Srilakshmi Mandayam Adhyapak, Venkateswara Rao Parachuri
Ischemic cardiomyopathy often presents with advanced heart failure necessitating an intensive multidisciplinary approach to management. These patients constitute a very difficult population presenting with angina and ventricular tachyarrhythmias sometimes presenting as sudden cardiac death. In those patients presenting with spontaneous ventricular tachyarrhythmias, treatment with automated intracardiac defibrillator implantation has a significant mortality benefit. In patients with significant left ventricular (LV) dilatation and a left ventricular ejection fraction ≤ 30%, guidelines state that device implantation should be preceded by programmed electrical stimulation, which demonstrates inducible ventricular tachyarrhythmia...
January 2020: Indian Journal of Thoracic and Cardiovascular Surgery
Dongxu Xu, Hao Zhang, Huiling Cheng, Tianbao Xu, Wei Sun, Yanhui Sheng, Rong Yang, Dongjie Xu, Fang Zhou, Yanli Zhou, Fang Xu, Hongyan Zhu, IokFai Cheang, Qiang Ding, Yan Chen, Pei Zhao, Xiangqing Kong, Xinli Li, Haifeng Zhang
BACKGROUND: Pulmonary hypertension due to left heart disease (PH-LHD) is the most prevalent type of pulmonary hypertension (PH). The hemodynamic diagnostic standard of pulmonary arterial wedge pressure (PAWP) >15 mm Hg that is traditionally recommended by guidelines is being challenged. METHODS: To address this problem, we analyzed the data of 154 patients with PH-LHD admitted to our center from April 2013 to March 2018. Pharmacological or nonpharmacological treatment of underlying left heart disease was offered to all 154 patients...
September 29, 2020: Herz
Alessandra Carnevale, Sandra Rosas-Madrigal, Rigoberto Rosendo-Gutiérrez, Enrique López-Mora, Sandra Romero-Hidalgo, Nydia Avila-Vazzini, Leonor Jacobo-Albavera, Mayra Domínguez-Pérez, Gilberto Vargas-Alarcón, Fernando Pérez-Villatoro, Juana Inés Navarrete-Martínez, María Teresa Villarreal-Molina
BACKGROUND: Dilated cardiomyopathy (DCM) is a major cause of nonischemic heart failure and death in young adults. Next generation sequencing (NGS) has become part of the diagnostic workup in idiopathic and familial DCM. More than 50 DCM genes have been identified, revealing great molecular heterogeneity and variable diagnostic yield. Interpretation of variant pathogenicity is challenging particularly in underrepresented populations, as pathogenic variant databases include studies mainly from European/Caucasian populations...
September 24, 2020: Molecular Genetics & Genomic Medicine
Sindhu Chadalawada, Stefan Sillau, Solana Archuleta, William Mundo, Mehdi Bandali, Gabriel Parra-Henao, Alfonso J Rodriguez-Morales, Wilmer E Villamil-Gomez, José Antonio Suárez, Leland Shapiro, Peter J Hotez, Laila Woc-Colburn, Kristen DeSanto, Anis Rassi, Carlos Franco-Paredes, Andrés F Henao-Martínez
Importance: Chagas cardiomyopathy is associated with substantial morbidity and mortality. Precise estimates of the risk of developing cardiomyopathy among patients with the acute or indeterminate chronic forms of Chagas disease are lacking. Objective: To estimate the risk of developing chronic cardiomyopathy in patients with acute and indeterminate chronic forms of Chagas disease. Data Sources: A systematic search in the Cochrane Library, Embase, Latin American and Caribbean Health Sciences Literature (LILACS), Medline, and Web of Science Core Collection databases was conducted from October 8 to October 24, 2018...
August 3, 2020: JAMA Network Open
Qiqing Sun, Jun Guo, Chanjuan Hao, Ruolan Guo, Xuyun Hu, Yuanying Chen, Weili Yang, Wei Li, Yingjun Feng
Importance: Pathogenic variants in the RBM20 gene are associated with aggressive dilated cardiomyopathy (DCM). Recently, RBM20 was found to be associated with left ventricular non-compaction cardiomyopathy (LVNC). Thus far, only five families with LVNC have been reported to carry variants in RBM20 . It remains unknown whether the variants in RBM20 associated with DCM can also cause LVNC. Objective: To elucidate the causative RBM20 variant in two unrelated patients with both LVNC and DCM, and to identify the clinical characteristics associated with variants in RBM20 ...
