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Left Ventricular Noncompaction

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2. Left Ventricular Twist Mechanics to Identify Left Ventricular Noncompaction in Childhood. (PubMed)

Left Ventricular Twist Mechanics to Identify Left Ventricular Noncompaction in Childhood. Left ventricular noncompaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood. Standard diagnostic criteria are still controversial, especially in young patients. Recent studies in adults demonstrated that left ventricular (LV) twist is abnormal in LVNC, but it has not been investigated in pediatric patients to date. Our aim was to assess LV cardiac mechanics, LV twist

2019 Circulation. Cardiovascular imaging

3. Usefulness of a left ventricular assist device in patients with left ventricular noncompaction (PubMed)

Usefulness of a left ventricular assist device in patients with left ventricular noncompaction Left ventricular noncompaction (LVNC) is a multifactorial structural abnormality of the myocardial wall characterized by prominent trabeculae and deep trabecular recesses. LVNC may present as a congenital or acquired defect characterized by 2 distinct tissue layers: a spongy, noncompacted inner myocardium and a thin, compacted outer myocardium. Patients with LVNC are prone to thromboembolic events (...) , either due to deep trabeculations in the noncompacted myocardium or due to arrhythmias accompanying the defect. There are sparse data concerning treatment options for patients with LVNC who fail medical management. We present 2 such patients with LVNC who, following failed medical management, received a left ventricular assist device (LVAD): one for long-term management and one as a bridge to transplant. Both were managed successfully without thromboembolic events to date. The success of these cases

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2018 Proceedings (Baylor University. Medical Center)

4. Catheter ablation of a right posterior accessory pathway in a patient with left ventricular noncompaction: A case report. (PubMed)

Catheter ablation of a right posterior accessory pathway in a patient with left ventricular noncompaction: A case report. Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by the presence of a thin compacted layer of myocardium and a spongy subendocardial layer with trabeculations and recesses. LVNC associated Wolf-Parkinson-White syndrome is very rare.A 32-year-old male presented with short episodes of palpitations and a syncope 6 months before his (...) hospitalization.His ECG revealed the presence of a right posterior accessory pathway. Echocardiography identified trabeculations of the septal, apical, and lateral wall of the left ventricle, consistent with left ventricular noncompaction. Cardiac MRI confirmed the diagnosis, as the ratio between the noncompacted and compacted myocardial layer was 2.3.The electrophysiological study revealed a malignant right posterior accessory pathway. Catheter ablation was successfully performed at the level of posterior

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2019 Medicine

5. Successful Treatment of an Infant with Left Ventricular Noncompaction Presenting with Fatal Ventricular Arrhythmia Treated with Cardiac Resynchronization Therapy and an Implantable Cardioverter Defibrillator (PubMed)

Successful Treatment of an Infant with Left Ventricular Noncompaction Presenting with Fatal Ventricular Arrhythmia Treated with Cardiac Resynchronization Therapy and an Implantable Cardioverter Defibrillator We herein report the successful treatment of a 4-year-old girl with left ventricular noncompaction (LVNC) who presented with incessant ventricular fibrillation at 5 months of age. An implantable cardioverter defibrillator (ICD) was implanted, and dual chamber (DDD) pacing was initiated at 7 (...) months of age. At her 10-month follow-up, her left ventricular ejection fraction (LVEF) had decreased from 45% to 20% with mechanical dyssynchrony. After upgrading to cardiac resynchronization therapy (CRT), the LVEF improved to 50%. The usefulness of CRT in pediatric LVNC has not been fully elucidated. However, our case suggests that CRT therapy may be an effective option for LVNC-induced cardiac dysfunction.

