Late diagnosis of mucopolysaccharidosis type IVB and successful aortic valve replacement in a 60-year-old female patient
Mucopolysaccharidosis type IVB (MPS IVB) is a very rare lysosomal storage disorder characterized by skeletal dysplasia, hearing disorder, and cardiac valvular disease. Herein, we report an extremely rare manifestation of MPS IVB in a 60-year-old female patient who underwent a successful aortic valve replacement. The patient presented with mild coarse facial features, short stature, mild dyspnea, sternal protrusion, mild lumbar hyperlordosis and waddling gait owing to bilateral femoral head necroses and bilateral arthrosis of the knees. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 12, 2018 Category: Cardiology Authors: Gabriela Dostalova, Zuzana Hlubocka, Jaroslav Lindner, Helena Hulkova, Helena Poupetova, Hana Vlaskova, Jakub Sikora, Ales Linhart, Jiri Zeman, Martin Magner Source Type: research

Reasons for missing noncompaction in myopathies and vice versa
Feature article (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 12, 2018 Category: Cardiology Authors: Josef Finsterer, Claudia St öllberger Tags: Original Article Source Type: research

Late diagnosis of mucopolysaccharidosis type IVB and successful aortic valve replacement in a 60-year-old female patient
Mucopolysaccharidosis type IVB (MPS IVB) is a very rare lysosomal storage disorder characterized by skeletal dysplasia, hearing disorder, and cardiac valvular disease. Herein, we report an extremely rare manifestation of MPS IVB in a 60-year-old female patient who underwent a successful aortic valve replacement. The patient presented with mild coarse facial features, short stature, mild dyspnea, sternal protrusion, mild lumbar hyperlordosis and waddling gait owing to bilateral femoral head necroses and bilateral arthrosis of the knees. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 12, 2018 Category: Cardiology Authors: Gabriela Dostalova, Zuzana Hlubocka, Jaroslav Lindner, Helena Hulkova, Helena Poupetova, Hana Vlaskova, Jakub Sikora, Ales Linhart, Jiri Zeman, Martin Magner Source Type: research

Long non-coding RNA CHRF facilitates cardiac hypertrophy through regulating Akt3 via miR-93
Non-coding RNAs, including long non-coding RNAs (lncRNAs) and microRNAs (miRNAs), have been demonstrated as central mediators in cardiac hypertrophy responses. LncRNA cardiac hypertrophy related factor (CHRF) has been reported to be implicated in cardiac hypertrophy. However, the underlying mechanisms of CHRF have not been thoroughly elucidated. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 11, 2018 Category: Cardiology Authors: Yang Wo, Jie Guo, Peihui Li, Hongxia Yang, Jinshan Wo Tags: Original Article Source Type: research

Thrombospondin-4 mediates cardiovascular remodelling in angiotensin II-induced hypertension
We reported increased heart weight, as well as the occurrence of aortic aneurysms in the Ang II-treated Thbs4−/− animals. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 7, 2018 Category: Cardiology Authors: Teresa Palao, Lejla Medzikovic, Catarina Rippe, Shaynah Wanga, Claudia Al-Mardini, Angela van Weert, Judith de Vos, Nicole N. van der Wel, Henk A. van Veen, Ed T. van Bavel, Karl Sw ärd, Vivian de Waard, Erik NTP Bakker Tags: Original Article Source Type: research

Lymphocyte and monocyte subpopulations in severe aortic stenosis at the time of surgical intervention
Aortic stenosis (AS) is the most common acquired valvular heart disease in adults. Immune system involvement becomes evident during AS development. We sought to investigate the role of different circulating lymphocyte and monocyte subpopulations, with focus on CD4+CD8+ and natural killer T (NKT) cells, in AS. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 6, 2018 Category: Cardiology Authors: Piotr Mazur, Aleksandra Mielimonka, Joanna Natorska, Ewa Wypasek, Bogus ław Gawęda, Dorota Sobczyk, Przemysław Kapusta, Krzysztof Piotr Malinowski, Bogusław Kapelak Source Type: research

