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Hypertrophy cardiomyopathy

Ali J Marian, Yanli Tan, Lili Li, Jeffrey T Chang, Petros Syrris, Manouchehr Hessabi, Mohammad H Rahbar, James T Willerson, Benjamin Y Cheong, Chia-Ying Liu, Neal S Kleiman, David A Bluemke, Sherif F Nagueh
<u>Rationale:</u> Hypertrophic cardiomyopathy (HCM) is a genetic paradigm of cardiac hypertrophy. Cardiac hypertrophy and interstitial fibrosis are important risk factors for sudden death and morbidity in HCM. Oxidative stress is implicated in the pathogenesis of cardiac hypertrophy and fibrosis. Treatment with anti-oxidant N-acetylcysteine (NAC) reverses cardiac hypertrophy and fibrosis in animal models of HCM. <u>Objective:</u> To determine effect sizes of NAC on indices of cardiac hypertrophy and fibrosis in patients with established HCM...
March 14, 2018: Circulation Research
Gabriela Miana de Mattos Paixão, Horácio Eduardo Veronesi, Halsted Alarcão Gomes Pereira da Silva, José Nunes de Alencar Neto, Carolina de Paulo Maldi, Luciano de Figueiredo Aguiar Filho, Ibrahim Masciarelli Francisco Pinto, Francisco Faustino de Albuquerque Carneiro de França, Edileide de Barros Correia
BACKGROUND: Electrocardiogram is the initial test in the investigation of heart disease. Electrocardiographic changes in hypertrophic cardiomyopathy have no set pattern, and correlates poorly with echocardiographic findings. Cardiac magnetic resonance imaging has been gaining momentum for better assessment of hypertrophy, as well as the detection of myocardial fibrosis. OBJECTIVES: To correlate the electrocardiographic changes with the location of hypertrophy in hypertrophic cardiomyopathy by cardiac magnetic resonance...
January 2018: Arquivos Brasileiros de Cardiologia
Shuyi Wang, Cong Wang, Subat Turdi, Kacy L Richmond, Yingmei Zhang, Jun Ren
BACKGROUND AND AIMS: Uncorrected obesity contributes to cardiac remodeling and contractile dysfunction although the underlying mechanism remains poorly understood. Mitochondrial aldehyde dehydrogenase (ALDH2) is a mitochondrial enzyme with some promises in a number of cardiovascular diseases. This study was designed to evaluate the impact of ALDH2 on cardiac remodeling and contractile property in high fat diet-induced obesity. METHODS: Wild-type (WT) and ALDH2 transgenic mice were fed low (10% calorie from fat) or high (45% calorie from fat) fat diet for 5 months prior to the assessment of cardiac geometry and function using echocardiography, IonOptix system, Lectin, and Masson Trichrome staining...
March 13, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
Patrício Aguiar, Olga Azevedo, Rui Pinto, Jacira Marino, Carlos Cardoso, Nuno Sousa, Damião Cunha, Derralynn Hughes, José Luís Ducla Soares
BACKGROUND: Cardiomyopathy is a major determinant of overall Fabry disease (FD) prognosis, with the worst outcomes in patients with myocardial fibrosis. Late gadolinium enhancement is currently the gold standard for evaluation of replacement myocardial fibrosis; however, this event is irreversible, thus identification of biomarkers of earlier diffuse fibrosis is paramount. METHODS AND RESULTS: Type I collagen synthesis and degradation biomarkers (PICP [carboxyterminal propeptide of procollagen type I], ICTP [carboxyterminal telopeptide of type I collagen], and MMP1 [matrix metalloproteinase 1] and MMP2) and markers of bone synthesis and degradation were evaluated (to adjust type I collagen metabolism to bone turnover) in FD patients and controls...
March 13, 2018: Journal of the American Heart Association
Konstantin V Borisov
BACKGROUND: In patients with hypertrophic obstructive cardiomyopathy myocardial fibrosis is an independent predictor of adverse outcome. A new technique of HOCM surgical correction in patients with severe hypertrophy and septal myocardial fibrosis has been proposed. METHODS: The excision of the asymmetrical hypertrophied area of the interventricular septum causing obstruction was performed from the conal part of the right ventricle corresponding to the zone of obstruction of the left ventricle (LV)...
March 10, 2018: Annals of Thoracic Surgery
Mustafa Dawood, Noman Lateef, Abubakar Tauseef, Janki Patel
Our article refers to a 50-year-old woman with previously diagnosed rheumatoid arthritis (RA) who presented with symptoms of dyspnea on exertion and dizziness. An echocardiogram revealed a 17-mm asymmetric interventricular septum hypertrophy and systolic anterior motion of the anterior mitral valve leaflet. Association of hypertrophic obstructive cardiomyopathy (HCM) with connective tissue diseases has been well documented. For RA the human leukocyte antigen (HLA) system, particularly HLA-DR4, may possibly be a link between the two entities, as it is associated with both RA and HCM...