March 2020: Pediatric Investigation
Tiina Heliö, Perry Elliott, Juha W Koskenvuo, Juan R Gimeno, Luigi Tavazzi, Michal Tendera, Juan Pablo Kaski, Nicolas Mansencal, Zofia Bilińska, Gerry Carr-White, Thibaud Damy, Andrea Frustaci, Ingrid Kindermann, Tomas Ripoll-Vera, Jelena Čelutkienė, Anna Axelsson, Massimiliano Lorenzini, Aly Saad, Aldo P Maggioni, Cécile Laroche, Alida L P Caforio, Philippe Charron
AIMS: Cardiomyopathies comprise a heterogeneous group of diseases, often of genetic origin. We assessed the current practice of genetic counselling and testing in the prospective European Society of Cardiology EURObservational Research Programme Cardiomyopathy Registry. METHODS AND RESULTS: A total of 3208 adult patients from 69 centres in 18 countries were enrolled. Genetic counselling was performed in 60.8% of all patients [75.4% in hypertrophic cardiomyopathy (HCM), 39...
August 7, 2020: ESC Heart Failure
Panagiota Mitropoulou, Georgios Georgiopoulos, Stefano Figliozzi, Dimitrios Klettas, Flavia Nicoli, Pier Giorgio Masci
Heart failure (HF) is recognized as a leading cause of morbidity and mortality worldwide. Dilated cardiomyopathy (DCM) is a common phenotype in patients presenting with HF. Timely diagnosis, appropriate identification of the underlying cause, individualized risk stratification, and prediction of clinical response to treatment have improved the prognosis of DCM over the last few decades. In this article, we reviewed the current evidence on available imaging techniques used for DCM patients. In this direction, we evaluated appropriate scenarios for the implementation of echocardiography, nuclear imaging, and cardiac computed tomography, and we focused on the primordial role that cardiac magnetic resonance (CMR) holds in the diagnosis, prognosis, and tailoring of therapeutic options in this population of special clinical interest...
2020: Frontiers in Cardiovascular Medicine
M T A Wetscherek, W Rutschke, C Frank, C Stehning, P Lurz, M Grothoff, H Thiele, M Gutberlet, C Lücke
AIM: To investigate the observer agreement for the assessment of chronic myocarditis by native T1 and T2 relaxation times, post-contrast T1 relaxation time, and extracellular volume (ECV) fraction, compared to Lake Louise Criteria: oedema ratio (OR) and early gadolinium enhancement ratio (EGEr). MATERIALS AND METHODS: Data were collected retrospectively on 71 consecutive patients who underwent cardiac magnetic resonance imaging as part of a complete diagnostic work-up according to current guidelines for suspected myocarditis...
July 19, 2020: Clinical Radiology
Hyeonju Jeong, Shinjeong Song, Jiwon Seo, Iksung Cho, Geu-Ru Hong, Jong-Won Ha, Chi Young Shim
AIMS: Arrhythmogenic cardiomyopathy (AC) is characterized by right ventricular (RV) dilatation and dysfunction and is often seen in combination with tricuspid regurgitation (TR). The aim of this study was to investigate the characteristics and prognostic implications of TR in patients with AC. METHODS AND RESULTS: Clinical, echocardiographic, and cardiac magnetic resonance data of 52 patients with AC fulfilling 2010 Task Force criteria in a single centre were retrospectively evaluated...
July 22, 2020: ESC Heart Failure
Daniel J Hammersley, Brian P Halliday
PURPOSE OF REVIEW: Sudden cardiac death is recognised as a devastating consequence of non-ischaemic dilated cardiomyopathy. Although implantable cardiac defibrillators offer protection against some forms of sudden death, the identification of patients in this population most likely to benefit from this therapy remains challenging and controversial. In this review, we evaluate current guidelines and explore established and novel predictors of sudden cardiac death in patients with non-ischaemic dilated cardiomyopathy...
