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Procedure: Heart Transplant

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Total 58 results found since Jan 2013.

LMNA-associated myopathies: The Italian experience in a large cohort of patients
Conclusions: Our data provided new insights in LMNA-related myopathies, whose natural history appears to be dominated by cardiac involvement and related complications.
Source: Neurology - October 27, 2014 Category: Neurology Authors: Maggi, L., D'Amico, A., Pini, A., Sivo, S., Pane, M., Ricci, G., Vercelli, L., D'Ambrosio, P., Travaglini, L., Sala, S., Brenna, G., Kapetis, D., Scarlato, M., Pegoraro, E., Ferrari, M., Toscano, A., Benedetti, S., Bernasconi, P., Colleoni, L., Lattanzi, Tags: Muscle disease ARTICLE Source Type: research

A family with limb girdle muscular dystrophy type 1B and multiple exostoses.
DISCUSSION: The clinical, genetic, and muscle biopsy findings suggest that both mutations are pathogenic. The EXT2 mutation was most likely responsible for the multiple exostoses phenotype in mother and son, whereas the myopathy was probably caused by a combined effect of the LMNA and EXT2 mutations.
. PMID: 31296281 [PubMed - as supplied by publisher]
Source: Clinical Neuropathology - July 11, 2019 Category: Pathology Authors: Romeike BFM, Becker K, Großkreutz J, Schulz S, Weis J, Cirak S Tags: Clin Neuropathol Source Type: research

Clinical Findings and Prognosis of Danon Disease. An Analysis of the Spanish Multicenter Danon Registry
ConclusionsThe clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent.ResumenIntroducción y objetivosLa enfermedad de Danon (ED) es una enfermedad producida por mutaciones en el gen LAMP2. Se la considera una enfermedad multisistémica caracterizada por miocardiopatía hipertrófica con preexcitación e hipertrofia extrema, discapacidad intelectual, miopatía, presentación infantil y peor pronóstico en varones. Hay pocas series que permitan conocer las características clínicas y e...
Source: Revista Espanola de Cardiologia - August 12, 2018 Category: Cardiology Source Type: research

Single-Cell RNA Sequencing to Dissect the Immunological Network of Autoimmune Myocarditis.
Conclusions: We herein present a comprehensive single-cell landscape of the cardiac immune cells in different EAM phases. In addition, we elucidated the contribution of Hif1a to the inflammatory response through the regulation of immune cell activity, particularly of macrophage cluster 2 and Th17 cells. Moreover, a Hif1a inhibitor alleviated inflammatory cell infiltration of the EAM model and may serve as a potential therapeutic target in the clinic. PMID: 32431172 [PubMed - as supplied by publisher]
Source: Circulation - May 19, 2020 Category: Cardiology Authors: Hua X, Hu G, Hu Q, Chang Y, Hu Y, Gao L, Chen X, Yang PC, Zhang Y, Li M, Song J Tags: Circulation Source Type: research

Early cardiac rehabilitation after heart transplantation in a patient with limb-girdle muscular dystrophy: A case report
Rationale: Cardiac rehabilitation (CR) after heart transplantation (HT) decreases the mortality rate and increases exercise capacity of patients. Dilated cardiomyopathy develops in most patients with muscular dystrophy (MD), leading to advanced heart failure, necessitating the use of left ventricular assist devices or HT. As the clinical outcomes of left ventricular assist devices and HT in patients with myopathy differ from those in patients without myopathy, CR adapted to patients with MD should be considered. Patient concerns: A 39-year-old man with limb-girdle muscular dystrophy developed dilated cardiomyopa...
Source: Medicine - July 29, 2022 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Cardio Notes: Curbing Heart Worries After Liver Transplant
(MedPage Today) -- More intense exercise after a liver transplant may stave off metabolic syndrome, at least in some patients. Also, a possible genetic basis for statin-related myopathy and news about a peripheral stent and a heart valve.
Source: MedPage Today State Required CME - August 12, 2013 Category: Consumer Health News Source Type: news

Statins side effects are minimal, study argues
ConclusionThis meta-analysis pooled results from 29 studies and has shown a very small increased risk of newly diagnosed diabetes mellitus. This is the same as the decreased risk of any cause of death in people taking statins, compared to placebo, to prevent a heart attack or stroke.The researchers point out some limitations to the meta-analysis: Each study did not report on all of the side effects, meaning that for each category of side effect, the number of participants differed. The side effect categories were only included if at least 500 people had reported suffering from it. This means there may be numerous other si...
Source: NHS News Feed - March 13, 2014 Category: Consumer Health News Tags: Heart/lungs Medication Source Type: news

G.P.145: LMNA-related muscular dystrophies: Clinical and histopathological spectrum in Argentina
Conclusion: There is a broad clinical spectrum of laminopathies, including a congenital form. Even when muscle biopsy shows inflammation or features suggestive of congenital myopathy, the clinical phenotype should prompt molecular testing for lamin A/C mutations to prevent cardiac complications.
Source: Neuromuscular Disorders - September 4, 2014 Category: Neurology Authors: S. Monges, F. Lubieniecki, F. de Castro, V. Lafuente, M. Gonzalez, G. Reyes, L. Chertkoff, S. Quijano-Roy, N.B. Romero, P. Richard, G. Bonne, P. Guicheney, M. Sacolitti, A.L. Taratuto Source Type: research

