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Hypertrophe cardiomyopathy

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https://www.readbyqxmd.com/read/30102484/hemolytic-uremic-syndrome-and-iga-nephropathy-in-a-child-coincidence-or-not
#1
Serra Sürmeli-Döven, Ali Delibaş, İclal Gürses, Uğur Raşit Kayacan, Banu Coşkun-Yılmaz, Kaan Esen, Emine Korkmaz, Fatih Özaltın
Sürmeli-Döven S, Delibaş A, Gürses İ, Kayacan UR, Coşkun-Yılmaz B, Esen K, Korkmaz E, Özaltın F. Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not? Turk J Pediatr 2018; 60: 81-85. A previously healthy 18-month old boy, presenting with diarrhea, anemia, thrombocytopenia and acute renal failure was admitted to our hospital. Hemolytic uremic syndrome (HUS) was diagnosed with his clinical and laboratory findings. His stool was negative for Shiga toxin producing E. coli (STEC)...
2018: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/30102150/prolonged-cross-bridge-binding-triggers-muscle-dysfunction-in-a-fly-model-of-myosin-based-hypertrophic-cardiomyopathy
#2
William A Kronert, Kaylyn M Bell, Meera C Viswanathan, Girish C Melkani, Adriana S Trujillo, Alice Huang, Anju Melkani, Anthony Cammarato, Douglas M Swank, Sanford I Bernstein
K146N is a dominant mutation in human β-cardiac myosin heavy chain, which causes hypertrophic cardiomyopathy. We examined how Drosophila muscle responds to this mutation and integratively analyzed the biochemical, physiological and mechanical foundations of the disease. ATPase assays, actin motility, and indirect flight muscle mechanics suggest at least two rate constants of the cross-bridge cycle are altered by the mutation: increased myosin attachment to actin and decreased detachment, yielding prolonged binding...
August 13, 2018: ELife
https://www.readbyqxmd.com/read/30101858/phenotypic-screening-using-patient-derived-induced-pluripotent-stem-cells-identified-pyr3-as-a-candidate-compound-for-the-treatment-of-infantile-hypertrophic-cardiomyopathy
#3
Taku Sakai, Atsuhiko T Naito, Yuki Kuramoto, Masamichi Ito, Katsuki Okada, Tomoaki Higo, Akito Nakagawa, Masato Shibamoto, Toshihiro Yamaguchi, Tomokazu Sumida, Seitaro Nomura, Akihiro Umezawa, Shigeru Miyagawa, Yoshiki Sawa, Hiroyuki Morita, Jong-Kook Lee, Ichiro Shiojima, Yasushi Sakata, Issei Komuro
Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by hypertrophy of the myocardium. Some of the patients are diagnosed for HCM during infancy, and the prognosis of infantile HCM is worse than general HCM. Nevertheless, pathophysiology of infantile HCM is less investigated and remains largely unknown. In the present study, we generated induced pluripotent stem cells (iPSCs) from two patients with infantile HCM: one with Noonan syndrome and the other with idiopathic HCM. We found that iPSC-derived cardiomyocytes (iPSC-CMs) from idiopathic HCM patient were significantly larger and showed higher diastolic intracellular calcium concentration compared with the iPSC-CMs from healthy subject...
August 11, 2018: International Heart Journal
https://www.readbyqxmd.com/read/30101852/chronic-pressure-overload-induces-cardiac-hypertrophy-and-fibrosis-via-increases-in-sglt1-and-il-18-gene-expression-in-mice
#4
Naoko Matsushita, Nanae Ishida, Miho Ibi, Maki Saito, Atsushi Sanbe, Hisashi Shimojo, Satoshi Suzuki, Hermann Koepsell, Yasuchika Takeishi, Yoshihiro Morino, Eiichi Taira, Yohei Sawa, Masamichi Hirose
Increased gene expression levels of sodium-glucose cotransporter 1 (SGLT1) are associated with hypertrophic and ischemic cardiomyopathy. However, it remains unclear whether chronic pressure overload increases SGLT1 expression, which in turn induces hypertrophic cardiomyopathy. We hypothesized that pressure overload could increase SGLT1 gene expression, leading to the development of hypertrophic cardiomyopathy.To create pressure overload-induced cardiomyopathy, transverse aortic constriction (TAC) was performed in SGLT1-deficient (SGLT1-/- ) and wild-type (WT) mice...