January 5, 2018: Curēus
Stephen A McCullough, Michael A Fifer, Pouya Mohajer, Patricia A Lowry, Caitlin O'Callaghan Reen, Aaron L Baggish, Gus J Vlahakes, Yuichi J Shimada
BACKGROUND: The clinical characteristics associated with elevated right atrial pressure (RAP) in hypertrophic cardiomyopathy (HCM) are unknown. Few data exist as to whether elevated RAP has prognostic implications in patients with HCM. This study investigated the clinical correlates and prognostic value of elevated RAP in HCM.Methods and Results:This retrospective cohort study was performed on 180 patients with HCM who underwent right heart catheterization between 1997 and 2014. Elevated RAP was defined as >8 mmHg...
March 9, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Akiomi Yoshihisa, Yusuke Kimishima, Takatoyo Kiko, Yu Sato, Shunsuke Watanabe, Yuki Kanno, Satoshi Abe, Makiko Miyata, Takamasa Sato, Satoshi Suzuki, Masayoshi Oikawa, Atsushi Kobayashi, Takayoshi Yamaki, Hiroyuki Kunii, Kazuhiko Nakazato, Takafumi Ishida, Yasuchika Takeishi
Titin is associated with myocardial stiffness and hypertrophy, and mutations in its gene have been identified in cardiac myopathies such as dilated cardiomyopathy (DC). It has recently been reported that in damaged muscle, the N-terminal fragment of titin (Titin-N) is cleaved by calpain-3, and urinary Titin-N (U-TN) could be a marker of sarcomere damage. We aimed to investigate the impact of U-TN on prognosis of DC. We measured urinary levels of Titin-N/creatinine ratio (U-TN/Cr; pmol/mg/dl) in 102 patients with DC, and followed up all the patients (mean 1,167 days)...
February 12, 2018: American Journal of Cardiology
Szu-Ying Tsai, Shan-Ying Wang, Yu-Chien Shiau, Yen-Wen Wu
Hypertrophic cardiomyopathy (HCM) is an often under-diagnosed cause of left ventricular hypertrophy (LVH). It affects 1/500 of the population, is the most commonly inherited cardiovascular disorder, and can present in apical, concentric, or septal forms. Although most patients are asymptomatic, sudden cardiac death can be the initial presentation of HCM. By retrospectively enrolling patients suspected of having three different types of HCM in the absence of epicardial coronary stenosis, we aimed to examine systolic and diastolic dysfunction and perfusion abnormalities using both Doppler echocardiography and state-of-the-art gated single-photon emission computerized tomography (SPECT) myocardial perfusion imaging (MPI) with a cadmium-zinc-telluride camera and thallium-201...
March 8, 2018: Scientific Reports
C Rupérez, C Lerin, G Ferrer-Curriu, M Cairo, A Mas-Stachurska, M Sitges, J Villarroya, M Giralt, F Villarroya, A Planavila
OBJECTIVE: High-fat diet-induced obesity leads to the development of hypertrophy and heart failure through poorly understood molecular mechanisms. We have recently shown that fibroblast growth factor-21 (FGF21) is produced by the heart and exerts protective effects that prevent cardiac hypertrophy development and oxidative stress. The aim of this study was to determine the effects of FGF21 on the cardiomyopathy associated with obesity development. RESULTS: Fgf21-/- mice showed an enhanced increase in the heart weight/tibia length (HW/TL) ratio in response to the high-fat diet...
March 5, 2018: International Journal of Cardiology
Ales Linhart, Franco Cecchi
Left ventricular hypertrophy may be a consequence of a hemodynamic overload or a manifestation of several diseases affecting different structural and functional proteins of cardiomyocytes. Among these, sarcomeric hypertrophic cardiomyopathy (HCM) represents the most frequent cause. In addition, several metabolic diseases lead to myocardial thickening, either due to intracellular storage (glycogen storage and lysosomal diseases), extracellular deposition (TTR and AL amyloidosis) or due to abnormal energy metabolism (mitochondrial diseases)...
April 15, 2018: International Journal of Cardiology
Giuseppe Cannavale, Marco Francone, Nicola Galea, Francesco Vullo, Antonio Molisso, Iacopo Carbone, Carlo Catalano
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...
2018: BioMed Research International
Robert G Nampiaparampil, Daniel G Swistel, Michael Schlame, Muhamed Saric, Mark V Sherrid
Transesophageal echocardiography is essential in guiding the surgical approach for patients with obstructive hypertrophic cardiomyopathy. Septal hypertrophy, elongated mitral valve leaflets, and abnormalities of the subvalvular apparatus are prominent features, all of which may contribute to left ventricular outflow tract obstruction. Surgery aims to alleviate the obstruction via an extended myectomy, often with an intervention on the mitral valve and subvalvular apparatus. The goal of intraoperative echocardiography is to assess the anatomic pathology and pathophysiology in order to achieve a safe intraoperative course and a successful repair...