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2018 Case reports in cardiology

6. Left Ventricular Noncompaction Cardiomyopathy and Recurrent Polymorphic Ventricular Tachycardia: A Case Report and Literature Review (PubMed)

Left Ventricular Noncompaction Cardiomyopathy and Recurrent Polymorphic Ventricular Tachycardia: A Case Report and Literature Review Noncompaction cardiomyopathy is a rare phenotype of cardiomyopathy associated with severe cardiac arrhythmia and thromboembolic complications.A 55-year-old woman presented with frank pulmonary edema and received a diagnosis of noncompaction cardiomyopathy.Left ventricular noncompaction cardiomyopathy is increasingly being diagnosed because of advances in imaging

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2017 The Permanente journal

7. The TNNI3 Arg192His mutation in a 13-year-old girl with left ventricular noncompaction (PubMed)

The TNNI3 Arg192His mutation in a 13-year-old girl with left ventricular noncompaction Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy that is morphologically characterized by a two-layered myocardium, numerous prominent trabeculations, and deep intertrabecular recesses communicating with the left ventricular cavity. We present a case report regarding the identification of a new mutation in TNNI3 in a patient with LVNC using next-generation sequencing. A 13-year-old girl who (...) . left ventricular noncompaction using next-generation sequencing (NGS). Mutations in TNNI3 have been reported in patients with hypertrophic cardiomyopathy and restrictive cardiomyopathy. The use of NGS also results in the identification of multiple genetic variants of unknown significance to the investigated disease.>.

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2018 Journal of cardiology cases

8. A Rare Case of Left Ventricular Noncompaction in LEOPARD Syndrome (PubMed)

A Rare Case of Left Ventricular Noncompaction in LEOPARD Syndrome 29629024 2019 02 26 1975-4612 26 1 2018 Mar Journal of cardiovascular ultrasound J Cardiovasc Ultrasound A Rare Case of Left Ventricular Noncompaction in LEOPARD Syndrome. 43-44 10.4250/jcu.2018.26.1.43 Kim Kyehwan K Department of Internal Medicine, Gyeonsang National University School of Medicine, Gyeongsang National University Hospital, Jinju, Korea. Kang Min Gyu MG Department of Internal Medicine, Gyeonsang National University (...) National University Hospital, Jinju, Korea. Hwang Seok-Jae SJ Department of Internal Medicine, Gyeonsang National University School of Medicine, Gyeongsang National University Hospital, Jinju, Korea. Hwang Jin-Yong JY Department of Internal Medicine, Gyeonsang National University School of Medicine, Gyeongsang National University Hospital, Jinju, Korea. eng Case Reports 2018 03 28 Korea (South) J Cardiovasc Ultrasound 101477138 1975-4612 Atrial fibrillation LEOPARD syndrome Left ventricular non

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2018 Journal of cardiovascular ultrasound

9. Clinical and Echocardiography Features of Diagnosed in Adulthood Isolated Left Ventricular Noncompaction: A Case Series Study (PubMed)

Clinical and Echocardiography Features of Diagnosed in Adulthood Isolated Left Ventricular Noncompaction: A Case Series Study Left ventricular noncompaction cardiomyopathy (LVNC) is a primary genetic cardiomyopathy with morphologically unique characteristics, including loose "spongy" meshwork. Subjects carrying these disorders were typically presented with triad of heart failure, cardiac arrhythmias, and consequences of mural thrombi formation. The clinical and echocardiographic features (...) regarding LVNC, however, are not widely known.A retrospective survey involving 11 patients who fulfilled echocardiographic criteria for LVNC defined by Jenni et al. was conducted at MacKay Memorial Hospital from January 2009 to March 2017. Parameters assessed by echocardiography and clinical data were further analyzed.Significantly depressed left ventricular systolic function assessed by echocardiography was noticed in a majority of our adult study cases.Considering the fatal complications LVNC may lead

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2018 Journal of medical ultrasound

10. Advanced Biventricular Heart Failure due to Left Ventricular Noncompaction Cardiomyopathy Leading to the Formation of a Gastric Bezoar: The Implications of Heart Failure on the Gastrointestinal Tract (PubMed)

