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Noncompaction cardiomyopathy

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https://www.readbyqxmd.com/read/29035186/left-ventricular-noncompaction-cardiomyopathy-and-recurrent-polymorphic-ventricular-tachycardia-a-case-report-and-literature-review
#1
Oluwaseun A Akinseye, Uzoma N Ibebuogu, Sunil K Jha
INTRODUCTION: Noncompaction cardiomyopathy is a rare phenotype of cardiomyopathy associated with severe cardiac arrhythmia and thromboembolic complications. CASE PRESENTATION: A 55-year-old woman presented with frank pulmonary edema and received a diagnosis of noncompaction cardiomyopathy. DISCUSSION: Left ventricular noncompaction cardiomyopathy is increasingly being diagnosed because of advances in imaging modalities. It is important to differentiate this new phenotype of cardiomyopathy from others because its diagnosis, management, and prognosis differ...
2017: Permanente Journal
https://www.readbyqxmd.com/read/29032583/third-generation-ventricular-assist-device-mid-term-outcomes-of-the-heartware-hvad-in-pediatric-patients
#2
Mustafa Pac, Sinan Sabit Kocabeyoglu, Umit Kervan, Dogan Emre Sert, Serhat Koca, Ibrahim Ece, Feyza Aysenur Pac
The HeartWare HVAD is a small, third generation continuous flow pump that is intracorporeally placed for support of a failing ventricle in adult patients. This device is small in size when compared to other left ventricular assist devices and can therefore be used in smaller sized pediatric patients. We present our initial experience using the HVAD as a bridge to heart transplantation in the pediatric population. We performed a retrospective, single center, nonrandomized review of 17 pediatric patients who underwent HVAD implantation between June 2013 and March 2016...
October 15, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28980384/noncompaction-cardiomyopathy-in-an-infant-with-walker-warburg-syndrome
#3
Sarah Abdullah, Cynthia Hawkins, Gregory Wilson, Grace Yoon, Luc Mertens, Melissa T Carter, Andrea Guerin
Walker-Warburg syndrome (WWS) is a rare autosomal recessive, congenital muscular dystrophy that is associated with brain and eye anomalies. Several genes encoding proteins involved in α-dystroglycan glycosylation have been implicated in the aetiology of WWS. We describe a patient with nonclassical features of WWS presenting with heart failure related to noncompaction cardiomyopathy resulting in death at 4 months of age. Muscle biopsy revealed absent α-dystroglycan on immunostaining and genetic testing confirmed the diagnosis with two previously described POMT2 mutations...
October 5, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28966588/ischemic-stroke-in-a-young-patient-heralding-a-left-ventricular-noncompaction-cardiomyopathy
#4
Fanny Lestienne, Chiara Bruno, David Bertora, Jeanne Benoit, Marie-Hélène Mahagne, Laurent Suissa
Strokes in young patients may be the clinical expression of many complex and extremely rare diseases. Uncommon causes constitute less than 5% of all strokes, but are present in 30% of strokes in young patients. We report the case of a young woman whose ischemic stroke led to the diagnosis of a rare embolic cardiomyopathy, left ventricular noncompaction cardiomyopathy, requiring a heart transplant.
May 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28939283/histiocytoid-cardiomyopathy-and-ventricular-noncompaction-presenting-as-sudden-death-in-an-adult-male
#5
J Fernando Val-Bernal, Marta Mayorga, Clara Ortega, Emma Linares
Histiocytoid/oncocytic cardiomyopathy (HCM) is a rare, distinctive arrhythmogenic disorder that presents as arrhythmia or sudden death in infants and children. Ventricular noncompaction (VNC) is a rare cardiomyopathy characterized by a thickened endocardial layer of noncompacted myocardium and a thin epicardial layer of compacted myocardium. Only six cases of the association of both cardiomyopathies have been reported previously in the literature. All these cases were in children. To the best of our knowledge, a case of HCM has not been described in the adult...
September 7, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28917495/complications-associated-with-implantable-cardioverter-defibrillators-in-adults-with-congenital-heart-disease-or-left-ventricular-noncompaction-cardiomyopathy-from-the-ncdr-%C3%A2-implantable-cardioverter-defibrillator-registry
#6
Marye J Gleva, Yongfei Wang, Jeptha P Curtis, Charles I Berul, Charles B Huddleston, Jeanne E Poole
Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry...