August 11, 2018: International Heart Journal
https://www.readbyqxmd.com/read/30100681/significant-left-ventricular-outflow-tract-obstruction-secondary-to-systolic-anterior-motion-in-a-patient-without-hypertrophic-cardiomyopathy-an-echocardiographic-study
#5
Hanan Alrammah, Sami Ghazal
Dynamic left ventricular outflow tract obstruction (LVOTO) can be hemodynamically significant and can adversely affect the heart and quality of life. It is caused by systolic anterior motion (SAM) of the anterior mitral valve into the LVOT. The mechanism underlying SAM has been an area of special interest. However, SAM occurrence in the absence of septal hypertrophy is exceedingly uncommon. Here we present a case of a young male patient who sought medical care with a complaint of exertional dyspnea, New York Heart Association functional Class 2-3, and was found to have SAM and severe LVOTO at rest without hypertrophic cardiomyopathy...
October 2018: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/30099631/unraveling-obscurins-in-heart-disease
#6
REVIEW
Alyssa Grogan, Aikaterini Kontrogianni-Konstantopoulos
Obscurins, expressed from the single OBSCN gene, are a family of giant, modular, cytoskeletal proteins that play key structural and regulatory roles in striated muscles. They were first implicated in the development of heart disease in 2007 when two missense mutations were found in a patient diagnosed with hypertrophic cardiomyopathy (HCM). Since then, the discovery of over a dozen missense, frameshift, and splicing mutations that are linked to various forms of cardiomyopathy, including HCM, dilated cardiomyopathy (DCM), and left ventricular non-compaction (LVNC), has highlighted OBSCN as a potential disease-causing gene...
August 11, 2018: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/30097789/the-effects-of-septal-myectomy-and-alcohol-septal-ablation-for-hypertrophic-cardiomyopathy-on-the-cardiac-conduction-system
#7
Patrick Fitzgerald, Fred Kusumoto
AV conduction abnormalities are observed in 15-30% of patients with hypertrophic cardiomyopathy but are usually not severe enough to require permanent pacemaker implant. Both septal myectomy and alcohol septal ablation are effective options to relieve symptoms due to left ventricular outflow tract gradient in patients with hypertrophic cardiomyopathy but have procedure-specific effects on AV conduction and the His Purkinje system. Septal myectomy is associated with the development of LBBB in 50-100% of patients, while alcohol septal ablation is associated with RBBB in 37-70% of patients...
August 10, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/30097628/author-correction-the-significance-of-interstitial-fibrosis-on-left-ventricular-function-in-hypertensive-versus-hypertrophic-cardiomyopathy
#8
Meng Jiang, Zi Wang, Xuan Su, Xingrong Gong, Jun Pu, Lianming Wu, Chang Liu, Qiuying Yao, Lingcong Kong, Jianrong Xu, Ben He
A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has been fixed in the paper.
August 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30093255/french-society-of-cardiology-guidelines-on-exercise-tests-part-2-indications-for-exercise-tests-in-cardiac-diseases
#9
Dany-Michel Marcadet, Bruno Pavy, Gilles Bosser, Frédérique Claudot, Sonia Corone, Hervé Douard, Marie-Christine Iliou, Bénédicte Vergès-Patois, Pascal Amedro, Thierry Le Tourneau, Caroline Cueff, Taniela Avedian, Alain Cohen Solal, François Carré
The exercise test is performed routinely in cardiology; its main indication is the diagnosis of myocardial ischemia, evaluated along with the subject's pretest probability and cardiovascular risk level. Other criteria, such as analysis of repolarization, must be taken into consideration during the interpretation of an exercise test, to improve its predictive value. An exercise test is also indicated for many other cardiac diseases (e.g. rhythm and conduction disorders, severe asymptomatic aortic stenosis, hypertrophic cardiomyopathy, peripheral artery disease, hypertension)...
