.hlc.2018.10.013DOI Listing
January 2019
32 Reads

Form Follows Function?

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.

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http://dx.doi.org/10.1161/CIRCIMAGING.118.008271DOI Listing
September 2018
2 Reads

Advanced Arrhythmogenic Cardiomyopathy in Former Marathon Runner.

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.).

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http://dx.doi.org/10.1161/CIRCIMAGING.118.008204DOI Listing
October 2018
3 Reads

Arrhythmogenic right ventricular dysplasia, cutaneous manifestations and desmoplakin mutation: Carvajal syndrome.

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

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

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http://dx.doi.org/10.1111/ped.13683DOI Listing
October 2018
13 Reads

Uhl's anomaly: rare but does exist.

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

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http://dx.doi.org/10.1177/0218492316674859DOI Listing
September 2018
9 Reads

Cardiac Magnetic Resonance Imaging (MRI) Findings in Arrhythmogenic Right Ventricular Dysplasia (ARVD) Compared with Echocardiography.

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

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http://www.mdpi.com/2076-3271/6/3/80
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http://dx.doi.org/10.3390/medsci6030080DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6163444PMC
September 2018
7 Reads

Echocardiographic assessment of right ventricular systolic function in Boxers with arrhythmogenic right ventricular cardiomyopathy.

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

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http://dx.doi.org/10.1016/j.jvc.2018.07.005DOI Listing
October 2018
2 Reads

Clinical Diagnosis, Imaging, and Genetics of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: JACC State-of-the-Art Review.

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

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http://dx.doi.org/10.1016/j.jacc.2018.05.065DOI Listing
August 2018
10 Reads

Role of PET/CT in multimodality imaging in differentiating cardiac sarcoidosis from arrhythmogenic right ventricular dysplasia.

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

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

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http://dx.doi.org/10.1007/s12350-018-1382-9DOI Listing
July 2018
3 Reads

Clinical profile in arrhythmogenic cardiomyopathy and a recessive plakophilin-2 gene mutation.

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

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http://dx.doi.org/10.1016/j.ihj.2017.10.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6034022PMC
September 2018
2 Reads

Right bundle branch block and conduction disturbances in Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy.

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

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http://dx.doi.org/10.1007/s10840-018-0386-3DOI Listing
August 2018
5 Reads

Electrocardiogram and Imaging: An Integrated Approach to Arrhythmogenic Cardiomyopathies.

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

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http://dx.doi.org/10.1016/j.ccep.2018.02.011DOI Listing
June 2018
6 Reads

Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy: Progress and Pitfalls.

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

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http://dx.doi.org/10.1016/j.hlc.2018.03.023DOI Listing
November 2018
3 Reads
1 Citation
1.172 Impact Factor

Blockade of the renin-angiotensin-aldosterone system in patients with arrhythmogenic right ventricular dysplasia: A double-blind, multicenter, prospective, randomized, genotype-driven study (BRAVE study).

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

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http://dx.doi.org/10.1002/clc.22884DOI Listing
March 2018
13 Reads

A Prenatal Case of Arrhythmogenic Right Ventricular Dysplasia.

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.

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http://dx.doi.org/10.5935/abc.20180022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5855915PMC
February 2018
3 Reads

Pregnancy and newborn outcomes in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

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

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http://dx.doi.org/10.1016/j.ijcard.2017.11.067DOI Listing
May 2018
3 Reads

Endomyocardial biopsy in differential diagnosis between arrhythmogenic right ventricular cardiomyopathy and dilated cardiomyopathy: an in vitro simulated study.

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

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http://dx.doi.org/10.1016/j.carpath.2018.02.003DOI Listing
October 2018
11 Reads

Diagnostic Criteria, Genetics, and Molecular Basis of Arrhythmogenic Cardiomyopathy.

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

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http://dx.doi.org/10.1016/j.hfc.2018.01.002DOI Listing
April 2018
15 Reads

Common presentation of rare diseases: Arrhythmogenic right ventricular cardiomyopathy and its mimics.