Advanced Biventricular Heart Failure due to Left Ventricular Noncompaction Cardiomyopathy Leading to the Formation of a Gastric Bezoar: The Implications of Heart Failure on the Gastrointestinal Tract Congestive heart failure (CHF) is a chronic disease process affecting multiple organ systems and is associated with significant morbidity and mortality. We report a case of a 43-year-old male with a history of unspecified cardiomyopathy who presented to the hospital with abdominal pain, distention (...) , and nausea for 4 months. He was diagnosed with left ventricular noncompaction and gastroparesis. While symptoms of dyspnea, orthopnea, or increasing peripheral edema are the first that come to mind when thinking of a CHF exacerbation, we must broaden our scope to include such things as nausea, vomiting, abdominal pain, and bloating which can also indicate worsening cardiac function. This case report highlights the significant yet often forgotten gastrointestinal (GI) symptoms that result from advanced

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2018 Case reports in cardiology

11. Holt-Oram syndrome in two families diagnosed with left ventricular noncompaction and conduction disease (PubMed)

Holt-Oram syndrome in two families diagnosed with left ventricular noncompaction and conduction disease 29755943 2019 02 26 2214-0271 4 4 2018 Apr HeartRhythm case reports HeartRhythm Case Rep Holt-Oram syndrome in two families diagnosed with left ventricular noncompaction and conduction disease. 146-151 10.1016/j.hrcr.2017.12.002 Ross Samantha Barratt SB Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Newtown, Australia. Sydney Medical School, University of Sydney, Sydney (...) Cardiology, Centenary Institute, Newtown, Australia. Sydney Medical School, University of Sydney, Sydney, Australia. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. eng Case Reports 2018 02 13 United States HeartRhythm Case Rep 101656239 2214-0271 Genetics Holt-Oram syndrome Left ventricular noncompaction Skeletal anomalies TBX5 2018 5 15 6 0 2018 5 15 6 0 2018 5 15 6 1 epublish 29755943 10.1016/j.hrcr.2017.12.002 S2214-0271(17)30213-0 PMC5944048 J Med Genet. 1996 Apr;33(4):300

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2018 HeartRhythm Case Reports

12. Sarcomere gene variants act as a genetic trigger underlying the development of left ventricular noncompaction. (PubMed)

Sarcomere gene variants act as a genetic trigger underlying the development of left ventricular noncompaction. Left ventricular noncompaction (LVNC) is a primary cardiomyopathy with heterogeneous genetic origins. The aim of this study was to elucidate the role of sarcomere gene variants in the pathogenesis and prognosis of LVNC.We screened 82 Japanese patients (0-35 years old), with a diagnosis of LVNC, for mutations in seven genes encoding sarcomere proteins, by direct DNA sequencing. We

2018 Pediatric Research

13. Potential Common Pathogenic Pathways for the Left Ventricular Noncompaction Cardiomyopathy (LVNC). (PubMed)

Potential Common Pathogenic Pathways for the Left Ventricular Noncompaction Cardiomyopathy (LVNC). Ventricular trabeculation and compaction are two essential morphogenetic events for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with hypoplastic wall and ventricular compact zone deficiencies, which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, the arrest of ventricular wall compaction (...) (noncompaction) is believed to be causative to the left ventricular noncompaction (LVNC), a genetically heterogeneous disorder and the third most common cardiomyopathy among pediatric patients. After critically reviewing recent findings from genetically engineered mouse models, we suggest a model which proposes that defects in myofibrillogenesis and polarization in trabecular cardiomyocytes underly the common pathogenic mechanism for ventricular noncompaction.

