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restrictive cardiomyopathy

Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypman, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
Giulio Calcagni, Giuseppe Limongelli, Angelo D'Ambrosio, Francesco Gesualdo, Maria Cristina Digilio, Anwar Baban, Sonia B Albanese, Paolo Versacci, Enrica De Luca, Giovanni B Ferrero, Giuseppina Baldassarre, Gabriella Agnoletti, Elena Banaudi, Jan Marek, Juan P Kaski, Giulia Tuo, Maria Giovanna Russo, Giuseppe Pacileo, Ornella Milanesi, Daniela Messina, Maurizio Marasini, Francesca Cairello, Roberto Formigari, Maurizio Brighenti, Bruno Dallapiccola, Marco Tartaglia, Bruno Marino
A comprehensive description of morbidity and mortality in patients affected by mutations in genes encoding for signal transducers of the RAS-MAPK cascade (RASopathies) was performed in our study recently published in the International Journal of Cardiology. Seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET), collaborated in this multicentric, observational, retrospective data analysis and collection. In this study, clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed...
February 2018: Data in Brief
Mark R Hazebroek, Ingrid Krapels, Job Verdonschot, Arthur van den Wijngaard, Els Vanhoutte, Marije Hoos, Luc Snijders, Lieke van Montfort, Maryvonne Witjens, Robert Dennert, Harry J G M Crijns, Hans-Peter Brunner-La Rocca, Han G Brunner, Stephane Heymans
BACKGROUND: Genetic evaluation is recommended in patients with unexplained dilated cardiomyopathy (DCM), but its diagnostic yield and prognostic relevance in unexplained isolated left ventricular dysfunction (LVdys) is unknown. METHODS AND RESULTS: A total of 127 LVdys and 262 DCM patients underwent genetic screening. Long-term outcome consisted of a combined end point of life-threatening arrhythmia, heart transplantation, and death. At baseline, LVdys patients were younger and had less frequently New York Heart Association class ≥3 when compared with DCM (55±13 versus 58±12; P =0...
March 2018: Circulation. Heart Failure
Ming Gao, Weihua Zhang, Waiou Zhao, Ling Qin, Fei Pei, Yang Zheng
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis." PATIENT CONCERNS: We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy...
March 2018: Medicine (Baltimore)
Heather N Anderson, Frank Cetta, David J Driscoll, Timothy M Olson, Michael J Ackerman, Jonathan N Johnson
Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975 and 2013 at our institution. Demographic, clinical, echocardiographic, and catheterization data were abstracted...
February 12, 2018: American Journal of Cardiology
Naveen L Pereira, Martha Grogan, G William Dec
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies...
March 13, 2018: Journal of the American College of Cardiology
Naveen L Pereira, Martha Grogan, G William Dec
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies...
March 13, 2018: Journal of the American College of Cardiology
Mohan John, Leonard L Bailey
Neonatal heart transplantation was developed and established in the 1980's as a durable modality of therapy for complex-uncorrectable heart disease. Patients transplanted in the neonatal period have experienced unparalleled long-term survival, better than for any other form of solid-organ transplantation. However, the limited availability of neonatal and young infant donors has restricted the indications and applicability of heart transplantation among newborns in the current era. Indications for heart transplantation include congenital heart disease not amenable to other forms of surgical palliation, and cardiomyopathy, including some primary tumors...
January 2018: Annals of Cardiothoracic Surgery
Roland Hetzer, Mariano Francisco Del Maria Javier, Eva Maria Delmo Walter
Background: While heart transplantation has gained recognition as the gold standard therapy for advanced heart failure, the scarcity of donor organs has become an important concern. The evolution of surgical alternatives such as ventricular assist devices (VADs), allow for recovery of the myocardium and ensure patient survival until heart transplantation becomes possible. This report elaborates the role of VADs as a bridge to heart transplantation in infants and children (≤18 years old) with end-stage heart failure...
January 2018: Annals of Cardiothoracic Surgery
Tamanna K Singh, Aaron L Baggish
Premature ventricular beats (PVBs) in competitive athletes are incidentally found during pre-participation ECG screening. Their clinical significance remains debatable with several studies suggesting they are a benign reflection of athlete's heart, and others proposing they may indicate underlying structural heart disease and heightened risk for sudden cardiac death (SCD). Areas covered: Effective management of athletes with PVBs may best be accomplished using an algorithmic approach for risk stratification with a goal of differentiating benign PVBs from those reflective of underlying cardiomyopathies...
February 23, 2018: Expert Review of Cardiovascular Therapy
Gianvincenzo Sparacia, Roberto Cannella, Vincenzina Lo Re, Angelo Gambino, Giuseppe Mamone, Roberto Miraglia
PURPOSE: Cerebral microbleeds (CMBs) are small rounded lesions representing cerebral hemosiderin deposits surrounded by macrophages that results from previous microhemorrhages. The aim of this study was to review the distribution of cerebral microbleeds in patients with end-stage organ failure and their association with specific end-stage organ failure risk factors. MATERIALS AND METHODS: Between August 2015 and June 2017, we evaluated 15 patients, 9 males, and 6 females, (mean age 65...