August 8, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#7
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28912186/cardiomyopathies-due-to-left-ventricular-noncompaction-mitochondrial-and-storage-diseases-and-inborn-errors-of-metabolism
#8
REVIEW
Jeffrey A Towbin, John Lynn Jefferies
The normal function of the human myocardium requires the proper generation and utilization of energy and relies on a series of complex metabolic processes to achieve this normal function. When metabolic processes fail to work properly or effectively, heart muscle dysfunction can occur with or without accompanying functional abnormalities of other organ systems, particularly skeletal muscle. These metabolic derangements can result in structural, functional, and infiltrative deficiencies of the heart muscle. Mitochondrial and enzyme defects predominate as disease-related etiologies...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28912179/classification-epidemiology-and-global-burden-of-cardiomyopathies
#9
REVIEW
William J McKenna, Barry J Maron, Gaetano Thiene
In the past 25 years, major advances were achieved in the nosography of cardiomyopathies, influencing the definition and taxonomy of this important chapter of cardiovascular disease. Nearly, 50% of patients dying suddenly in childhood or adolescence or undergoing cardiac transplantation are affected by cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic, restrictive, and noncompacted) and added to update the World Health Organization classification. Myocarditis has also been named inflammatory cardiomyopathy...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28911804/case-report-left-ventricular-noncompaction-cardiomyopathy-and-rasopathies
#10
Juli Ann Sublett, Carlos Enrique Prada, John Lynn Jefferies
The following is a case report of 6 patients with Noonan syndrome (NS) and/or a related RASsopathy that also have evidence of left ventricular noncompaction cardiomyopathy (LVNC). Noonan syndrome,a type of RASopathy, is an autosomal dominant disorder that is typically associated with congenital heart defects and hypertrophic cardiomyopathy. There have been minimal reports of Noonan syndrome or other RASopathy and the association of LVNC. This report promulgates 6 nonrelated cases of Noonan syndrome or unspecified RASopathy and LVNC...
September 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28901675/sudden-cardiac-death-in-isolated-right-ventricular-hypertrabeculation-noncompaction-cardiomyopathy
#11
Soad Bekheit, Boutros Karam, Farshid Daneshvar, Julie Zaidan, Rabih Tabet, Jonathan Spagnola, James Lafferty
Hypertrabeculation/noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/noncompaction on echocardiography...
September 12, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28878579/left-ventricular-noncompaction-cardiomyopathy-presenting-with-heart-failure-in-a-35-year-old-man
#12
Kyriacos Papadopoulos, Petros M Petrou, Demos Michaelides
Isolated ventricular noncompaction, a rare genetic cardiomyopathy, is thought to be caused by the arrest of normal myocardial morphogenesis. It is characterized by prominent, excessive trabeculation in a ventricular wall segment and deep intertrabecular recesses perfused from the ventricular cavity. The condition can present with heart failure, systematic embolic events, and ventricular arrhythmias. Two-dimensional echocardiography is the typical diagnostic method. We report a case of heart failure in a 35-year-old man who presented with palpitations...
August 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28855170/a-wide-and-specific-spectrum-of-genetic-variants-and-genotype-phenotype-correlations-revealed-by-next-generation-sequencing-in-patients-with-left-ventricular-noncompaction
#13
Ce Wang, Yukiko Hata, Keiichi Hirono, Asami Takasaki, Sayaka Watanabe Ozawa, Hideyuki Nakaoka, Kazuyoshi Saito, Nariaki Miyao, Mako Okabe, Keijiro Ibuki, Naoki Nishida, Hideki Origasa, Xianyi Yu, Neil E Bowles, Fukiko Ichida
BACKGROUND: Left ventricular noncompaction (LVNC) has since been classified as a primary genetic cardiomyopathy, but the genetic basis is not fully evaluated. The aim of the present study was to identify the genetic spectrum using next-generation sequencing and to evaluate genotype-phenotype correlations in LVNC patients. METHODS AND RESULTS: Using next-generation sequencing, we targeted and sequenced 73 genes related to cardiomyopathy in 102 unrelated LVNC patients...
August 30, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28817544/left-ventricular-noncompaction-cardiomyopathy-with-severe-coronary-artery-disease-in-a-young-patient-with-familial-hypercholesterolemia
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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