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Noncompaction cardiomyopathy

Arun Gopi, Placid Sebastian, C D Ramakrishna
Isolated ventricular noncompaction (IVNC) is an unclassified cardiomyopathy characterized by prominent intraventricular trabeculations separated by deep intertrabecular recesses. Although microvascular dysfunction is known, myocardial infarction is rare and usually seen as a consequence of coincidental coronary artery disease. We report the case of a 19-year-old male patient who presented to us with symptoms and signs of heart failure. Echocardiography revealed IVNC with severe left ventricular dysfunction...
September 2016: Indian Heart Journal
Nikhil Kumar, Christopher A Troianos, Joshua S Baisden
In this report, we present the case of a patient with biventricular noncompaction cardiomyopathy, Ebstein anomaly, and a left atrial mass who required emergent placement of a left ventricular assist device. The noncompaction cardiomyopathy complicated the left ventricular assist device implantation procedure because the thickened, trabeculated myocardium made it difficult to place the inflow cannula. We discuss our perioperative management strategy, in which transesophageal echocardiography was used, to help the surgical team identify the proper cannula placement and provide a bridge to transplantation...
October 5, 2016: A & A Case Reports
Josef Finsterer, Sinda Zarrouk-Mahjoub
No abstract text is available yet for this article.
2016: Case Reports in Cardiology
Gonenc Kocabay, Serdar Yilmazer, Yetkin Korkmaz, Asli Karadeniz, Nilufer Eksi Duran
We report a case of cyst was initially labeled as left ventricular noncompaction cardiomyopathy. An accurate diagnosis is essential to establish the most effective treatment strategy. In particular, echocardiographic examination assists in identifying the correct diagnosis. In this case, two-dimensional and three-dimensional echocardiography and computed tomography were used for definitive diagnosis of cardiac hydatid cyst.
September 30, 2016: Echocardiography
Juan M Vinardell, Maria D Avila, Orlando Santana
Isolated left ventricular noncompaction is either a distinct cardiomyopathy or a morphologic trait shared by several different types of cardiomyopathies. Although there is no current gold standard for its diagnosis, cardiac imaging is the most commonly accepted modality. Described is a case of left ventricular noncompaction that resolved 2 years after the initial diagnosis.
2016: Reviews in Cardiovascular Medicine
Attila Nemes, Péter Domsik, Anita Kalapos, Henriette Gavallér, Mónika Oszlánczi, Tamás Forster
OBJECTIVE: Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy characterized by a prominent trabecular meshwork and deep intertrabecular recesses. The present study aimed to examine right atrial (RA) volumes, volume-based functional properties and strains by three-dimensional speckle-tracking echocardiography (3DSTE) in ILVNC patients. METHODS: The study group consisted of 13 patients with ILVNC (mean age: 54.6±14.1 years, six male) and 23 healthy age- and gender-matched volunteers (mean age: 50...
October 2016: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
Sophie Van Malderen, Sip Wijchers, Ferdi Akca, Kadir Caliskan, Tamas Szili-Torok
BACKGROUND: Noncompaction cardiomyopathy (NCCM) is considered to be the result of an arrest in the normal myocardial embryogenesis. The histological, developmental, and electrophysiological explanation of ventricular arrhythmias in NCCM is still unknown. The aim of this study was to determine the origin of premature ventricular contractions (PVCs) in NCCM and to identify any predominant arrhythmic foci. METHODS: Retrospective data from our NCCM registry including 101 patients were analyzed...
August 29, 2016: Annals of Noninvasive Electrocardiology
Nay Aung, Filip Zemrak, Saidi A Mohiddin, Steffen E Petersen
No abstract text is available yet for this article.
August 20, 2016: JACC. Cardiovascular Imaging
Eloisa Arbustini, Valentina Favalli, Nupoor Narula, Alessandra Serio, Maurizia Grasso
Left ventricular noncompaction (LVNC) describes a ventricular wall anatomy characterized by prominent left ventricular (LV) trabeculae, a thin compacted layer, and deep intertrabecular recesses. Individual variability is extreme, and trabeculae represent a sort of individual "cardioprinting." By itself, the diagnosis of LVNC does not coincide with that of a "cardiomyopathy" because it can be observed in healthy subjects with normal LV size and function, and it can be acquired and is reversible. Rarely, LVNC is intrinsically part of a cardiomyopathy; the paradigmatic examples are infantile tafazzinopathies...
August 30, 2016: Journal of the American College of Cardiology
Josef Finsterer, Claudia Stöllberger
Objectives. Psychosis and left ventricular hypertrabeculation (or noncompaction) (LVHT) have not been described in the same patient. Here we report a patient with a long-term history of schizophrenia who was later diagnosed with dilated cardiomyopathy (dCMP) and LVHT. Case Report. A 47-year-old Caucasian male developed nondifferentiated schizophrenia at the age of 26 y. Since the age of 33 y he was regularly drinking alcohol. At the age of 47 y he developed heart failure. Transthoracic echocardiography showed an enlarged left ventricle, reduced systolic function, and surprisingly LVHT in the apical segment...
