scholarly journals Natural History of Arrhythmogenic Cardiomyopathy

2020 ◽  
Vol 9 (3) ◽  
pp. 878 ◽  
Author(s):  
Giulia Mattesi ◽  
Alessandro Zorzi ◽  
Domenico Corrado ◽  
Alberto Cipriani
Keyword(s):  
Natural History ◽  
Genetic Basis ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
The Elderly ◽  
Ventricular Myocardium ◽  
Muscle Disease ◽  
Arrhythmogenic Cardiomyopathy ◽  
Age Related ◽  
Genes Encoding ◽  
History Of

Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease characterized by a scarred ventricular myocardium with a distinctive propensity to ventricular arrhythmias (VAs) and sudden cardiac death, especially in young athletes. Arrhythmogenic right ventricular cardiomyopathy (ARVC) represents the best characterized variant of AC, with a peculiar genetic background, established diagnostic criteria and management guidelines; however, the identification of nongenetic causes of the disease, combined with the common demonstration of biventricular and left-dominant forms, has led to coin the term of “arrhythmogenic cardiomyopathy”, to better define the broad spectrum of the disease phenotypic expressions. The genetic basis of AC are pathogenic mutations in genes encoding the cardiac desmosomes, but also non-desmosomal and nongenetic variants were reported in patients with AC, some of which showing overlapping phenotypes with other non-ischemic diseases. The natural history of AC is characterized by VAs and progressive deterioration of cardiac performance. Different phases of the disease are recognized, each characterized by pathological and clinical features. Arrhythmic manifestations are age-related: Ventricular fibrillation and SCD are more frequent in young people, while sustained ventricular tachycardia is more common in the elderly, depending on the different nature of the myocardial lesions. This review aims to address the genetic basis, the clinical course and the phenotypic variants of AC.

Evidence-based management of arrhythmogenic right ventricular cardiomyopathy in pregnancy

2020 ◽  
Author(s):  
Jagjit Khosla ◽  
Reshma Golamari ◽  
Alice Cai ◽  
Jamal Benson ◽  
Wilbert S Aronow ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Retrospective Studies ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Genetic Disorder ◽  
Ventricular Myocardium ◽  
Evidence Based ◽  
Ventricular Cardiomyopathy ◽  
Genes Encoding ◽  
In Pregnancy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder resulting in fibrofatty replacement of the myocardium. Genetic mutations in genes encoding for desmosome proteins result in a ventricular myocardium prone to arrhythmias and heart failure. Although ARVC is known for a few decades, most of the outcomes in pregnancy are reported recently. Pregnancy leads to significant physiological changes with excess mechanical stress on the myocardium. All the retrospective studies suggest that pregnancy is well tolerated in these patients despite the high risk of arrhythmias and heart failure. Our review focuses on the most up-to-date evidence on the management of ARVC patients during the antepartum and postpartum period.

Left Side Right Ventricular Cardiomyopathy

2002 ◽  
Vol 42 (4) ◽  
pp. 313-317 ◽  
Author(s):  
M Michalodimitrakis ◽  
A Papadomanolakis ◽  
J Stiakakis ◽  
K Kanaki
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Pathologic Examination ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
First Case ◽  
Fibrofatty Tissue ◽  
Myocardial Muscle

Arrhythmogenic right ventricular cardiomyopathy or dysplasia, a heart muscle disease of unknown cause, is anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadipose tissue. It is usually considered a selective disorder whereas concomitant left ventricular involvement has been noted in a few cases. Two cases of the disease with evidence of extensive left ventricular involvement at pathologic examination are described. Hearts from two patients who died suddenly showed extensive biventricular infiltration by fibrofatty tissue in the first case and exclusively in the wall of the left ventricle the localization of the fatty and fibrotic lesions. These findings might suggest that the various localizations of the fibroadipose tissue are rather different expressions of the same disease and it is preferable to be termed ‘arrhythmogenic cardiomyopathy’ as other studies also indicate.

scholarly journals 105 Natural History of Crohn's Disease in the Elderly: A Population-Based Study

2012 ◽  
Vol 142 (5) ◽  
pp. S-26 ◽  
Author(s):  
Cloe Charpentier ◽  
Corinne Gower-Rousseau ◽  
Guillaume Savoye ◽  
Julia Salleron ◽  
Mathurin Fumery ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Natural History ◽  
The Elderly ◽  
Population Based ◽  
Population Based Study ◽  
History Of

scholarly journals Natural History of Drusen Morphology in Age-Related Macular Degeneration Using Spectral Domain Optical Coherence Tomography

Ophthalmology ◽  
2011 ◽  
Vol 118 (12) ◽  
pp. 2434-2441 ◽  
Author(s):  
Zohar Yehoshua ◽  
Fenghua Wang ◽  
Philip J. Rosenfeld ◽  
Fernando M. Penha ◽  
William J. Feuer ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Natural History ◽  
Macular Degeneration ◽  
Age Related Macular Degeneration ◽  
Spectral Domain ◽  
Optical Coherence ◽  
Age Related ◽  
History Of

scholarly journals Age-related exacerbation of hematopoietic organ damage induced by systemic hyper-inflammation in senescence-accelerated mice

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tomonori Harada ◽  
Isao Tsuboi ◽  
Hirotsugu Hino ◽  
Miyuki Yuda ◽  
Yoko Hirabayashi ◽  
...  
Keyword(s):  
Stromal Cells ◽  
The Elderly ◽  
Organ Damage ◽  
Hematopoietic Organ ◽  
Aged Mice ◽  
Gm Csf ◽  
Expression Of Genes ◽  
Age Related ◽  
Genes Encoding ◽  
Positive Regulators

AbstractHemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic hyper-inflammatory disorder. The mortality of HLH is higher in the elderly than in young adults. Senescence-accelerated mice (SAMP1/TA-1) exhibit characteristic accelerated aging after 30 weeks of age, and HLH-like features, including hematopoietic organ damage, are seen after lipopolysaccharide (LPS) treatment. Thus, SAMP1/TA-1 is a useful model of hematological pathophysiology in the elderly with HLH. In this study, dosing of SAMP1/TA-1 mice with LPS revealed that the suppression of myelopoiesis and B-lymphopoiesis was more severe in aged mice than in young mice. The bone marrow (BM) expression of genes encoding positive regulators of myelopoiesis (G-CSF, GM-CSF, and IL-6) and of those encoding negative regulators of B cell lymphopoiesis (TNF-α) increased in both groups, while the expression of genes encoding positive-regulators of B cell lymphopoiesis (IL-7, SDF-1, and SCF) decreased. The expression of the GM-CSF-encoding transcript was lower in aged mice than in young animals. The production of GM-CSF by cultured stromal cells after LPS treatment was also lower in aged mice than in young mice. The accumulation of the TNF-α-encoding transcript and the depletion of the IL-7-encoding transcript were prolonged in aged mice compared to young animals. LPS dosing led to a prolonged increase in the proportion of BM M1 macrophages in aged mice compared to young animals. The expression of the gene encoding p16INK4a and the proportion of β-galactosidase- and phosphorylated ribosomal protein S6-positive cells were increased in cultured stromal cells from aged mice compared to those from young animals, while the proportion of Ki67-positive cells was decreased in stromal cells from aged mice. Thus, age-related deterioration of stromal cells probably causes the suppression of hematopoiesis in aged mice. This age-related latent organ dysfunction may be exacerbated in elderly people with HLH, resulting in poor prognosis.

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