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Left ventricle non compaction

Andreina Carbone, Antonello D'Andrea, Lucia Riegler, Raffaella Scarafile, Enrica Pezzullo, Francesca Martone, Raffaella America, Biagio Liccardo, Maurizio Galderisi, Eduardo Bossone, Raffaele Calabrò
Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete's blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias...
June 26, 2017: World Journal of Cardiology
Elisabetta Chiodi, Marianna Nardozza, Maria Rita Gamberini, Alessia Pepe, Massimo Lombardi, Giorgio Benea, Donato Mele
To differentiate left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to LV remodeling in β-thalassemia major (β-TM) patients, cardiac magnetic resonance (CMR) images of 38 β-TM patients and 10 LVNC patients were compared using 3 diagnostic criteria: ratio of diastolic segmental non-compacted to compacted myocardium (NC/C ratio) >2.5, percentage of non-compacted LV mass (NC-LVM%) >20% and >25% of global LV mass. Specificity of NC/C ratio of >2.5 was the lowest (58%) and of NC-LVM% of >25% the highest (93%)...
May 17, 2017: Clinical Imaging
Afsoon Fazlinezhad, Mohammad Vojdanparast, Shadi Sarafan, Pouya Nezafati
BACKGROUND: Although isolated left ventricular noncompaction (ILVNC) has been described almost two decades ago, our knowledge about its diagnosis, presentation, echocardiographic features and clinical outcome is sparse. We aimed to assess the echocardiographic and clinical characteristics of ILVNC in a group of patients referred to our center. METHODS: Patients who were referred to a tertiary referral center, affiliated with Mashhad University of Medical Sciences, with primary diagnosis of dilated cardiomyopathy underwent comprehensive echocardiographic evaluation...
September 2016: ARYA Atherosclerosis
Pasquale Piccolo, Sergio Attanasio, Ilaria Secco, Riccardo Sangermano, Caterina Strisciuglio, Giuseppe Limongelli, Erasmo Miele, Margherita Mutarelli, Sandro Banfi, Vincenzo Nigro, Tirso Pons, Alfonso Valencia, Lorena Zentilin, Severo Campione, Gerardo Nardone, Ty C Lynnes, Patricia B S Celestino-Soper, Katherine G Spoonamore, Francesco P D'Armiento, Mauro Giacca, Annamaria Staiano, Matteo Vatta, Chiara Collesi, Nicola Brunetti-Pierri
We performed whole exome sequencing in individuals from a family with autosomal dominant gastropathy resembling Ménétrier disease, a premalignant gastric disorder with epithelial hyperplasia and enhanced EGFR signalling. Ménétrier disease is believed to be an acquired disorder, but its aetiology is unknown. In affected members, we found a missense p.V742G variant in MIB2, a gene regulating NOTCH signalling that has not been previously linked to human diseases. The variant segregated with the disease in the pedigree, affected a highly conserved amino acid residue, and was predicted to be deleterious although it was found with a low frequency in control individuals...
January 1, 2017: Human Molecular Genetics
Hoang H Nguyen, Rabia Khan, Norman H Silverman, Gautam K Singh
Left ventricle non-compaction (LVNC) has worse outcomes when associated with congenital heart defects (CHD). The co-occurrence and outcomes of LVNC with tricuspid atresia (TA) are not well described. Our study aims to determine the prevalence of LVNC with functionally single ventricle due to TA, and to describe the early outcomes of surgical palliation. A retrospective database search for patients (n = 167,566) and echocardiograms (n = 44,053) was performed in order to collect clinical, echocardiographic, and hemodynamic data of pediatric patients with TA and LVNC at St...
March 2017: Pediatric Cardiology
Jagdish C Mohan, Vishwas Mohan, Madhu Shukla, Arvind Sethi
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation...
