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Front Neurol 2021 24;12:668180. Epub 2021 May 24.

Department of Neurology, Mayo Clinic, Rochester, MN, United States.

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common muscular dystrophies and predominantly affects facial and shoulder girdle muscles. Previous case reports and cohort studies identified minor cardiac abnormalities in FSHD patients, but their nature and frequency remain incompletely characterized. We reviewed cardiac, neurological and genetic findings of 104 patients with genetically confirmed FSHD. Read More

Pol J Radiol 2021 9;86:e195-e203. Epub 2021 Apr 9.

All India Institute of Medical Sciences, New Delhi, India.

The left ventricular outflow is an anatomically complex region situated between the anterior leaflet of the mitral valve and the left ventricular aspect of the muscular and membranous interventricular septum. It gives rise to the aorta, provides support to the aortic valvular cusps, and houses important components of the conduction system. The left ventricular outflow handles high pressures and pressure variations and is subsequently affected by a variety of aetio-pathological conditions. Read More

Intern Emerg Med 2021 May 30. Epub 2021 May 30.

Cardiology and Cardiovascular Pathophysiology, Azienda Ospedaliero-Universitaria "S. Maria Della Misericordia", Perugia, Italy.

Despite the good specificity of exercise stress echocardiography (ESE) for the detection of coronary artery disease (CAD), false positive (FP) results may occur. We have previously reported that chest abnormalities may affect parameters of cardiac contractility. The influence of chest shape on ESE results has never been previously investigated. Read More

Echocardiography 2021 May 28. Epub 2021 May 28.

Cardiovascular Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.

Background: The present study is based on the World Heart Federation (WHF) echocardiographic criteria to assess the prevalence of subclinical rheumatic heart disease (RHD) and elucidate evolution of the disease when the cases were placed on appropriate antibiotic prophylaxis and regular follow-up. The prevalence of subclinical RHD reported by previous active surveillance studies among asymptomatic school children is not comparable to our study because of major differences in screening methods.

Methods: A random inclusion strategy was adopted to recruit urban and rural school children of Bikaner district in the state of Rajasthan, India. Read More

Rev Med Suisse 2021 May;17(740):1029-1033

Service de cardiologie, CHUV, 1011 Lausanne.

Mitral prolapse is a common condition, defined by the systolic bulging of at least one mitral leaflet into the left atrium, which is often accompanied by various degree of mitral insufficiency. While for most of the patients the prognosis is linked to the severity of the valve regurgitation and its repercussions on the left ventricle (dilation and/or dysfunction), a minority of patients present with severe ventricular arrhythmia and an increased risk of sudden cardiac death, irrespective of the severity of the mitral regurgitation. To describe this particular condition, the terms arrhythmic or malignant mitral valve prolapse have been coined. Read More

Medicine (Baltimore) 2021 May;100(21):e26148

Department of Cardiothoracic and Vascular Surgery, Kepler University Hospital, Medical Faculty, Johannes Kepler University, Linz, Austria.

Rationale: Redo surgeries after mitral valve repair are technically demanding. Procedures applying the NeoChord device (NeoChord Inc, St. Louis Park, MN) have proven to be safe and feasible in selected patients requiring mitral valve repair due to a leaflet prolapse or flail. Read More

Eur Heart J Cardiovasc Imaging 2021 Apr 27. Epub 2021 Apr 27.

Aurora Cardiovascular and Thoracic Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway, Ste. 880, Milwaukee, WI 53215, USA.

Kardiol Pol 2021 May 20. Epub 2021 May 20.

Department of Cardiology and Electrotherapy,Medical University of Gdansk, Gdańsk, Poland.

Gen Thorac Cardiovasc Surg 2021 May 10. Epub 2021 May 10.

Department of Cardiology, Osaka City General Hospital, 2-13-22, Miyakojima Hondori, Miyakojima, Osaka, 534-0021, Japan.

Atrial functional mitral regurgitation is a hot research topic in the field of mitral valve disease. Atrial functional mitral regurgitation is distinctly different from ventricular functional mitral regurgitation. The surgical indications for atrial functional mitral regurgitation have not been well established because of the small amount of evidence gathered to date. Read More

Neth Heart J 2021 May 4. Epub 2021 May 4.

Department of Cardiology, Maastricht University Medical Centre, Maastricht, The Netherlands.

Introduction: In patients with mitral annular disjunction (MAD), it can be difficult to assess the severity of mitral regurgitation (MR), as they present with a prolapsing volume (i.e. volume resulting from mitral valve prolapse, blood volume shift) rather than a regurgitant jet. Read More

Arrhythm Electrophysiol Rev 2021 Apr;10(1):33-37

Arrhythmia Unit and Electrophysiology Laboratories, IRCCS San Raffaele Scientific Institute, Milano, Italy.