March 2018: Journal of the American Society of Echocardiography
Gianluca Di Bella, Fausto Pizzino, Rocco Donato, Dalia Di Nunzio, Cesare de Gregorio
Cardiomyopathies (Cs) are a heterogeneous group of myocardial diseases with structural and/or functional abnormalities.The aetiology is due to genetic-family substrate in most cases, however, the correct and detailed analysis of morphofunctional abnormalities (severity and distribution of hypertrophy, ventricular dilatation, ventricular dysfunction) and tissue characteristics (myocardial fibrosis, myocardial infiltration) are a crucial element for a definite diagnosis.Among the different diagnostic imaging modalities applied in clinical practice (echocardiography, nuclear medicine), cardiac magnetic resonance (CMR) has emerged as a non-invasive diagnostic tool having high ability to quantify systolic function and tissue abnormalities that represent the substrates of many Cs...
March 2, 2018: Advances in Experimental Medicine and Biology
Peta M A Alexander, Alan W Nugent, Piers E F Daubeney, Katherine J Lee, Lynn A Sleeper, Tibor Schuster, Christian Turner, Andrew M Davis, Chris Semsarian, Steven D Colan, Terry Robertson, James Ramsay, Robert Justo, Gary F Sholler, Ingrid King, Robert G Weintraub
Background -Late survival and symptomatic status of children with hypertrophic cardiomyopathy (HCM) have not been well defined. We examined long-term outcomes for pediatric HCM. Methods -The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end-point was time to death or cardiac transplantation. Results -There were 80 patients with HCM with median age at diagnosis of 0...
February 28, 2018: Circulation
Paul Wijnker, Vasco Sequeira, Diederik Kuster, Jolanda van der Velden
SIGNIFICANCE: Hypertrophic cardiomyopathy (HCM) is a cardiac genetic disease characterized by left ventricular hypertrophy, diastolic dysfunction and myocardial disarray. Disease onset occurs between 20 and 50 years of age, thus affecting patients in the prime of their life. HCM is caused by mutations in sarcomere proteins, the contractile building blocks of the heart. Despite increased knowledge of causal mutations, the exact path from genetic defect leading to cardiomyopathy is complex and involves additional disease hits...
February 28, 2018: Antioxidants & Redox Signaling
Birgül Kirel, Özkan Bozdağ, Pelin Köşger, Sultan Durmuş Aydoğdu, Eylem Alıncak, Neslihan Tekin
Donohue syndrome (Leprechaunism) is characterized by severe insulin resistance, hyperinsulinemia, postprandial hyperglycemia, preprandial hypoglycemia, intrauterine and postnatal growth retardation, dysmorphic findings, and clinical and laboratory findings of hyperandrogenemia due to homozygous or compound heterozygous inactivating mutations in the insulin receptor gene. A female newborn presented with lack of subcutaneous fat tissue, bilateral simian creases, hypertrichosis, especially on her face, gingival hypertrophy, cliteromegaly, and prominent nipples...
December 2017: Türk Pediatri Arşivi
Gherardo Finocchiaro, Harshil Dhutia, Andrew D'Silva, Aneil Malhotra, Nabeel Sheikh, Rajay Narain, Bode Ensam, Stathis Papatheodorou, Maite Tome, Rajan Sharma, Michael Papadakis, Sanjay Sharma
BACKGROUND: The association between athletic participation and alteration in diastolic function is not well established. The aims of this study were to determine the spectrum of Doppler parameters of left ventricular (LV) diastolic function in a large cohort of healthy athletes and to quantify the overlap between physiologic LV hypertrophy and hypertrophic cardiomyopathy (HCM). METHODS: A retrospective analysis of indices of LV diastolic function was performed in 1,510 healthy athletes (mean age, 22 ± 5 years; range, 13-33 years; 72% men)...
February 23, 2018: Journal of the American Society of Echocardiography
Arash Yavari, Dhruv Sarma, Eduardo B Sternick
In humans, dominant mutations in the gene encoding the regulatory γ2-subunit of AMP-activated protein kinase (PRKAG2) result in a highly penetrant phenotype dominated by cardiac features: left ventricular hypertrophy, ventricular pre-excitation, atrial tachyarrhythmia, cardiac conduction disease, and myocardial glycogen storage. The discovery of a link between the cell's fundamental energy sensor, AMPK, and inherited cardiac disease catalyzed intense interest into the biological role of AMPK in the heart. In this chapter, we provide an introduction to the spectrum of human disease resulting from pathogenic variants in PRKAG2, outlining its discovery, clinical genetics, and current perspectives on its pathogenesis and highlighting mechanistic insights derived through the evaluation of disease models...
2018: Methods in Molecular Biology
Shizuka Konishi, Takashi Kotera, Masaaki Koga, Makoto Ueda
The term cardiomyopathy is used to describe heart disease resulting from an abnormality in the myocardium. It is rare in cynomolgus macaques ( Macaca fascicularis ). Here, we report a case of hypertrophic cardiomyopathy in an 11-year-old male cynomolgus macaque. Macroscopically, the interventricular septum (IVS) and the left ventricular (LV) and right ventricular (RV) walls of the heart were thickened. Histologically, cardiomyocytes showed hypertrophy and disarray with interstitial fibrosis, and some myocytes showed karyomegaly and vacuoles...
January 2018: Journal of Toxicologic Pathology
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