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hypertrophic heart

Frank Spillmann, Uwe Kühl, Sophie Van Linthout, Fernando Dominguez, Felicitas Escher, Heinz-Peter Schultheiss, Burkert Pieske, Carsten Tschöpe
We report the case of a 17-year-old female patient with known hypertrophic cardiomyopathy and a Wolff-Parkinson-White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Non-invasive and invasive examinations including magnetic resonance imaging, electrophysiological examinations, and angiography did not lead to a conclusive diagnosis...
June 2016: ESC Heart Failure
Abhijeet B Shelke, Rajeev Menon, Anuj Kapadiya, Sachin Yalagudri, Daljeet Saggu, Sandeep Nair, C Narasimhan
OBJECTIVE: Alcohol septal ablation (ASA) is a therapeutic alternative to surgical myectomy in patients with hypertrophic obstructive cardiomyopathy (HOCM). However, the anatomical variability of the septal branch, risk of complete heart block, and late onset ventricular arrhythmias are limitations to its therapeutic usage. There is recent interest in the use of radiofrequency catheter ablation (RFCA) as a therapeutic option in HOCM. We aimed to assess the safety and efficacy of RFCA in the treatment of symptomatic HOCM...
September 2016: Indian Heart Journal
Aindrila Chatterjee, Janine Seyfferth, Jacopo Lucci, Ralf Gilsbach, Sebastian Preissl, Lena Böttinger, Christoph U Mårtensson, Amol Panhale, Thomas Stehle, Oliver Kretz, Abdullah H Sahyoun, Sergiy Avilov, Stefan Eimer, Lutz Hein, Nikolaus Pfanner, Thomas Becker, Asifa Akhtar
A functional crosstalk between epigenetic regulators and metabolic control could provide a mechanism to adapt cellular responses to environmental cues. We report that the well-known nuclear MYST family acetyl transferase MOF and a subset of its non-specific lethal complex partners reside in mitochondria. MOF regulates oxidative phosphorylation by controlling expression of respiratory genes from both nuclear and mtDNA in aerobically respiring cells. MOF binds mtDNA, and this binding is dependent on KANSL3. The mitochondrial pool of MOF, but not a catalytically deficient mutant, rescues respiratory and mtDNA transcriptional defects triggered by the absence of MOF...
October 20, 2016: Cell
Arne O Melleby, Mari E Strand, Andreas Romaine, Kate M Herum, Biljana Skrbic, Christen P Dahl, Ivar Sjaastad, Arnt E Fiane, Jorge Filmus, Geir Christensen, Ida G Lunde
Pressure overload is a frequent cause of heart failure. Heart failure affects millions of patients worldwide and is a major cause of morbidity and mortality. Cell surface proteoglycans are emerging as molecular players in cardiac remodeling, and increased knowledge about their regulation and function is needed for improved understanding of cardiac pathogenesis. Here we investigated glypicans (GPC1-6), a family of evolutionary conserved heparan sulfate proteoglycans anchored to the extracellular leaflet of the cell membrane, in experimental and clinical heart failure, and explored the function of glypican-6 in cardiac cells in vitro...
2016: PloS One
Irfan Sahin, Baris Gungor, Berk Ozkaynak, Fatih Uzun, Suat Hayri Küçük, Ilhan Iker Avci, Ender Ozal, Burak Ayça, Sukru Cetın, Ertugrul Okuyan, Mustafa Hakan Dinckal
BACKGROUND: Correlation of increased copeptin levels with various cardiovascular diseases has been described. The clinical use of copeptin levels in patients with hypertrophic cardiomyopathy (HCM) has not been investigated before. HYPOTHESIS: In this study, we aimed to investigate the prognostic value of copeptin levels in patients with hypertrophic cardiomyopathy (HCM). METHODS: HCM was defined as presence of left ventricular wall thickness ≥15 mm in a subject without any concomitant disease that may cause left ventricular hypertrophy...
October 21, 2016: Clinical Cardiology
Alexander Bernt, Ashraf Y Rangrez, Matthias Eden, Andreas Jungmann, Sylvia Katz, Claudia Rohr, Oliver J Müller, Hugo A Katus, Samuel T Sossalla, Tatjana Williams, Oliver Ritter, Derk Frank, Norbert Frey
The objective of this study was to identify unknown modulators of Calcineurin (Cn)-NFAT signaling. Measurement of NFAT reporter driven luciferase activity was therefore utilized to screen a human cardiac cDNA-library (~10(7) primary clones) in C2C12 cells through serial dilutions until single clones could be identified. This extensive screening strategy culminated in the identification of SUMO2 as a most efficient Cn-NFAT activator. SUMO2-mediated activation of Cn-NFAT signaling in cardiomyocytes translated into a hypertrophic phenotype...