Mitral valve prolapse (MVP) is the most common valvular heart disease, affecting 2-3% of the general population. Barlow's disease is a clinical syndrome characterised by MVP. Initially thought a benign condition, MVP is now recognised as a cause of sudden cardiac death and ventricular arrhythmias. Read More

Can J Cardiol 2021 Apr 29. Epub 2021 Apr 29.

Department of Neuroscience, Imaging and Clinical Sciences, "G.d'Annunzio" University of Chieti-Pescara, Italy. Electronic address:

We present the case of a 28-year-old man with a history of unexplained syncope, frequent ventricular arrhythmias, familial LMNA-related dilated cardiomyopathy (DCM) and mitral annular disjunction (MAD). We provide the first association of a novel truncating LMNA variant serving as a potential vulnerable substrate for arrhythmogenic MAD syndrome. This could suggest a possible synergistic role between concealed genetic variants (resulting in fibrosis as a 'substrate' for arrhythmogenesis) and the presence of mitral annular disjunction (the 'trigger' with mechanical stretch initiating ventricular arrhythmias), which may provide a link between mitral valve prolapse and sudden cardiac death. Read More

Int J Cardiol 2021 Jul 29;334:154-156. Epub 2021 Apr 29.

Section of Cardiovascular Imaging, Heart, Vascular and Thoracic Institute, Cleveland Clinic, OH, United States. Electronic address:

Background: Mitral annular disjunction (MAD) is an increasingly recognized entity associated with mitral valve prolapse (MVP), ventricular arrhythmias and death. Few studies have investigated the utility of myocardial deformation analysis in MAD. We compared chamber quantification including strain by transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) between MVP patients with and without MAD. Read More

Diagnostics (Basel) 2021 Apr 10;11(4). Epub 2021 Apr 10.

Cardiac MR Center (CRMC), Lausanne University Hospital (CHUV), 1100 Lausanne, Switzerland.

Mitral valve prolapse (MVP) was first described in the 1960s, and it is usually a benign condition. However, a subtype of patients are known to have a higher incidence of ventricular arrhythmias and sudden cardiac death, the so called "arrhythmic MVP." In recent years, several studies have been published to identify the most important clinical features to distinguish the benign form from the potentially lethal one in order to personalize patient's treatment and follow-up. Read More

Am J Case Rep 2021 Apr 29;22:e931359. Epub 2021 Apr 29.

Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

BACKGROUND Systolic anterior motion (SAM) is the dynamic anteriorly directed movement of the anterior mitral valve leaflet during systole toward the left ventricular outflow tract (LVOT). The history of SAM in progressive hypertrophic cardiomyopathy (HCM) is unclear. It is believed that SAM is an irreversible process that progresses as the gradient over the LVOT increases. Read More

Int J Cardiovasc Imaging 2021 Apr 25. Epub 2021 Apr 25.

Department of Cardiovascular Imaging - Centro, Cardiologico Fondazione Monzino IRCCS, via Carlo Parea 4, 20138, Milan, MI, Italy.

Patients with mitral valve prolapse (MVP), undergoing early surgery for severe regurgitation, are usually characterized by a low degree of right chambers' remodeling. In this selected population, the mechanisms leading to tricuspid annular (TA) dilatation (TAD) are not well understood. In this setting, we aimed to evaluate, using three-dimensional echocardiography (3DE), how right chambers affect TA size and might contribute to functional tricuspid regurgitation (FTR) progression. Read More

J Card Surg 2021 Apr 24. Epub 2021 Apr 24.

Division of Cardiac Surgery, Yale School of Medicine, New Haven, Connecticut, USA.

Objective: To assess the impact of surgeon experience on the outcomes of degenerative mitral valve disease.

Methods: We reviewed all patients who had surgery for degenerative mitral valve disease between 2011 and 2016. Experienced surgeon was defined as performing ≥ 25 mitral valve operations/year. Read More

Cardiol Clin 2021 May;39(2):289-294

Department of Cardiovaasucular Medicine, Kobe City Medical Center General Hospital, 2-1-1 Minatojima-Minamimachi, Chuo-ku, Kobe 650-0047, Japan.

Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus, which has been described by pathologists to be associated with mitral leaflet prolapse. Mitral annular disjunction is a common finding in patients with myxomatous mitral valve diseases. The prevalence of mitral annular disjunction should be checked routinely during presurgical imaging. Read More

Herz 2021 Apr 21. Epub 2021 Apr 21.

Emergency Medicine Department, SBU Kanuni Sultan Suleyman Training and Research Hospita, Istanbul, Turkey.