New Morphological Classification of Congenital Quadricuspid Aortic Valve and its Histopathologic Features
We report a 52-year-old male patient who had a quadricuspid aortic valve (QAV) associated with aortic regurgitation (AR) and left ventricular hypertrophy (LVH). A new accessory cusp (ACC) with maximum thickness than other cusps was located between right coronary cusp (RCC) and left coronary cusp (LCC). The histopathological features revealed markedly thickened and distorted cusp architecture with fibrosis and/or myxomatous degeneration in both non-coronary cusp (NCC) and ACC. Two equal sizes for larger cusps (RCC and NCC) and two equal sizes for smaller cusps (LCC and ACC) were obtained. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 6, 2018 Category: Cardiology Authors: Ching-Li Cheng, Hsiao-Huang Chang, Wei-Chen Wang, Pei-Jung Huang, Shan-Yang Lin Source Type: research

Lymphocyte and monocyte subpopulations in severe aortic stenosis at the time of surgical intervention
Aortic stenosis (AS) is the most common acquired valvular heart disease in adults. Immune system involvement becomes evident during AS development. We sought to investigate the role of different circulating lymphocyte and monocyte subpopulations, with focus on CD4+CD8+ and natural killer T (NKT) cells, in AS. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 6, 2018 Category: Cardiology Authors: Piotr Mazur, Aleksandra Mielimonka, Joanna Natorska, Ewa Wypasek, Bogus ław Gawęda, Dorota Sobczyk, Przemysław Kapusta, Krzysztof Piotr Malinowski, Bogusław Kapelak Source Type: research

New Morphological Classification of Congenital Quadricuspid Aortic Valve and its Histopathologic Features
We report a 52-year-old male patient who had a quadricuspid aortic valve (QAV) associated with aortic regurgitation (AR) and left ventricular hypertrophy (LVH). A new accessory cusp (ACC) with maximum thickness than other cusps was located between right coronary cusp (RCC) and left coronary cusp (LCC). The histopathological features revealed markedly thickened and distorted cusp architecture with fibrosis and/or myxomatous degeneration in both non-coronary cusp (NCC) and ACC. Two equal sizes for larger cusps (RCC and NCC) and two equal sizes for smaller cusps (LCC and ACC) were obtained. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 6, 2018 Category: Cardiology Authors: Ching-Li Cheng, Hsiao-Huang Chang, Wei-Chen Wang, Pei-Jung Huang, Shan-Yang Lin Source Type: research

Cardiomyocytes have mosaic patterns of protein expression
Skeletal myocytes have well-established fast and slow twitch fibers with unique gene and protein specific expression patterns. By immunohistochemical staining, these show a mosaic pattern across myocytes. We hypothesized cardiac myocytes may behave similarly where some proteins are differentially expressed between mature cardiomyocytes. We utilized the tool HPASubC on over 52,000 cardiac images of the Human Protein Atlas to identify differential protein expression patterns by immunohistochemistry across the cardiomyocytes. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - March 26, 2018 Category: Cardiology Authors: Tony Y. Wang, Dongwon Lee, Karen Fox-Talbot, Dan E. Arking, Aravinda Chakravarti, Marc K. Halushka Tags: Original Article Source Type: research

Cerebrovascular myocardium-tissue embolism: a rare complication of heart surgery: autopsy case report
Cardiovascular surgery is often associated with significant mortality ranging from 3% to 6%, and complications labeled as cerebral are identified as a cause of death in about 3% –9% of heart surgery associated mortality, including cerebrovascular embolism. With regards to heart surgery patients, neurologic complications are caused by arterial embolism predominantly associated with left-sided infective endocarditis. In this paper, we would like to present an unusual autops y finding of cellular–myocardial embolism in the middle cerebral artery in a patient after acute mitral valve repair, coronary artery bypass graft, a...
Source: Cardiovascular Pathology - March 18, 2018 Category: Cardiology Authors: Jan Hrudka, Jan Hlavi čka, Marie Claire Šmůlová, Petr Kujal Tags: Case Report Source Type: research

Cerebrovascular myocardium-tissue embolism: a rare complication of heart surgery: autopsy case report
Cardiovascular surgery is often associated with significant mortality ranging from 3 –6%, and complications labeled as cerebral are identified as a cause of death in about 3–9% of heart surgery associated mortality, including cerebrovascular embolism. With regards to heart surgery patients, neurologic complications caused by arterial embolism predominantly associated with left-s ided infective endocarditis. In this paper, we would like to present an unusual autopsy finding of cellular-myocardial embolism in the middle cerebral artery, in a patient after acute mitral valve repair, coronary artery bypass graft and maze p...
Source: Cardiovascular Pathology - March 18, 2018 Category: Cardiology Authors: Jan Hrudka, Jan Hlavi čka, Marie Claire Šmůlová, Petr Kujal Source Type: research