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Noncompaction cardiomyopathy

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https://www.readbyqxmd.com/read/28817544/left-ventricular-noncompaction-cardiomyopathy-with-severe-coronary-artery-disease-in-a-young-patient-with-familial-hypercholesterolemia
#1
Marta Kałużna-Oleksy, Jacek Migaj, Magdalena Dudek, Maciej Lesiak, Ewa Straburzyńska-Migaj
No abstract text is available yet for this article.
August 9, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28803523/left-ventricular-noncompaction-or-hypertrophic-cardiomyopathy-both
#2
Bruno Brochado, Sofia Cabral, António Pinheiro-Vieira, Henrique Carvalho, Severo Torres
No abstract text is available yet for this article.
August 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28798025/novel-genetic-triggers-and-genotype-phenotype-correlations-in-patients-with-left-ventricular-noncompaction
#3
Karol Miszalski-Jamka, John L Jefferies, Wojciech Mazur, Jan Głowacki, Jianhong Hu, Monika Lazar, Richard A Gibbs, Jacek Liczko, Jan Kłyś, Eric Venner, Donna M Muzny, Jarosław Rycaj, Jacek Białkowski, Ewa Kluczewska, Zbigniew Kalarus, Shalini Jhangiani, Hussein Al-Khalidi, Tomasz Kukulski, James R Lupski, William J Craigen, Matthew N Bainbridge
BACKGROUND: Left ventricular noncompaction (LVNC) is a genetically and phenotypically heterogeneous disease and, although increasingly recognized in clinical practice, there is a lack of widely accepted diagnostic criteria. We sought to identify novel genetic causes of LVNC and describe genotype-phenotype correlations. METHODS AND RESULTS: A total of 190 patients from 174 families with left ventricular hypertrabeculation (LVHT) or LVNC were referred for cardiac magnetic resonance and whole-exome sequencing...
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28794111/clinical-characteristics-and-long-term-outcome-of-hypertrophic-cardiomyopathy-in-individuals-with-a-mybpc3-myosin-binding-protein-c-founder-mutation
#4
Hannah G van Velzen, Arend F L Schinkel, Rogier A Oldenburg, Marjon A van Slegtenhorst, Ingrid M E Frohn-Mulder, Jolanda van der Velden, Michelle Michels
BACKGROUND: MYBPC3 (Myosin-binding protein C) founder mutations account for 35% of hypertrophic cardiomyopathy (HCM) cases in the Netherlands. We compared clinical characteristics and outcome of MYBPC3 founder mutation (FG+) HCM with nonfounder genotype-positive (G+) and genotype-negative (G-) HCM. METHODS AND RESULTS: The study included 680 subjects: 271 FG+ carriers, 132 G+ probands with HCM, and 277 G- probands with HCM. FG+ carriers included 134 FG+ probands with HCM, 54 FG+ relatives diagnosed with HCM after family screening, 74 FG+/phenotype-negative relatives, and 9 with noncompaction or dilated cardiomyopathy...
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#5
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28729659/identification-of-a-hybrid-myocardial-zone-in-the-mammalian-heart-after-birth
#6
Xueying Tian, Yan Li, Lingjuan He, Hui Zhang, Xiuzhen Huang, Qiaozhen Liu, Wenjuan Pu, Libo Zhang, Yi Li, Huan Zhao, Zhifu Wang, Jianhong Zhu, Yu Nie, Shengshou Hu, David Sedmera, Tao P Zhong, Ying Yu, Li Zhang, Yan Yan, Zengyong Qiao, Qing-Dong Wang, Sean M Wu, William T Pu, Robert H Anderson, Bin Zhou
Noncompaction cardiomyopathy is characterized by the presence of extensive trabeculations, which could lead to heart failure and malignant arrhythmias. How trabeculations resolve to form compact myocardium is poorly understood. Elucidation of this process is critical to understanding the pathophysiology of noncompaction disease. Here we use genetic lineage tracing to mark the Nppa(+) or Hey2(+) cardiomyocytes as trabecular and compact components of the ventricular wall. We find that Nppa(+) and Hey2(+) cardiomyocytes, respectively, from the endocardial and epicardial zones of the ventricular wall postnatally...
July 20, 2017: Nature Communications
https://www.readbyqxmd.com/read/28709664/current-results-of-single-ventricle-palliation-of-patients-with-double-inlet-left-ventricle
#7
Bahaaldin Alsoufi, Courtney McCracken, Kirk Kanter, Subhadra Shashidharan, Brian Kogon
BACKGROUND: Double inlet left ventricle (DILV) is a heterogeneous single ventricle anomaly in which initial presentation, and consequently, timing and palliation mode vary based on morphology and degree of pulmonary or systemic outflow obstruction. Very few reports, mostly old, focused on palliation outcomes of DILV. We report current-era results and examine whether morphologic and subsequently surgical factors influence survival. METHODS: Fifty-eight infants with DILV underwent single ventricle palliation...
July 11, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28703265/noncompaction-may-not-only-be-non-isolated-but-also-myopathic-commentary-to-the-article-left-ventricular-non-compaction-associated-with-hypertrophic-cardiomyopathy-in-the-same-patient
#8
https://www.readbyqxmd.com/read/28695818/progress-in-molecular-genetic-study-of-mitochondrial-cardiomyopathy
#9
Ruiqi Zhuge, Rong Zhou, Xinhai Ni
Mitochondria plays a key role in providing ATP for the energy-consuming cardiac tissues. Mitochondrial cardiomyopathy is a myocardial condition characterized by abnormal heart structure and/or function secondary to genetic defects involving the mitochondrial respiratory chain. The typical cardiac manifestations of mitochondrial cardiomyopathy include hypertrophic and dilated cardiomyopathy,while left ventricular myocardial noncompaction is less common. Recent research has suggested that most mitochondrial diseases result from mitochondrial DNA mutation,which can be found in genes that encode ancillary proteins needed for genetic transcription (tRNA),in genes that encode subunits of the electron transport chain complexes,or in genes that control the activities of the mitochondria called D-loop zone...