August 6, 2018: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/30089560/have-we-reached-the-apex-catheter-ablation-in-hypertrophic-cardiomyopathy-and-apical-aneurysm
#10
EDITORIAL
Ammar M Killu, Samuel J Asirvatham
No abstract text is available yet for this article.
March 2018: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/30089559/radiofrequency-catheter-ablation-of-ventricular-tachycardia-in-patients-with-hypertrophic-cardiomyopathy-and-apical-aneurysm
#11
Miyako Igarashi, Akihiko Nogami, Kenji Kurosaki, Yuichi Hanaki, Yuki Komatsu, Seiji Fukamizu, Itsuro Morishima, Kazuaki Kaitani, Suguru Nishiuchi, Ahmed Karim Talib, Takeshi Machino, Kenji Kuroki, Hiro Yamasaki, Nobuyuki Murakoshi, Yukio Sekiguchi, Keisuke Kuga, Kazutaka Aonuma
OBJECTIVES: This study evaluated the characteristics and results of radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in patients with hypertrophic cardiomyopathy (HCM) and left ventricular apical aneurysm (AA). BACKGROUND: Monomorphic VT in patients with HCM and left ventricular AA has been reported. However, outcome data of RFCA are insufficient. METHODS: Fifteen patients with HCM and AA who underwent RFCA for VT at 5 different institutions were included in this study...
March 2018: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/30089499/extracellular-volume-fraction-measurements-derived-from-the-longitudinal-relaxation-of-blood-based-synthetic-hematocrit-may-lead-to-clinical-errors-in-3-t-cardiovascular-magnetic-resonance
#12
Yongning Shang, Xiaochun Zhang, Xiaoyue Zhou, Jian Wang
BACKGROUND: The extracellular volume (ECV), derived from cardiovascular magnetic resonance (CMR) T1 mapping, is a biomarker of the extracellular space in the myocardium. The hematocrit (HCT), measured from venipuncture, is required for ECV measurement. We test the clinic values of synthetic ECV, which is derived from the longitudinal relaxation of blood-based (T1blood ) synthetic hematocrit in 3 T CMR. METHODS: A total of 226 subjects with CMR T1 mapping and HCT measurement taken on the same day as the CMR were retrospectively enrolled and randomly split into derivation (n = 121) and validation (n = 105) groups, comprising healthy subjects (n = 45), type 2 diabetes mellitus (T2DM) patients (n = 60), hypertrophic cardiomyopathy (HCM) patients (n = 93), and 28 other patients...
August 9, 2018: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/30087038/clinical-and-prognostic-impact-of-chronotropic-incompetence-in-patients-with-hypertrophic-cardiomyopathy
#13
Damiano Magri, Piergiuseppe Agostoni, Gianfranco Sinagra, Federica Re, Michele Correale, Giuseppe Limongelli, Elisabetta Zachara, Vittoria Mastromarino, Caterina Santolamazza, Matteo Casenghi, Giuseppe Pacileo, Fabio Valente, Marco Morosin, Beatrice Musumeci, Erika Pagannone, Antonello Maruotti, Massimo Uguccioni, Massimo Volpe, Camillo Autore
BACKGROUND: A blunted heart rate (HR) response is associated with an impaired peak oxygen uptake (pVO2 ), a powerful outcome predictor in hypertrophic cardiomyopathy (HCM). The present multicenter study sought to determine the prognostic role for exercise-induced HR response in HCM. METHODS: A total of 681 consecutive HCM outpatients on optimized treatment were recruited. The heart failure (HF) end-point was death due to HF, cardiac transplantation, NYHA III-IV class progression, HF worsening leading to hospitalization and severe functional deterioration leading to septal reduction...
August 4, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/30085025/relation-of-regional-myocardial-structure-and-function-in-hypertrophic-cardiomyopathy-and-amyloidois-a-combined-two-dimensional-speckle-tracking-and-cardiovascular-magnetic-resonance-analysis
#14
Efstathios D Pagourelias, Oana Mirea, Georgios Vovas, Jürgen Duchenne, Blazej Michalski, Johan Van Cleemput, Jan Bogaert, Vasilios P Vassilikos, Jens-Uwe Voigt
Aims: We aimed at investigating the relationship between segmental morphology [wall thickness (WT) and WT location (LT: base-mid-apex)], loading conditions and underlying pathological substrate [histology (H), hypertrophy vs. infiltration] with segmental longitudinal (SLS) and circumferential (SCS) strain in a group of patients with hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA). Methods and results: We included 30 patients with biopsy-proven CA (65...