September 2016: Indian Heart Journal
Natalia Y Osovska, Natalia V Kuzminova
INTRODUCTION: isolated left ventricular non-compaction (LVNC) is a heart disease with rather distinct morphologic and clinical manifestations. Available in the literature information about LVNC considering multiple left ventricle abnormal chords (LVAC) as one of its criterion motivated us to review the results obtained in the study of young patients with this pathology. The aim of the research was to demonstrate different clinical variants of left ventricular non-compaction course in adult patients and to clarify some pathogenetic aspects of this pathology...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Rebeca Lorca, María Martín, José Julián R Reguero, Beatriz Díaz-Molina, César Morís, José Luis Lambert, Aurora Astudillo
No abstract text is available yet for this article.
November 15, 2016: International Journal of Cardiology
N Osovska, N Kuzminova, M Ovcharuk, O Serhiychuk
Structural heart anomalies (SHA) are congenital abnormalities of cardiovascular system, characterized by various anatomical departures of heart and great vessels from normal conditions. SHA are a part of connective tissue dysplasia syndrome (CTDS), one of the most common congenital autosomal-dominant diseases in people of young and middle age. The most common SHA are a mitral valve prolapse, abnormal chords of left ventricle and their combinations. The clinical significance of these anomalies depends on a degree of severity and impact on intracardial hemodynamics, as described in the article...
June 2016: Georgian Medical News
Umair Hayat, Chris Lim, Sylvia Chen
CLINICAL INTRODUCTION: A 75-year-old patient with hypertension and severe aortic stenosis underwent elective coronary angiography that showed mild non-obstructive disease in the mid left anterior descending artery (LAD). A left ventriculogram, however, demonstrated segmental systolic dysfunction with dilated akinetic apex (figure 1A, see online supplementary video 1). There was no history of prior myocardial infarction and the patient had not experienced any chest pain recently. A 12-lead ECG showed widespread deep symmetrical inverted T-waves with the exception of leads I, aVL and V1 (see online supplementary figure S1)...
July 14, 2016: Heart: Official Journal of the British Cardiac Society
C Fiorillo, G Astrea, M Savarese, D Cassandrini, G Brisca, F Trucco, M Pedemonte, R Trovato, L Ruggiero, L Vercelli, A D'Amico, G Tasca, M Pane, M Fanin, L Bello, P Broda, O Musumeci, C Rodolico, S Messina, G L Vita, M Sframeli, S Gibertini, L Morandi, M Mora, L Maggi, A Petrucci, R Massa, M Grandis, A Toscano, E Pegoraro, E Mercuri, E Bertini, T Mongini, L Santoro, V Nigro, C Minetti, F M Santorelli, C Bruno
BACKGROUND: Myosin heavy chain 7 (MYH7)-related myopathies are emerging as an important group of muscle diseases of childhood and adulthood, with variable clinical and histopathological expression depending on the type and location of the mutation. Mutations in the head and neck domains are a well-established cause of hypertrophic cardiomyopathy whereas mutation in the distal regions have been associated with a range of skeletal myopathies with or without cardiac involvement, including Laing distal myopathy and Myosin storage myopathy...
July 7, 2016: Orphanet Journal of Rare Diseases
Sourabh Aggarwal, Jagadeesh Kalavakunta, Vishal Gupta
Non-compaction of ventricular myocardium (NCVM) is a rare genetic disorder caused by intrauterine arrest of endomyocardial development. Left ventricle is the usual site of NCVM with very rare reports of isolated right ventricular non-compaction (IRVNC). We describe a case of asymptomatic IRVNC with unique EKG changes.
January 2016: Heart Views: the Official Journal of the Gulf Heart Association
Gaetano D'Amato, Guillermo Luxán, José Luis de la Pompa
The vertebrate heart is the first organ to form and function during embryogenesis. Primitive streak-derived cardiac progenitors located bilaterally move rostral to form the primitive heart tube that subsequently undergoes rightward looping, remodelling and septation to give rise to the mature four-chambered heart. Tightly regulated tissue interactions orchestrate the patterning, proliferation and differentiation processes that give rise to the adult ventricles. Studies in animal models have demonstrated the crucial function of the Notch signalling pathway in ventricular development and how alterations in human NOTCH signalling may lead to disease in the form of cardiomyopathies, such as left ventricular noncompaction (LVNC)...