2016: Case Reports in Cardiology
Asaad A Khan, Lucy Safi, Malissa Wood
Athletic heart syndrome refers to the physiological and morphological changes that occur in a human heart after repetitive strenuous physical exercise. Examples of exercise-induced changes in the heart include increases in heart cavity dimensions, augmentation of cardiac output, and increases in heart muscle mass. These cardiac adaptations vary based on the type of exercise performed and are often referred to as sport-specific cardiac remodeling. The hemodynamic effects of endurance and strength training exercise lead to these adaptations...
April 2016: Methodist DeBakey Cardiovascular Journal
Ricardo Ronderos, Gustavo Avegliano, Evelyn Borelli, Paola Kuschnir, Florencia Castro, Gustavo Sanchez, Gabriel Perea, Mariana Corneli, Maria M Zanier, Sol Andres, Adriana Aranda, Diego Conde, Marcelo Trivi
The prevalence of left ventricular noncompacted myocardium (LVNC) is not clearly defined yet. The goal of this study was to prospectively assess the prevalence of LVNC in a population of patients assessed for cardiovascular disease and to analyze the coincidence between observers using the echocardiographic criteria for diagnosis of LVNC. We included patients prospectively during a 1-year period. To analyze the concordance between different observers, we performed a blind analysis of 50 patients between 7 operators to calculate Kappa index for each criteria...
September 15, 2016: American Journal of Cardiology
Sinem Özyılmaz, Özgür Akgül, Adem Kiriş, Aydın Yıldırım, İhsan Bakır
No abstract text is available yet for this article.
July 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Mareomi Hamada, Yoshimi Takamura, Takashi Otani, Kiyotaka Ohshima, Akiyoshi Ogimoto, Shuntaro Ikeda, Taiko Horii
No abstract text is available yet for this article.
October 1, 2016: International Journal of Cardiology
Andraž Cerar, Juš Kšela, Gregor Poglajen, Bojan Vrtovec, Ivan Kneževič
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare hereditary cardiomyopathy characterized by the formation of an outer compacted and inner noncompacted layer of the myocardium. The latter is characterized by prominent trabeculations and deep intertrabecular recesses and is functionally inferior to the compacted myocardium. As there is no specific treatment for patients with LVNC who develop heart failure, the management of these patients is limited and many patients progress to advanced stages of the disease...
2016: Heart Surgery Forum
Doyeon Kim, Eunhee Kim, Jong-Hwan Lee, Chung Su Kim, Sangmin Maria Lee, Jung Eun Lee
Isolated left ventricular noncompaction (LVNC) is a rare primary genetic cardiomyopathy characterized by prominent trabeculation of the left ventricular wall and intertrabecular recesses. Perioperative management of the patient with LVNC might be challenging due to the clinical symptoms of heart failure, systemic thromboembolic events, and fatal left ventricular arrhythmias. We conducted real time intraoperative transesophageal echocardiography in a patient with LVNC undergoing general anesthesia for ovarian cystectomy...
June 2016: Korean Journal of Anesthesiology
Marie-Louise Bang
The cardiac Z-line at the boundary between sarcomeres is a multiprotein complex connecting the contractile apparatus with the cytoskeleton and the extracellular matrix. The Z-line is important for efficient force generation and transmission as well as the maintenance of structural stability and integrity. Furthermore, it is a nodal point for intracellular signaling, in particular mechanosensing and mechanotransduction. Mutations in various genes encoding Z-line proteins have been associated with different cardiomyopathies, including dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, restrictive cardiomyopathy, and left ventricular noncompaction, and mutations even within the same gene can cause widely different pathologies...
January 2017: Journal of Cellular Physiology
Marlies Antlanger, Andreas A Kammerlander, Robert Ullrich, Michael Haidinger, Diana Bonderman, Julia Mascherbauer, Marcus D Säemann
Pregnancy and associated pre-eclampsia carry a high maternal risk in hemodialysis patients, yet no guidelines on how to monitor these patients' cardiovascular function exist. A 34-year-old hemodialysis patient presented with peripartum cardiomyopathy after a late second trimester miscarriage. On cardiac magnetic resonance imaging, diagnostic features of left ventricular noncompaction were apparent. Yet, histological and gene panel analyses remained negative. Upon stringent dry weight control and pharmacological heart failure therapy, the pathological changes showed complete regression...
May 5, 2016: Hemodialysis International
Federico Migliore, Maria Silvano, Alessandro Zorzi, Emanuele Bertaglia, Mariachiara Siciliano, Loira Leoni, Pietro De Franceschi, Sabino Iliceto, Domenico Corrado
AIMS: This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies. METHODS AND RESULTS: The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation...
July 2016: Journal of Cardiovascular Medicine
Giulia Ottaviani, Ana Maria Segura, Indranee N Rajapreyar, Bihong Zhao, Rajko Radovancevic, Pranav Loyalka, Biswajit Kar, Igor Gregoric, L Maximilian Buja
BACKGROUND: Previous studies reported that left ventricular noncompaction (LVNC) is a cardiomyopathy, familial or sporadic, arising from arrest of the normal process of trabecular remodeling during embryonic development. The diagnosis is usually made by echocardiography, but to date, there has been little research on the occurrence and clinicopathological features of LVNC in the explanted hearts of orthotopic heart transplant (OHT) recipients. DESIGN: The clinical, echocardiographic, and pathologic findings were reviewed for evidence of LVNC, diagnosed by echocardiographic criteria, in 105 patients with end-stage heart failure (HF) undergoing OHT...
July 2016: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
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