August 1, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/30085013/mitral-valve-abnormalities-in-hypertrophic-cardiomyopathy-a-primary-expression-of-the-disease-getting-closer-to-the-answer
#15
Paolo Spirito, Maria Iascone, Paolo Ferrazzi
No abstract text is available yet for this article.
July 31, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/30083548/cardiac-amyloidosis-the-great-masquerader
#16
Jubran Rind, Nagib Chalfoun, Richard McNamara
Cardiac amyloidosis is an elusive condition that is notorious for mimicking various cardiovascular conditions that present with left ventricular hypertrophy (LVH). The hypertrophy in amyloidosis is typically diffuse; however, rare reports of echocardiographic resemblances with hypertrophic cardiomyopathy (HCM) exist, such as asymmetric septal hypertrophy and left ventricular outflow tract obstruction. Cardiac MRI can help differentiate amyloidosis from hypertrophic cardiomyopathy in unclear situations. This differentiation from HCM and other forms of cardiomyopathy has important treatment implications...
June 30, 2018: Global Cardiology Science & Practice
https://www.readbyqxmd.com/read/30075967/exercise-hemodynamics-in-hypertrophic-cardiomyopathy-identify-risk-of-incident-heart-failure-but-not-ventricular-arrhythmias-or-sudden-cardiac-death
#17
Eric D Smith, June Tome, Ryan Mcgrath, Suwen Kumar, Maryann Concannon, Sharlene M Day, Sara Saberi, Adam S Helms
OBJECTIVE: To determine whether abnormal blood pressure response (ABPR), with or without left ventricular outflow tract obstruction (LVOTO), is associated with adverse heart failure and arrhythmia outcomes in hypertrophic cardiomyopathy (HCM). METHODS: A retrospective, single-center analysis was performed for adult HCM patients who underwent exercise stress testing. RESULTS: Of 589 patients included in the study, 192 (33%) demonstrated ABPR...
July 24, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/30066903/lncrna-and-mrna-interaction-study-based-on-transcriptome-profiles-reveals-potential-core-genes-in-the-pathogenesis-of-human-thoracic-aortic-dissection
#18
Yang Li, Nan Yang, Xianbao Zhou, Xuezhi Bian, Genqiang Qiu, Mo Zhang, Huagang Lin, Dingfeng Li
The aim of the present study was to determine the potential core genes in the pathogenesis of human thoracic aortic dissection (TAD) by analyzing microarray profiles of long non‑coding (lnc)‑RNAs between TAD and normal thoracic aorta (NTA). The differentially expressed lncRNA profiles of the aorta tissues between TAD patients (TAD group, n=6) and age‑matched donors with aortic diseases (NTA group, n=6) were analyzed by lncRNAs microarray. Gene ontology (GO), pathway and network analyses were used to further investigate candidate lncRNAs and mRNAs...
July 23, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/30065175/cardiomyopathies-and-related-changes-in-contractility-of-human-heart-muscle
#19
REVIEW
Petr G Vikhorev, Natalia N Vikhoreva
About half of hypertrophic and dilated cardiomyopathies cases have been recognized as genetic diseases with mutations in sarcomeric proteins. The sarcomeric proteins are involved in cardiomyocyte contractility and its regulation, and play a structural role. Mutations in non-sarcomeric proteins may induce changes in cell signaling pathways that modify contractile response of heart muscle. These facts strongly suggest that contractile dysfunction plays a central role in initiation and progression of cardiomyopathies...
July 31, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/30062317/cardiogenic-shock-secondary-to-takotsubo-cardiomyopathy-in-a-patient-with-preexisting-hypertrophic-obstructive-cardiomyopathy
#20
Nikhil Nalluri, Deepak Asti, Viswajit Reddy Anugu, Uroosa Ibrahim, James C Lafferty, Yefim Olkovsky
No abstract text is available yet for this article.
June 2018: CASE: Cardiovascular Imaging Case Reports
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