July 9, 2020: Current Cardiology Reports
Henrique Silveira Costa, Márcia Maria Oliveira Lima, Pedro Henrique Scheidt Figueiredo, Vanessa Pereira Lima, Matheus Ribeiro Ávila, Kenia Kiefer Parreiras de Menezes, Vanessa Amaral Mendonça, Ana Cristina Rodrigues Lacerda, Maria Carmo Pereira Nunes, Mauro Felippe Felix Mediano, Manoel Otávio da Costa Rocha
Patients with Chagas cardiomyopathy (ChC) usually progress with fatigue and dyspnea. Exercise tests are valuable for the functional evaluation of these patients. However, information about the applicability of the exercise tests is scattered, and no studies have systematically reviewed the results. Thus, the present review explored the general aspects and prognostic value of exercise tests in patients with ChC. A literature search of the MEDLINE, Web of Science, CINAHL, Scopus, and LILACS databases was performed to identify relevant studies...
2020: Revista da Sociedade Brasileira de Medicina Tropical
I Dobe, N Manafe, N Majid, I Zimba, B Manuel, A Mocumbi
INTRODUCTION: With improved access to anti-retroviral therapy (ART) the focus of HIV treatment is changing to reducing chronic co-morbidities and their effects, but guidelines for HIV care in many African countries do not include screening for cardiac disease. Our study aimed to determine the pattern of cardiac abnormalities in HIV-positive patients on ART. METHODS: We implemented a prospective, observational study for 24 months on a random sample of adult patients seen at a dedicated HIV clinic in Mozambique...
June 19, 2020: Cardiovascular Journal of Africa
Roberto Barriales-Villa, Juan P Ochoa, José M Larrañaga-Moreira, Joel Salazar-Mendiguchía, Carles Díez-López, María Alejandra Restrepo-Córdoba, Jorge Álvarez-Rubio, Ainhoa Robles-Mezcua, María C Olmo-Conesa, Elisa Nicolás-Rocamora, Jorge Sanz, Eduardo Villacorta, María Gallego-Delgado, Raquel Yotti, María Ángeles Espinosa, Ana Manovel, Luis M Rincón-Díaz, Juan Jiménez-Jaimez, Francisco J Bermúdez-Jiménez, M Teresa Basurte-Elorz, Vicente Climent-Payá, María I García-Álvarez, José Fernando Rodríguez-Palomares, Javier Limeres-Freire, Ainhoa Pérez-Guerrero, Eva M Cantero-Pérez, María L Peña-Peña, Julián Palomino-Doza, María G Crespo-Leiro, José M García-Pinilla, Esther Zorio, Tomás Ripoll-Vera, Pablo García-Pavía, Martín Ortiz-Genga, Lorenzo Monserrat
INTRODUCTION AND OBJECTIVES: According to sudden cardiac death guidelines, an implantable cardioverter-defibrillator (ICD) should be considered in patients with LMNA-related dilated cardiomyopathy (DCM) and ≥ 2 risk factors: male sex, left ventricular ejection fraction (LVEF) <45%, nonsustained ventricular tachycardia (NSVT), and nonmissense genetic variants. In this study we aimed to describe the clinical characteristics of carriers of LMNA genetic variants among individuals from a Spanish cardiac-laminopathies cohort (REDLAMINA registry) and to assess previously reported risk criteria...
June 29, 2020: Revista Española de Cardiología
Giusy Sirico, Andrea Montisci, Francesco Secchi, Massimo Mantica
A significant number of sudden death (SD) is observed in myotonic dystrophy (DM1) despite pacemaker implantation and some consider the ICD to be the preferential device in patients with conduction disease. According to the latest guidelines, prophylactic ICD implantation in patients with neuromuscular disorder should follow the same recommendations of non-ischemic dilated cardiomyopathy, being reasonable when pacing is needed. We here report a case of DM1 patient who underwent ICD implantation even in the absence of conduction disturbances on ECG and ventricular dysfunction/fibrosis at cardiac magnetic resonance...