2018 Pediatric Cardiology

14. Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease. (PubMed)

Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease. Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications

2017 Pediatric Cardiology

15. Left ventricular noncompaction cardiomyopathy: cardiac, neuromuscular, and genetic factors. (PubMed)

Left ventricular noncompaction cardiomyopathy: cardiac, neuromuscular, and genetic factors. Left ventricular hypertrabeculation (LVHT) or noncompaction is a myocardial abnormality of unknown aetiology, frequently associated with monogenic disorders, particularly neuromuscular disorders, or with chromosomal defects. LVHT is diagnosed usually by echocardiography by the presence of a bilayered myocardium consisting of a thick, spongy, noncompacted endocardial layer and a thin, compacted (...) , epicardial layer. The pathogenesis of LVHT is unsolved, and the diagnostic criteria, prognosis, and optimal treatment of patients with LVHT are under debate. LVHT is categorized as distinct primary genetic cardiomyopathy by the AHA and as unclassified cardiomyopathy by the ESC. LVHT is usually asymptomatic, but can be complicated by heart failure, thromboembolism, or ventricular arrhythmias, including sudden cardiac death. Mortality of patients with LVHT ranges from 5% to 47%. Anticoagulation

2017 Nature reviews. Cardiology

16. Complications Associated With Implantable Cardioverter Defibrillators in Adults With Congenital Heart Disease or Left Ventricular Noncompaction Cardiomyopathy (From the NCDR<sup>®</sup> Implantable Cardioverter-Defibrillator Registry). (PubMed)

Complications Associated With Implantable Cardioverter Defibrillators in Adults With Congenital Heart Disease or Left Ventricular Noncompaction Cardiomyopathy (From the NCDR® Implantable Cardioverter-Defibrillator Registry). 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. In-hospital complications were compared among different diagnoses using the chi-square test for categorical variables

2017 American Journal of Cardiology

17. Prevalence and Prognostic Significance of Left Ventricular Noncompaction in Patients Referred for Cardiac Magnetic Resonance Imaging. (PubMed)

Prevalence and Prognostic Significance of Left Ventricular Noncompaction in Patients Referred for Cardiac Magnetic Resonance Imaging. Presence of prominent left ventricular trabeculation satisfying criteria for left ventricular noncompaction (LVNC) on routine cardiac magnetic resonance examination is frequently encountered; however, the clinical and prognostic significance of these findings remain elusive. This registry aimed to assess LVNC prevalence by 4 current criteria and to prospectively (...) evaluate an association between diagnosis of LVNC by these criteria and adverse events.There were 700 patients referred for cardiac magnetic resonance: 42% were women, median age was 70 years (range, 45-71 years), mean left ventricular ejection fraction was 51% (±17%), and 32% had late gadolinium enhancement on cardiac magnetic resonance. The cohort underwent diagnostic assessment for LVNC by 4 separate imaging criteria-referenced by their authors as Petersen, Stacey, Jacquier, and Captur, with LVNC

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2017 Circulation. Cardiovascular imaging

18. Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism. (PubMed)

Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism. 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. In this review, left ventricular noncompaction cardiomyopathy, which is often caused by mutations in sarcomere and cytoskeletal proteins and is also associated with metabolic abnormalities, is discussed. In addition, cardiomyopathies resulting from mitochondrial dysfunction, metabolic

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2017 Circulation Research

19. Genetic Testing in Pediatric Left Ventricular Noncompaction. (PubMed)

Genetic Testing in Pediatric Left Ventricular Noncompaction. Left ventricular noncompaction (LVNC) can occur in isolation or can co-occur with a cardiomyopathy phenotype or cardiovascular malformation. The yield of cardiomyopathy gene panel testing in infants, children, and adolescents with a diagnosis of LVNC is unknown. By characterizing a pediatric population with LVNC, we sought to determine the yield of cardiomyopathy gene panel testing, distinguish the yield of testing for LVNC

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2017 Circulation. Cardiovascular genetics

20. Bradycardia, Syncope, and Left Ventricular Noncompaction Cardiomyopathy. (PubMed)

Bradycardia, Syncope, and Left Ventricular Noncompaction Cardiomyopathy. 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.Copyright © 2017 Elsevier Inc. All rights reserved.

2017 American Journal of Cardiology

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