June 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28649555/autosomal-dominant-carvajal-plus-syndrome-due-to-the-novel-desmoplakin-mutation-c-1678a%C3%A2-%C3%A2-t-p-ile560phe
#10
Josef Finsterer, Claudia Stöllberger, Eva Wollmann, Susanne Dertinger, Franco Laccone
BACKGROUND: Carvajal syndrome is an autosomal dominant or autosomal recessive disorder, manifesting with dilated cardiomyopathy, woolly hair, and palmoplantar keratoma. Additional manifestations can be occasionally found. Carvajal syndrome may be due to mutations in the desmocollin-2, desmoplakin, or plakophilin-2 gene. METHODS AND RESULTS: We report a family with Carvajal syndrome which additionally presented with hypoacusis, noncompaction, recurrent pharyngeal infections, oligodontia, and recurrent diarrhoea...
September 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28645472/bradycardia-syncope-and-left-ventricular-noncompaction-cardiomyopathy
#11
D Luke Glancy, Frederick R Helmcke, Allen P Hoang
A 55-year-old man with syncopal episodes was found to have sinus bradycardia at a rate of 37 beats/min, an episode of nonsustained ventricular tachycardia, and left ventricular noncompaction cardiomyopathy. After placement of a dual-chamber cardioverter defibrillator 4 years ago, he has had no further syncopal episodes, and there have been no defibrillator shocks.
May 30, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#12
Monique M Gardner, Meryl S Cohen
BACKGROUND: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. METHODS: We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28616812/biventricular-noncompaction-cardiomyopathy-with-malignant-arrhythmia-as-a-cause-of-sudden-death
#13
Petr Handlos, Tomáš Gruszka, Marie Staňková, Klára Marecová, Marek Joukal
No abstract text is available yet for this article.
June 15, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28545618/nosology-of-noncompaction-cardiomyopathy-the-emperor-still-wears-clothes
#14
EDITORIAL
Erwin Oechslin, Rolf Jenni
No abstract text is available yet for this article.
June 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28528884/acute-myocarditis-versus-ventricular-noncompaction-cardiomyopathy-in-infancy-role-of-magnetic-resonance
#15
Ferran Gran, María Martínez-Villar, Anna Sabaté-Rotés, Amparo Castellote, Ferran Rosés-Noguer, Dimpna C Albert
No abstract text is available yet for this article.
May 18, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28521630/isolated-ventricular-noncompaction-cardiomyopathy-presenting-as-fetal-hydrops-at-24-weeks-gestation
#16
Jane E Armes, Lisa Squires, Rohan Lourie, Mark Williams, Renee Gallagher, Gareth Price, Andrew Stubbs, Sigrid Ma Swagemakers, Peter J van der Spek, James Harraway, Joseph Thomas, Deon J Venter
Ventricular noncompaction cardiomyopathy is a rare form of congenital cardiomyopathy with increasing evidence of genetic etiology, especially when presenting in childhood. Fetal presentation is rare. We describe a case of fetal hydrops, presenting at 24 weeks gestation and leading to intrapartum death at 26 weeks gestation. Autopsy examination revealed characteristic features of left ventricular noncompaction. A genetic analysis identified a constellation of variants of unknown significance in MYH6, TNNC1, and MYBPC3, genes known to be important in sarcomeric function...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28488311/left-ventricular-noncompaction-cardiomyopathy-in-a-patient-with-trisomy-13-a-report-and-review-of-the-literature
#17
Anri Hayashi, Tomohiro Kumada, Fumihito Nozaki, Ikuko Hiejima, Minoru Shibata, Takashi Kusunoki, Tatsuya Fujii
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent trabecular meshwork, and it is thought to result from arrest of the normal compaction process during embryogenesis. Patients with LVNC may be asymptomatic or have symptoms ranging from heart failure to stroke, life-threatening arrhythmias, or sudden death. The frequency of LVNC in children has increased with longer clinical courses. About 80% of patients with trisomy 13 have a congenital cardiac abnormality, but a clinical description of LVNC with trisomy 13 is lacking because of its poor prognosis and lack of awareness about LVNC...
May 9, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28483032/advances-in-diagnosis-and-management-of-mitochondrial-cardiomyopathy
#18
Ruiqi Zhuge, Rong Zhou, Xinhai Ni
Mitochondrial cardiomyopathy (MCM) is a series of myocardial conditions characterized by abnormal heart-muscle structure,function,or both,secondary to genetic defects involving the mitochondrial respiratory chain,in the absence of concomitant coronary artery disease,hypertension,valvular disease,or congenital heart disease. MCM patients typically have hypertrophic or dilated cardiomyopathy. Arrhythmias and left ventricular myocardial noncompaction are less common,and heart failure may occur as the first symptom in some patients...
April 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28469993/left-ventricular-noncompaction-cardiomyopathy
#19
Amer Hawatmeh, Habib Habib, Fayez Shamoon
No abstract text is available yet for this article.
April 2017: Avicenna Journal of Medicine
https://www.readbyqxmd.com/read/28458700/echocardiographic-characteristics-of-isolated-left-ventricular-noncompaction
#20
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
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