October 21, 2016: Scientific Reports
Christopher Semsarian, Jodie Ingles
Sudden cardiac death (SCD) is a rare but devastating complication of a number of underlying cardiovascular diseases. While coronary artery disease and acute myocardial infarction are the most common causes of SCD in older populations, inherited cardiac disorders comprise a substantial proportion of SCD cases aged less than 40 years. Inherited cardiac disorders include primary inherited arrhythmogenic disorders such as familial long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and inherited cardiomyopathies, most commonly hypertrophic cardiomyopathy (HCM)...
October 2016: Journal of Arrhythmia
Francisco J Rios, Katie Y Hood, A Karvey, Karla B Neves, Panagiota Anyfanti, Ryszard Nosalski, Livia L Camargo Ll, Augusto C Montezano, Rhian M Touyz
OBJECTIVE: TRPM7 is cation channel with intrinsic kinase activity important for cellular Mg homeostasis. We recently showed that TRPM7-kinase plays a role in aldosterone-mediated vascular effects and inflammation. Here we explored the putative role of TRPM7-kinase in cardiac fibrosis and vascular function in aldosterone-induced hypertension in mice. DESIGN AND METHOD: Wild-type (WT) or heterozygote TRPM7-kinase domain (TRPM7+/-) mice were treated with infused aldosterone (600 μg/Kg/day) and NaCl 1% in drinking water (aldo/salt) for 4 weeks...
September 2016: Journal of Hypertension
Hemant Chaturvedi, Rudra Dev Pandey, Krishna Kumar Sharma, Jitendra Singh Makkar, Sanjeev K Sharma
We present a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who recently developed cardiac arrhythmias, and shortly discuss the diagnostic modalities, differential diagnosis, and treatment strategy for this condition. AHCM is a rare form of hypertrophic cardiomyopathy, which usually involves the apex of the left ventricle. AHCM can occur with varied presentations such as chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation, and congestive heart failure...
September 2016: Indian Heart Journal
Barry J Maron, Ethan J Rowin, Martin S Maron, Eugene Braunwald
Hypertrophic cardiomyopathy was first recognized as a disease of obstruction to left ventricular outflow, hence its early names and acronyms such as idiopathic hypertrophic subaortic obstruction. The nonobstructive subset of patients, incapable of developing mechanical impedance to left ventricular outflow at rest or with physiologic exercise, was initially recognized by the Braunwald group at the National Institutes of Health >50 years ago in the pre-imaging era and is now recognized as comprising about one-third of hypertrophic cardiomyopathy patients...
October 13, 2016: American Journal of Medicine
Yoshifumi Itoda, Kan Nawata, Haruo Yamauchi, Osamu Kinoshita, Mitsutoshi Kimura, Minoru Ono
Aortic insufficiency (AI) is a significant complication of long-term support of continuous flow left ventricular assist device (CF-LVAD) for patients with end-stage heart failure. A 26-year-old female with osteogenesis imperfecta (OI) was diagnosed with dilated phase hypertrophic cardiomyopathy (d-HCM)) and was implanted with Jarvik 2000, for bridge to transplantation. AI gradually developed and surgical intervention was indicated. We performed central aortic valve closure (CAVC) instead of valve replacement 20 months after CF-LVAD implantation...
October 15, 2016: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
Santosh Kumar Sinha, Shalini Garg, Ramesh Thakur, Vinay Krishna, Karandeep Singh, Mohit Sachan, Amit Goel, Mahamdula Razi, Umeshwar Pandey, Chandra Mohan Varma
BACKGROUND: The optimal timing of surgery in patients with chronic organic severe mitral regurgitation (MR) continues to be debated, especially for those who are asymptomatic. The aim of the study was to determine independent and additive prognostic value of exercise brain natriuretic peptide (eBNP) in patients with severe asymptomatic MR and normal left ventricular ejection fraction (LVEF). METHODS: Two hundred twenty-three consecutive patients with severe MR defined by effective regurgitant orifice (ERO) area ≥ 40 mm(2) and/or residual volume ≥ 60 mL, LVEF > 60%, and normal LV end-systolic diameter < 40 mm underwent symptom limited exercise treadmill test (TMT)...
November 2016: Journal of Clinical Medicine Research
Andrzej Wojtarowicz, Zdzisława Kornacewicz-Jach
BACKGROUND: Alcohol septal ablation (ASA) is a method of treatment in obstructive hypertrophic cardiomyopathy (HOCM), but there is little data on the long-term results of ASA and the natural course after treatment. The aim of the study was to evaluate the results of ASA in HOCM in multiannual observation, and its impact on patient survival, exercise capacity, electrical complications, and changes in the anatomy and function of the heart. METHODS: The study evaluated 47 patients with HOCM with a high left ventricular outflow tract (LVOT gradient) treated between 1997 and 2014 with ASA...