Danon disease: a phenotypic expression of LAMP-2 deficiency
Abstract Danon disease is an X-linked disorder clinically characterized by the triad of hypertrophic cardiomyopathy, myopathy, and intellectual disability. Cardiomyopathy is a severe and life-threatening problem, for which cardiac transplantation is the only therapeutic option. The most striking finding in muscle biopsy samples is small basophilic granules scattered in myofibers, which are in fact small autophagic vacuoles surrounded by membranes with sarcolemmal features characterized by the recruitment of sarcolemmal proteins and acetylcholine esterase and by the presence of basal lamina on its luminal side. The...
Source: Acta Neuropathologica - January 15, 2015 Category: Neurology Source Type: research

Recessive MYH6 Mutations in Hypoplastic Left Heart With Reduced Ejection Fraction Original Articles
Conclusions— In contrast to dominant MYH6 mutations with variable penetrance identified in other congenital heart defects and dilated cardiomyopathy, this study reveals compound heterozygosity for recessive MYH6 mutations in patients with hypoplastic left heart and reduced systemic right ventricular ejection fraction. These findings implicate a shared molecular basis for the developmental arrest and latent myopathy of left and right ventricles, respectively.
Source: Circulation: Cardiovascular Genetics - August 18, 2015 Category: Cardiology Authors: Theis, J. L., Zimmermann, M. T., Evans, J. M., Eckloff, B. W., Wieben, E. D., Qureshi, M. Y., O'Leary, P. W., Olson, T. M. Tags: Contractile function, Clinical genetics, Cardiac development Original Articles Source Type: research

Left Ventricular Noncompaction Cardiomyopathy in Children Listed for Heart Transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC 4 and a recent evaluation of LVNC outcomes utilizing the United Network for Organ Sharing...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Source Type: research

Left ventricular non-compaction cardiomyopathy in children listed for heart transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course, including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC,4 and a recent evaluation of LVNC outcomes using the United Network for Organ Sharing d...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Tags: Research Correspondence Source Type: research

Prenatal features of Pena‐Shokeir sequence with atypical response to acoustic stimulation
ABSTRACT A fetal sonographic screening examination performed at 23 weeks showed polyhydramnios, micrognathia, fixed postures of all long bones, but no movement and no breathing. The fetus showed fetal heart rate acceleration but no movement when acoustic stimulation was applied with artificial larynx. All these findings persisted on serial examinations. The neonate was stillborn at 37 weeks and a final diagnosis of Pena‐Shokeir sequence was made. In addition to typical sonographic features of Pena‐Shokeir sequence, fetal heart rate accelerations with no movement in response to acoustic stimulation suggests that periphe...
Source: Journal of Clinical Ultrasound - June 17, 2016 Category: Radiology Authors: Sirida Pittyanont, Phudit Jatavan, Songkiat Suwansirikul, Theera Tongsong Tags: Case Report Source Type: research

Prenatal features of Pena ‐Shokeir sequence with atypical response to acoustic stimulation
ABSTRACT A fetal sonographic screening examination performed at 23 weeks showed polyhydramnios, micrognathia, fixed postures of all long bones, but no movement and no breathing. The fetus showed fetal heart rate acceleration but no movement when acoustic stimulation was applied with artificial larynx. All these findings persisted on serial examinations. The neonate was stillborn at 37 weeks and a final diagnosis of Pena‐Shokeir sequence was made. In addition to typical sonographic features of Pena‐Shokeir sequence, fetal heart rate accelerations with no movement in response to acoustic stimulation suggests that periphe...
Source: Journal of Clinical Ultrasound - June 16, 2016 Category: Radiology Authors: Sirida Pittyanont, Phudit Jatavan, Songkiat Suwansirikul, Theera Tongsong Tags: Case Report Source Type: research

Clinical Reasoning: A 33-year-old man with cardiomyopathy and myopathy
An 18-year-old Hmong man sought medical care because of worsening performance on military training exercises. He had a previous syncopal episode with prompt recovery. His medical and developmental history were otherwise unremarkable. A chest radiograph revealed cardiomegaly and, after further cardiac tests, he was diagnosed with postinfectious or idiopathic cardiomyopathy. His cardiac function deteriorated and heart transplantation was pursued. During preoperative evaluation, his serum creatine kinase (CK) was noted to be persistently elevated in the 4,000s, prompting further investigation, but since he was not weak or oth...
Source: Neurology - August 21, 2016 Category: Neurology Authors: Foster, L. A., Courville, E. L., Manousakis, G. Tags: Lipidoses, Muscle disease, Cardiac, All Genetics RESIDENT AND FELLOW SECTION Source Type: research