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

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http://dx.doi.org/10.1016/j.ijcard.2018.01.021DOI Listing

Fatty Images of the Heart: Spectrum of Normal and Pathological Findings by Computed Tomography and Cardiac Magnetic Resonance Imaging.

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

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http://dx.doi.org/10.1155/2018/5610347DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818975PMC
August 2018
26 Reads

Association of regional epicardial right ventricular electrogram voltage amplitude and late gadolinium enhancement distribution on cardiac magnetic resonance in patients with arrhythmogenic right ventricular cardiomyopathy: Implications for ventricular tachycardia ablation.

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

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https://linkinghub.elsevier.com/retrieve/pii/S15475271183014
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http://dx.doi.org/10.1016/j.hrthm.2018.02.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6026041PMC
July 2018
23 Reads

Intracardiac ultrasound to detect aneurysm in arrhythmogenic right ventricular dysplasia/cardiomyopathy.

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

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http://dx.doi.org/10.1093/omcr/omx088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5786252PMC
January 2018
7 Reads

Coupling interval variability of premature ventricular contractions in patients with different underlying pathology: an insight into the arrhythmia mechanism.

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

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http://dx.doi.org/10.1007/s10840-017-0309-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5797566PMC
January 2018
10 Reads

Right ventricular remodeling in athletes and in arrhythmogenic cardiomyopathy.

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

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http://dx.doi.org/10.1080/14017431.2017.1416158DOI Listing
February 2018
38 Reads

Isolated Cardiac Sarcoidosis Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy.

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

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http://dx.doi.org/10.2169/internalmedicine.9395-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5891523PMC
March 2018
7 Reads

Auxiliary diagnostic potential of ventricle geometry and late gadolinium enhancement in left ventricular non-compaction; non-randomized case control study.

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

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http://dx.doi.org/10.1186/s12872-017-0721-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5718034PMC
December 2017
44 Reads

Right ventricular systolic function and mechanical dispersion identify patients with arrhythmogenic right ventricular cardiomyopathy.

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

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http://dx.doi.org/10.1111/cpf.12479DOI Listing
September 2018
9 Reads

Total pericardium agenesis mistaken for arrhythmogenic right ventricular cardiomyopathy.

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.

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http://dx.doi.org/10.1093/ehjci/jex251DOI Listing
January 2018
9 Reads

Epicardial ablation of ventricular tachycardia in a patient with arrhythmogenic right ventricular dysplasia after failed conventional endocardial ablation: A case for remote navigation with functional image integration.

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

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http://dx.doi.org/10.21542/gcsp.2016.39DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5624191PMC
December 2016
2 Reads

Exercise and the right ventricle: a potential Achilles' heel.

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

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http://dx.doi.org/10.1093/cvr/cvx156DOI Listing
October 2017
7 Reads

Combining computer modelling and cardiac imaging to understand right ventricular pump function.

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

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http://dx.doi.org/10.1093/cvr/cvx154DOI Listing
October 2017
10 Reads

Arrhythmogenic Cardiomyopathy.

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

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http://dx.doi.org/10.1161/CIRCRESAHA.117.309345DOI Listing
September 2017
7 Reads

Detection of intramyocardial fat infiltration by computerized tomography in a patient with arrhythmogenic biventricular cardiomyopathy.

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.

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http://dx.doi.org/10.1016/j.jcct.2017.09.004DOI Listing
November 2017
1 Read

Arrhythmogenic Right Ventricular Cardiomyopathy in an Endurance Athlete Presenting with Ventricular Tachycardia and Normal Right Ventricular Function.

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

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http://dx.doi.org/10.14503/THIJ-16-6025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5577959PMC
August 2017
4 Reads

Heart Failure Is Common and Under-Recognized in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.