Background: The incidence of supraventricular arrhythmia (SVA) is high in patients with mitral valve prolapse (MVP). The purpose of our study was to determine the role of parameters showing atrial conduction heterogeneity such as P‑wave dispersion (PWD) and atrial electromechanical delay (AEMD) in predicting the development of SVA in MVP patients.

Methods: A total of 76 patients with MVP (56 female, 20 male) were included in the study. Read More

Eur Radiol 2021 Apr 19. Epub 2021 Apr 19.

Radiology Unit, Department of Surgical Sciences, University of Turin, Turin, Italy.

Objectives: Anatomical substrate and mechanical trigger co-act in arrhythmia's onset in patients with bileaflet mitral valve prolapse (bMVP). Feature tracking (FT) may improve risk stratification provided by cardiac magnetic resonance (CMR). The aim was to investigate differences in CMR and FT parameters in bMVP patients with and without complex arrhythmias (cVA and no-cVA). Read More

Heart Lung Circ 2021 Apr 15. Epub 2021 Apr 15.

Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Chiba, Japan.

Background: Long-term predictors of recurrent mitral regurgitation (MR) after mitral valve plasty (MVP) remain to be elucidated. This study sought to determine the prognostic factors of recurrent MR during long-term follow-up after MVP, by analysing findings of three-dimensional transoesophageal echocardiography (TEE) conducted after MVP.

Methods: This study analysed 207 patients who underwent MVP for A2 and/or P2 prolapse and received TEE before discharge. Read More

JACC Cardiovasc Imaging 2021 Apr 7. Epub 2021 Apr 7.

Department of Cardiology, Neural and Metabolic Sciences, Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milan, Italy.

JACC Cardiovasc Imaging 2021 Jun 14;14(6):1110-1112. Epub 2021 Apr 14.

Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.

Turk Kardiyol Dern Ars 2021 Apr;49(3):245

Department of Cardiac Surgery, Tehran University of Medical Sciences, Tehran, Iran.

Eur J Prev Cardiol 2021 Apr 13. Epub 2021 Apr 13.

Clinic for General and Interventional Cardiology/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universität Bochum, Bad Oeynhausen, Germany.

This article provides an overview of the recommendations from the Sports Cardiology section of the European Association of Preventive Cardiology on sports participation in individuals with valvular heart disease (VHD). The aim of these recommendations is to encourage regular physical activity including sports participation, with reasonable precaution to ensure a high level of safety for all affected individuals. Valvular heart disease is usually an age-related degenerative process, predominantly affecting individuals in their fifth decade and onwards. Read More

ESC Heart Fail 2021 Jun 9;8(3):2310-2315. Epub 2021 Apr 9.

MEBIC Consortium, San Raffaele Open University and IRCCS San Raffaele Pisana, Rome, Italy.

We report a novel cardiomyopathy associated to Usher syndrome and related to combined mutation of MYO7A and Calreticulin genes. A 37-year-old man with deafness and vision impairment because of retinitis pigmentosa since childhood and a MYO7A gene mutation suggesting Usher syndrome, developed a dilated cardiomyopathy with ventricular tachyarrhythmias and recurrent syncope. At magnetic resonance cardiomyopathy was characterized by left ventricular dilatation with hypo-contractility and mitral prolapse with valve regurgitation. Read More

Kyobu Geka 2021 Mar;74(3):213-216

Department of Cardiovascular Surgery, Hiroshima City Asa Citizens Hospital, Hiroshima, Japan.

An 83-year-old woman with congestive heart failure due to severe mitral regurgitation was referred to our department. Because acute coronary syndrome was suspected, the patient underwent emergent coronary artery angiography, which showed 75% stenosis of segment 2 and 90% stenosis of segment 11. Subsequently, segment 11 was treated by percutaneous coronary intervention. Read More

Am J Med Genet A 2021 Apr 3. Epub 2021 Apr 3.

Rare Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli-IRCCS, Rome, Italy.

Smith-Magenis syndrome (SMS) is a genetic disorder characterized by multiple congenital anomalies, sleep disturbance, behavioral impairment, and intellectual disability. Its genetic cause has been defined as an alteration in the Retinoic Acid-Induced 1 gene. Cardiac anomalies have been reported since the first description of this condition in patients with 17p11. Read More

Dev Dyn 2021 Apr 2. Epub 2021 Apr 2.

Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, South Carolina, USA.

Background: Mitral valve prolapse (MVP) is a common and progressive cardiovascular disease with developmental origins. How developmental errors contribute to disease pathogenesis are not well understood.

Results: A multimeric complex was identified that consists of the MVP gene Dzip1, Cby1, and β-catenin. Read More