December 2016: FEBS Journal
Yeonu Choi, Sung Mok Kim, Sang-Chol Lee, Sung-A Chang, Shin Yi Jang, Yeon Hyeon Choe
BACKGROUND: Left ventricular non-compaction (LVNC) is an unclassified cardiomyopathy and there is no consensus on the diagnosis of LVNC. The aims of this study were to establish quantitative methods to diagnose LVNC using cardiovascular magnetic resonance (CMR) and to suggest refined semi-quantitative methods to diagnose LVNC. METHODS: This retrospective study included 145 subjects with mild to severe trabeculation of the left ventricle myocardium [24 patients with isolated LVNC, 33 patients with non-isolated LVNC, 30 patients with dilated cardiomyopathy (DCM) with non-compaction (DCMNC), 27 patients with DCM, and 31 healthy control subjects with mild trabeculation]...
May 4, 2016: Journal of Cardiovascular Magnetic Resonance
Stefan Peters
No abstract text is available yet for this article.
July 15, 2016: International Journal of Cardiology
Eric Emmanuel Coris, Byron Keith Moran, Raymond De Cuba, Ted Farrar, Anne B Curtis
Isolated left ventricular non-compaction (LVNC) has usually been viewed as a rare cardiomyopathy in athletes. However, with advances in diagnostic imaging techniques and increased use of pre-participation screening electrocardiograms (ECGs), apparent LVNC is being recognized in an increasing number of athletes. Given the lack of a true gold standard for diagnosis, significant debate continues regarding optimal diagnostic criteria. There are increasing data to support the possibility of over-diagnosing this cardiomyopathy in an athletic population due to the physiologic adaptation to the extreme preload and afterload characteristic of intense athletic participation...
September 2016: Sports Medicine
Belma Kalaycı, Süleyman Kalaycı, Turgut Karabağ, Mustafa Aydın
Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by deep increased trabeculation in one or more segments of the ventricle. The apical segment of the left ventricle is most commonly affected, but left ventricular basal segment, biventricular involvement or right ventricle predominance have also been described. While some neuromuscular anomalies and myopathies had been described in systemic sclerosis patients, coexistence of chronic inflammatory disorders and NCM is unclear...
December 2015: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Aldo L Schenone, Aaron Cohen, Gosta Pettersson, David Majdalany
Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement...
May 2016: World Journal for Pediatric & Congenital Heart Surgery
J Dąbek, M Majewski, W Walkowicz, Z Gąsior
Left ventricular non-compaction (LVNC) is a rare cardiomyopathy that results from unsettled embryogenesis of myocardium. It is morphologically characterised by the presence of non-compacted, this is hypertrabeculated, myocardium of the left ventricle with deep endocardial recesses. The clinical spectrum of symptoms is very wide - from asymptomatic patients through the cases of heart failure to the patients requiring heart transplantation. The diagnosis is most frequently based on the echocardiography. LVNC is often coexisted with other heart defects and coronary artery abnormalities...
2015: Folia Morphologica (Warsz)
Emad Muhammad, Aviva Levitas, Sonia R Singh, Alex Braiman, Rivka Ofir, Sharon Etzion, Val C Sheffield, Yoram Etzion, Lucie Carrier, Ruti Parvari
Gene mutations, mostly segregating with a dominant mode of inheritance, are important causes of dilated cardiomyopathy (DCM), a disease characterized by enlarged ventricular dimensions, impaired cardiac function, heart failure and high risk of death. Another myocardial abnormality often linked to gene mutations is left ventricular noncompaction (LVNC) characterized by a typical diffuse spongy appearance of the left ventricle. Here, we describe a large Bedouin family presenting with a severe recessive DCM and LVNC...
December 20, 2015: Human Molecular Genetics
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