October 13, 2016: Cardiology Journal
Christian Faul
Fibroblast growth factors (FGF) are mitogenic signal mediators that induce cell proliferation and survival. Although cardiac myocytes are post-mitotic, they have been shown to be able to respond to local and circulating FGFs. While precise molecular mechanisms are not well characterized, some FGF family members have been shown to induce cardiac remodeling under physiologic conditions by mediating hypertrophic growth in cardiac myocytes and by promoting angiogenesis, both events leading to increased cardiac function and output...
October 7, 2016: Bone
Lihua Qi, Yu Yu, Xiaochun Chi, Danyu Lu, Yao Song, Youyi Zhang, Hongquan Zhang
Kindlin-2, a member of the Kindlin family focal adhesion proteins, plays an important role in cardiac development. It is known that defects in the Z-disc proteins lead to hypertrophic cardiomyopathy (HCM) or dilated cardiomyopathy (DCM). Our previous investigation showed that Kindlin-2 is mainly localized at the Z-disc and depletion of Kindlin-2 disrupts the structure of the Z-Disc. Here, we reported that depletion of Kindlin-2 leads to the disordered myocardial fibers, fractured and vacuolar degeneration in myocardial fibers...
September 20, 2016: Science China. Life Sciences
Ah Young Kim, Se Yong Jung, Jae Young Choi, Gi Beom Kim, Young-Hwue Kim, Woo Sup Shim, I-Seok Kang, Jo Won Jung
BACKGROUND AND OBJECTIVES: We conducted a review of current data on respiratory syncytial virus (RSV) prophylaxis with palivizumab, in Korean children with congenital heart diseases (CHD). In 2009, the Korean guideline for RSV prophylaxis had established up to five shots monthly per RSV season, only for children <1 year of age with hemodynamic significance CHD (HS-CHD). SUBJECTS AND METHODS: During the RSV seasons in 2009-2015, we performed a retrospective review of data for 466 infants with CHD, examined at six centers in Korea...
September 2016: Korean Circulation Journal
Mayra de A Marques, Guilherme A P de Oliveira
Inherited myopathies affect both skeletal and cardiac muscle and are commonly associated with genetic dysfunctions, leading to the production of anomalous proteins. In cardiomyopathies, mutations frequently occur in sarcomeric genes, but the cause-effect scenario between genetic alterations and pathological processes remains elusive. Hypertrophic cardiomyopathy (HCM) was the first cardiac disease associated with a genetic background. Since the discovery of the first mutation in the β-myosin heavy chain, more than 1400 new mutations in 11 sarcomeric genes have been reported, awarding HCM the title of the "disease of the sarcomere...
2016: Frontiers in Physiology
Wei-Ming Li, Yi-Fan Zhao, Guo-Fu Zhu, Wen-Hui Peng, Meng-Yun Zhu, Xue-Jing Yu, Wei Chen, Da-Chun Xu, Ya-Wei Xu
BACKGROUND: Pathological cardiac hypertrophy is an independent risk factor of heart failure. However, we still lack effective methods to reverse cardiac hypertrophy. DUSP12 is a member of the DUSP family, which is characterized by its dual specific phosphatase activity to dephosphorylate both tyrosine and serine/threonine residues on one substrate. Some DUSPs have been identified as being involved in the regulation of cardiac hypertrophy. However, the role of DUSP12 during pathological cardiac hypertrophy is still unclear...
October 4, 2016: Clinical Science (1979-)
U Smith, B B Kahn
Obesity, the major cause of the current global epidemic of type 2 diabetes (T2D), induces insulin resistance in peripheral insulin target tissues. Several mechanisms have been identified related to cross-talk between adipose tissue, skeletal muscle and liver. These mechanisms involve both increased free fatty acid release and altered secretion of adipokines from adipose tissue. A major determinant of metabolic health is the ability of subcutaneous adipose tissue (SAT) to store excess fat rather than allowing it to accumulate in ectopic depots including liver (i...
October 3, 2016: Journal of Internal Medicine
Shizhao Xiang, Ning Zhang, Zheng Yang, Zhouyan Bian, Yuan Yuan, Qizhu Tang
Bisoprolol is a drug that acts via the mechanism of specifically and selectively inhibiting the β1-adrenoreceptor in cardiac myocytes, and provides a pure reduction of heart rate without changing other cardiac parameters. It has long been clinically used to treat cerebrovascular and cardiovascular illnesses. However, there is little information available on whether the role of bisoprolol in the attenuation of ventricular remodeling is dependent upon the achievement of a target dose, and whether it must be used as a preferred option...
October 2016: Experimental and Therapeutic Medicine
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