February 17, 2018: La Radiologia Medica
Chiara Locatelli, Danitza Pradelli, Giulia Campo, Ilaria Spalla, Alice Savarese, Paola G Brambilla, Claudio Bussadori
Objectives Large studies focusing on restrictive cardiomyopathy (RCM) in the cat are scarce. The aims of this retrospective study were to describe epidemiological characteristics and to analyse prognostic factors affecting survival in cats with RCM. Methods The clinical archives of the Gran Sasso Veterinary Clinic (Milan, Italy) and of the cardiology unit of the Department of Veterinary Medicine (University of Milan, Italy) from 1997-2015 were reviewed for all cats diagnosed with RCM based on an echocardiographic examination (left atrial/bi-atrial enlargement, normal left ventricle wall thickness, normal or mildly decreased systolic function and restrictive left ventricle filling pattern with pulsed Doppler echocardiography)...
February 1, 2018: Journal of Feline Medicine and Surgery
Jay Brieler, Matthew A Breeden, Jane Tucker
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Dilated cardiomyopathy can be genetic or acquired and typically presents with classic symptoms of heart failure with reduced ejection fraction...
November 15, 2017: American Family Physician
Satoshi Kainuma, Toshihiro Funatsu, Haruhiko Kondoh, Takenori Yokota, Shusaku Maeda, Yasuhiro Shudo, Hajime Matsue, Masami Nishino, Takashi Daimon, Koichi Toda, Yoshiki Sawa, Kazuhiro Taniguchi
OBJECTIVES: The effects of restrictive mitral annuloplasty (RMA) on subvalvular geometry remains unknown. We evaluated changes in left ventricular (LV) function, severity of mitral regurgitation (MR), and leaflet tethering parameters after RMA and clarified their associations. METHODS: In 44 patients with clinically relevant functional MR who underwent RMA, distances between papillary muscle (PM) tips and anterior mitral annulus (PM tethering distance), leaflet angles relative to lines connecting annuli, and interpapillary muscle distance (IPMD) were serially quantified...
December 18, 2017: Journal of Thoracic and Cardiovascular Surgery
Jiao Mu, Guohui Zhang, Dazhong Xue, Mengrou Xi, Jiarui Qi, Hongmei Dong
BACKGROUND AND OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is increasingly recognized in forensic practice with controversial diagnosis. Here we described the epidemiological characteristics and reported the pathogenetic mechanism, diagnostic challenges, and forensic implications of Chinese ARVC autopsy cases. METHODS: Two cases of sudden cardiac death owing to ARVC were reported. Retrospective analysis were performed on such 2 cases and 45 cases of separate ARVC complete autopsy case reports through Chinese literature databases in the last 30 years...
November 2017: Medicine (Baltimore)
Philippe Charron, Perry M Elliott, Juan R Gimeno, Alida L P Caforio, Juan Pablo Kaski, Luigi Tavazzi, Michal Tendera, Carole Maupain, Cécile Laroche, Pawel Rubis, Ruxandra Jurcut, Leonardo Calò, Tiina M Heliö, Gianfranco Sinagra, Marija Zdravkovic, Aušra Kavoliuniene, Stephan B Felix, Jacek Grzybowski, Maria-Angela Losi, Folkert W Asselbergs, José Manuel García-Pinilla, Joel Salazar-Mendiguchia, Katarzyna Mizia-Stec, Aldo P Maggioni
Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. Methods and results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]...
January 24, 2018: European Heart Journal
Jacoba J Louw, Ricardo Nunes Bastos, Xiaowen Chen, Céline Verdood, Anniek Corveleyn, Yaojuan Jia, Jeroen Breckpot, Marc Gewillig, Hilde Peeters, Massimo M Santoro, Francis Barr, Koenraad Devriendt
Congenital or neonatal cardiomyopathies are commonly associated with a poor prognosis and have multiple etiologies. In two siblings, a male and female, we identified an undescribed type of lethal congenital restrictive cardiomyopathy affecting the right ventricle. We hypothesized a novel autosomal recessive condition. To identify the cause, we performed genetic, in vitro and in vivo studies. Genome-wide SNP typing and parametric linkage analysis was done in a recessive model to identify candidate regions. Exome sequencing analysis was done in unaffected and affected siblings...
January 22, 2018: PLoS Genetics
José Mario Baggio, Cristina Machado Camargo Afiune, Jorge Y Afiune, Alvaro V Sarabanda, Fernando A Atik
A 12-year-old child with end-stage heart failure due to restrictive cardiomyopathy was submitted to orthotopic heart transplantation. Primary graft dysfunction required venous arterial extra-corporeal membrane oxygenation. Heart function normalized, but complete atrioventricular block remained after 3 weeks. A dual-chamber pacing with transvenous left ventricle pacing through the coronary sinus was performed. At 5-year follow-up, the patient is stable with the same pacing system and with preserved ventricular function...
January 22, 2018: ESC Heart Failure
Muhammad U Dogar, Niel N Shah, Sameera Ishtiaq, Parin N Shah, Pratik Shah, Shawn Mathew, Timothy J Vittorio
No abstract text is available yet for this article.
January 20, 2018: Postgraduate Medical Journal
Patricia E Longmuir, Margaret Sampson, Jennifer Ham, Makenzie Weekes, Bhavika J Patel, Robert M Gow
Potentially fatal arrhythmias add to the mental health challenges of adolescence. This systematic review sought to summarise current knowledge regarding the mental health of adolescents and pre-adolescents diagnosed with inherited arrhythmia syndromes. Searches combining psychological problems with inherited cardiac arrhythmia diagnoses identified 16 studies with paediatric (<18 years) inherited arrhythmia patients. All studies were cross-sectional; 8/16 required an implantable cardioverter defibrillator...
January 18, 2018: Cardiology in the Young
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