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

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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.116.003819DOI Listing
September 2017
19 Reads
5.891 Impact Factor

Feature tracking CMR reveals abnormal strain in preclinical arrhythmogenic right ventricular dysplasia/ cardiomyopathy: a multisoftware feasibility and clinical implementation study.

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

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http://dx.doi.org/10.1186/s12968-017-0380-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5581480PMC
September 2017
33 Reads

Arrhythmogenic right-ventricular cardiomyopathy and cardiac microvascular disease: a rare association or a possible link?

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.

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http://dx.doi.org/10.2459/JCM.0b013e328361d1c7DOI Listing
October 2017
16 Reads

Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction.

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

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http://dx.doi.org/10.1177/2150135117707459DOI Listing
January 2017
4 Reads

Case reports of two pedigrees with recessive arrhythmogenic right ventricular cardiomyopathy associated with homozygous Thr335Ala variant in DSG2.

BMC Med Genet 2017 08 17;18(1):86. Epub 2017 Aug 17.

Blueprint Genetics, Helsinki, Finland.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease, involving changes in ventricular myocardial tissue and leading to fatal arrhythmias. Mutations in desmosomal genes are thought to be the main cause of ARVC. However, the exact molecular genetic etiology of the disease still remains largely inconclusive, and this along with large variabilities in clinical manifestations complicate clinical diagnostics. Read More

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http://dx.doi.org/10.1186/s12881-017-0442-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5561604PMC
August 2017
3 Reads

First Reported Case of Arrhythmogenic Right Ventricular Cardiomyopathy in Oman.

Oman Med J 2017 Jul;32(4):339-343

Department of Radiology and Molecular Imaging, Sultan Qaboos University Hospital, Muscat, Oman.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder characterized by fatty degeneration of the right ventricular myocardium with variable involvement of the left ventricle. The condition is associated with exercise-mediated ventricular tachycardia and is one of the recognized causes of sudden cardiac death in the young and in athletes. Here, we report the first confirmed case of ARVC in Oman and present its electrocardiographic, echocardiographic features, and radiological findings on gated, contrast-enhanced cardiac computed tomography. Read More

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http://dx.doi.org/10.5001/omj.2017.64DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5534230PMC
July 2017
4 Reads

Masked inherited primary arrhythmia syndromes in sudden cardiac death patients accompanied by coronary vasospasm.

Korean J Intern Med 2017 Sep 11;32(5):836-846. Epub 2017 Aug 11.

Department of Cardiovascular Medicine, Chonnam National University Hospital, Gwangju, Korea.

Background/aims: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. Read More

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http://dx.doi.org/10.3904/kjim.2015.283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5583445PMC
September 2017
58 Reads

A novel noninvasive surface ECG analysis using interlead QRS dispersion in arrhythmogenic right ventricular cardiomyopathy.

PLoS One 2017 3;12(8):e0182364. Epub 2017 Aug 3.

Division of Cardiology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.

Background: This study investigated the feasibility of using the precordial surface ECG lead interlead QRS dispersion (IQRSD) in the identification of abnormal ventricular substrate in arrhythmogenic right ventricular cardiomyopathy (ARVC).

Methods: Seventy-one consecutive patients were enrolled and reclassified into 4 groups: definite ARVC with epicardial ablation (Group 1), ARVC with ventricular tachycardia (VT, Group 2), idiopathic right ventricular outflow tract VT without ARVC (Group 3), and controls without VT (Group 4). IQRSD was quantified by the angular difference between the reconstruction vectors obtained from the QRS-loop decomposition, based on a principal component analysis (PCA). Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0182364PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5542590PMC
October 2017
31 Reads

An accordion not to be played.

J Cardiovasc Med (Hagerstown) 2017 09;18(9):710-712

aCardio-Thoracic and Vascular Department, San Raffaele Hospital and Vita-Salute University San Raffaele bIBFM CNR cRadiology Department, San Raffaele Hospital and Vita-Salute University San Raffaele, Milan, Italy.