March 2020: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
G Bazoukis, A Saplaouras, K P Letsas, C Yeung, S Xydonas, N Karamichalakis, C Thomopoulos, D Manolatos, G Papathanakos, K Vlachos, G Tse, P Korantzopoulos, M Efremidis, A Sideris, K K Naka
BACKGROUND: Cardiac resynchronization therapy (CRT) is an established therapeutic option for patients with heart failure (HF) and left ventricular ejection fraction (LVEF) ≤35 % who meet specific criteria according to current guidelines. However, up to 40 % of patients have no response to CRT. Our study aimed to investigate the association between different hematological and biochemical indices and response to CRT. METHODS: Patients with HF due to ischemic or dilated cardiomyopathy referred to our hospital for CRT implantation from January 2013 to November 2017 were included in the study...
July 2019: Hippokratia
Érico Santos, Luiz Menezes Falcão
Chagas disease is among the neglected tropical diseases recognized by the World Health Organization that have received insufficient attention from governments and health agencies. Chagas disease is endemic in 21 Latin America regions. Due to globalization and increased migration, it has crossed borders and reached other regions including North America and Europe. The clinical presentation of the disease is highly variable, from general symptoms to severe cardiac involvement that can culminate in heart failure...
June 9, 2020: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
A Akilzhanova, C Guelly, Z Abilova, S Rakhimova, A Akhmetova, U Kairov, U Kozhamkulov, O Nuralinov, A Abdrakhmanov, S Akilzhanova, S Trajanoski, M Bekbosynova, Z Zhumadilov
PURPOSE: Ventricular fibrillation (VF) and ventricular tachycardia (VT) are major causes of sudden cardiac death. In some cases clinical investigations fail to identify the underlying cause and the event is classified as idiopathic (iVT). Recent achievements in molecular genetics has unveiled the complex molecular basis of many cardiac conditions, paving the way for routine use of gene testing in clinical practice. In current practice, genetic testing can be used in a clinically affected patient to confirm diagnosis, or to formulate a differential diagnosis among overlapping phenotypes or between hereditary and acquired (nongenetic) forms of disease...
April 2020: Journal of Heart and Lung Transplantation
T G Moscarello, Y Moayedi, C Caleshu, K Khush, S Purewal, J Teuteberg
PURPOSE: Approximately half of patients undergoing heart transplant (HTX) have a primary cardiomyopathy. There is growing evidence for a genetic basis in many primary cardiomyopathies, with HFSA guidelines recommending genetics evaluations for many of these. We sought to assess the frequency of genetic disease in a single HTX center. METHODS: Chart review of consecutive patients scheduled in the post-HTX clinic from 6/2019 to 09/2019. Patients were divided into four categories of probability of genetic etiology based upon their pre-HTX diagnosis and family history, per their cardiologist (Figure 1)...
April 2020: Journal of Heart and Lung Transplantation
Daniel Quiat, Leora Witkowski, Hana Zouk, Kevin P Daly, Amy E Roberts
Background Genetic testing in pediatric primary dilated cardiomyopathy (DCM) patients has identified numerous disease-causing variants, but few studies have evaluated genetic testing outcomes in this population in the context of patient and familial clinical data or assessed the clinical implications of temporal changes in genetic testing results. Methods and Results We performed a retrospective analysis of all patients with primary DCM who presented to our institution between 2008 and 2018. Variants identified by genetic testing were reevaluated for pathogenicity on the basis of current guidelines for variant classification...
May 27, 2020: Journal of the American Heart Association
Julian Hoevelmann, Lina Hähnle, Julia Hähnle, Karen Sliwa, Charle Viljoen
Peripartum cardiomyopathy (PPCM) is an idiopathic dilated cardiomyopathy, in which previously healthy women present with heart failure secondary to left ventricular (LV) systolic dysfunction during the last months of pregnancy or up to 5 months postpartum. PPCM occurs worldwide. The incidence seems to be increasing, possibly due to increasing awareness of the condition and diagnosis thereof. Women diagnosed with PPCM present with symptoms and signs of heart failure, thromboembolism or arrhythmia. Although the incidence of arrhythmias in this condition is not well documented, patients with PPCM often have rhythm disturbances...
April 2020: Cardiovascular Diagnosis and Therapy
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