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http://dx.doi.org/10.2459/JCM.0b013e32835fa840DOI Listing
September 2017
6 Reads

Electrical and Structural Substrate of Arrhythmogenic Right Ventricular Cardiomyopathy Determined Using Noninvasive Electrocardiographic Imaging and Late Gadolinium Magnetic Resonance Imaging.

Circ Arrhythm Electrophysiol 2017 Jul;10(7)

From the Department of Biomedical Engineering (C.M.A., Y.R.) and Cardiac Bioelectricity and Arrhythmia Center (C.M.A., Y.R.), Washington University, St. Louis, MO; Department of Medicine, Cardiovascular Division, Washington University in St. Louis, MO (Y.R.); Department of Cardiac Electrophysiology, The Barts Heart Center, St Bartholomew's Hospital, London, United Kingdom (N.T.S., M.O., S.J., A.P., W.J.M., P.D.L.); and Institute of Cardiovascular Science, University College London, United Kingdom (N.T.S., S.R., H.B., M.O., S.J., A.P., W.J.M., J.C.M., P.D.L.).

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a significant cause of sudden cardiac death in the young. Improved noninvasive assessment of ARVC and better understanding of the disease substrate are important for improving patient outcomes.

Methods And Results: We studied 20 genotyped ARVC patients with a broad spectrum of disease using electrocardiographic imaging (a method for noninvasive cardiac electrophysiology mapping) and advanced late gadolinium enhancement cardiac magnetic resonance scar imaging. Read More

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http://dx.doi.org/10.1161/CIRCEP.116.005105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5533087PMC
July 2017
12 Reads

MiR-320a as a Potential Novel Circulating Biomarker of Arrhythmogenic CardioMyopathy.

Sci Rep 2017 07 6;7(1):4802. Epub 2017 Jul 6.

Cardiac Arrhythmia Research Center, Centro Cardiologico Monzino IRCCS, Milan, Italy.

Diagnosis of Arrhythmogenic CardioMyopathy (ACM) is challenging and often late after disease onset. No circulating biomarkers are available to date. Given their involvement in several cardiovascular diseases, plasma microRNAs warranted investigation as potential non-invasive diagnostic tools in ACM. Read More

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http://dx.doi.org/10.1038/s41598-017-05001-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5500514PMC
July 2017
36 Reads

Isolated cardiac sarcoidosis masquerading as right ventricular outflow tract ventricular tachycardia.

BMJ Case Rep 2017 Jun 30;2017. Epub 2017 Jun 30.

University of Massachusetts Medical School-Baystate, Springfield, Massachusetts, USA.

A 67-year-old man with coronary artery disease (CAD) and left anterior descending artery (LAD) stent presented with symptomatic monomorphic ventricular tachycardia (VT) at a rate of 190 bpm requiring cardioversion. ECG showed left bundle branch block pattern and inferior axis, suggestive of a right ventricular outflow tract (RVOT) focus rather than left ventricular scar due to LAD territory myocardial infarction (MI). Echocardiography showed normal wall motion. Read More

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http://dx.doi.org/10.1136/bcr-2017-220604DOI Listing
June 2017
35 Reads

Disease model discovery from 3,328 gene knockouts by The International Mouse Phenotyping Consortium.

Nat Genet 2017 Aug 26;49(8):1231-1238. Epub 2017 Jun 26.

William Harvey Research Institute, Queen Mary University of London, London, UK.

Although next-generation sequencing has revolutionized the ability to associate variants with human diseases, diagnostic rates and development of new therapies are still limited by a lack of knowledge of the functions and pathobiological mechanisms of most genes. To address this challenge, the International Mouse Phenotyping Consortium is creating a genome- and phenome-wide catalog of gene function by characterizing new knockout-mouse strains across diverse biological systems through a broad set of standardized phenotyping tests. All mice will be readily available to the biomedical community. Read More

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http://dx.doi.org/10.1038/ng.3901DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5546242PMC
August 2017
38 Reads